Materiały źródłowe

• #1 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Autoimmune diseases occur when the immune system, which normally protects you from infections and cancer, makes a mistake and starts to damage your bodys own tissues. Morphea (syn. localized scleroderma) occurs as a result of autoimmune fibrosing (leather-like) injury to the skin and sometimes the underlying fat, muscle and bone. Morphea is sometimes referred to as localized scleroderma. However, despite their similar names, patients with morphea are not at increased risk of developing internal organ scleroderma involvement (systemic sclerosis). […] In morphea, the immune system mistakenly causes inflammation of the skin. This inflammation then causes fibrosis (hardening) of the skin and underlying tissues. There are several different types of morphea some of which characteristically begin in adults while others characteristically start in childhood.

• #2 Morphea – Wikipedia

  https://en.wikipedia.org/wiki/Morphea

  Morphea most often presents as macules or plaques a few centimeters in diameter, but also may occur as bands or in guttate lesions or nodules. […] Morphea is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes specific conditions ranging from very small plaques only involving the skin to widespread disease causing functional and cosmetic deformities. […] Morphea discriminates from systemic sclerosis by its supposed lack of internal organ involvement. […] Up to 15% of morphea patients may fall into this previously unrecognized category. […] Morphea is a form of scleroderma that is more common in women than men, in a ratio 3:1. […] Morphea occurs in childhood as well as in adult life. […] Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people. […] Morphea profunda shows little response to corticosteroids and tends to run a more chronic debilitating course. […] Pansclerotic morphea is manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, the bone, all causing disabling limitation of motion of joints.

• #3 Morphea – Wikipedia

  https://en.wikipedia.org/wiki/Morphea

  Morphea most often presents as macules or plaques a few centimeters in diameter, but also may occur as bands or in guttate lesions or nodules. […] Morphea is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes specific conditions ranging from very small plaques only involving the skin to widespread disease causing functional and cosmetic deformities. […] Morphea discriminates from systemic sclerosis by its supposed lack of internal organ involvement. […] Up to 15% of morphea patients may fall into this previously unrecognized category. […] Morphea is a form of scleroderma that is more common in women than men, in a ratio 3:1. […] Morphea occurs in childhood as well as in adult life. […] Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people. […] Morphea profunda shows little response to corticosteroids and tends to run a more chronic debilitating course. […] Pansclerotic morphea is manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, the bone, all causing disabling limitation of motion of joints.

• #4 What Is New in Morphea—Narrative Review on Molecular Aspects and New Targeted Therapies

  https://www.mdpi.com/2077-0383/13/23/7134

  The only population-based study conducted by Peterson et al. in Olmsted County, Minnesota, USA, between 1960 and 1993 suggests that the incidence of morphea is approximately 0.4–2.7 cases/100,000. The disease is 2.6–6 times more common in women than in men. […] There are two peaks of morphea incidence, the first between 2 and 14 years of age and the second in adults between 40 and 50 years of age. […] Several forms of morphea have been described, each with different clinical manifestations and degrees of connective tissue involvement. […] In adults, the most common clinical form of morphea is the plaque-like variety (morphea en plaques, MEP), while in children it is the linear variety. […] The differential diagnosis of morphea is presented in Table 1. […] Depending on the clinical form of morphea, its activity or severity, the depth and extent of the lesions and the degree of tissue damage, local and systemic treatments are used in therapy. Decisions must take into account the chronic and recurrent nature of the disease and the presence of atrophy and extracutaneous complications, which can lead to significant disability.

• #5 Morphea: a practical review of its diagnosis, classification and treatment

  http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S0016-38132019000500483

  Morphea, also known as localized scleroderma, is a chronic inflammatory disease of the dermis and subcutaneous cellular tissue in which a sclerotic process with diffuse thickening and induration of the skin is generated, as well as atrophy at various levels. Usually, its evolution is benign; however, 10 % of patients suffer severe sclerosis and atrophy, which can cause deformity, contractures or growth disorders. […] Diagnosis and management of patients with morphea is not always easy and can be a challenge for the dermatologist. […] Morphea can affect any part of the body and is characterized by one or several well-defined plaques of varying sizes of thickened, bright, atrophic skin; either hyper or hypopigmented. The lesions begin insidiously as erythematous-edematous or violet plaques that spread centrifugally; they are usually asymptomatic and go unnoticed by the patient. As they evolve, they start to become sclerotic, indurated tissue, acquire a bright white color and, in the periphery, violet rings are appreciated, which disappear when progression of the lesion stops and give rise to residual pigmentary changes. They evolve chronically until atrophy reaches various levels: epidermis, dermis or subcutaneous cellular tissue. Sometimes, different clinical forms are combined in one single patient. Activity of the lesions lasts from three to five years, with a tendency to remission; however, there may be recurrences. In children, more severe, mutilating and disabling forms occur, such as disabling pan-sclerotic morphea of childhood.

• #6 Morphea – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559010/

  Morphea, also called localized scleroderma, is a rare inflammatory skin condition that can also affect the subcutaneous tissues. Morphea encompasses multiple variants with different outcomes ranging for small isolated benign skin lesions to aggressive lesions, which can cause significant deformities. […] Morphea symptoms are usually limited to the skin and the subcutaneous tissue. Still, extracutaneous involvement is reported in approximately 22% of patients, with linear and generalized subtypes in a cohort reported by Zulian et al. Localized scleroderma can rarely cause debilitating lesions, which result in joint contractures, limb growth defects, and other extracutaneous features in children. […] Early lesions of morphea present as erythematous skin lesions with some itching and tenderness. As the lesions progress, they develop sclerotic centers surrounded by a violaceous border. The lesions eventually result in hyperpigmentation or hypopigmentation with atrophy of the skin and subcutaneous tissue. In most patients, the lesions are self-limiting and resolve in a few years, but some patients can develop new lesions with ongoing symptoms.

• #7 What Is New in Morphea—Narrative Review on Molecular Aspects and New Targeted Therapies

  https://www.mdpi.com/2077-0383/13/23/7134

  The only population-based study conducted by Peterson et al. in Olmsted County, Minnesota, USA, between 1960 and 1993 suggests that the incidence of morphea is approximately 0.4–2.7 cases/100,000. The disease is 2.6–6 times more common in women than in men. […] There are two peaks of morphea incidence, the first between 2 and 14 years of age and the second in adults between 40 and 50 years of age. […] Several forms of morphea have been described, each with different clinical manifestations and degrees of connective tissue involvement. […] In adults, the most common clinical form of morphea is the plaque-like variety (morphea en plaques, MEP), while in children it is the linear variety. […] The differential diagnosis of morphea is presented in Table 1. […] Depending on the clinical form of morphea, its activity or severity, the depth and extent of the lesions and the degree of tissue damage, local and systemic treatments are used in therapy. Decisions must take into account the chronic and recurrent nature of the disease and the presence of atrophy and extracutaneous complications, which can lead to significant disability.

• #8 Morphea – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/symptoms-causes/syc-20375283

  Morphea is a rare skin condition characterized by small red or purple patches that develop firm white or ivory centers. The affected skin becomes tight and less flexible. […] Morphea (mor-FEE-uh) is a rare condition that causes painless, discolored patches on your skin. […] Typically, the skin changes appear on the belly, chest or back. But they might also appear on your face, arms and legs. Over time the patches may become firm, dry and smooth. Morphea tends to affect only the outer layers of your skin. But some forms of the condition also affect deeper tissues and may restrict movement in the joints. […] Signs and symptoms of morphea vary depending on the type and stage of the condition. They include: Reddish or purplish oval patches of skin, often on the belly, chest or back; Patches that gradually develop a lighter or whitish center; Linear patches, especially on the arms or legs and possibly the forehead or scalp; A gradual change in the affected skin, which becomes firm, thickened, dry and shiny.

• #9 Childhood Sclerodermas & Morphea | Riley Children’s Health

  https://www.rileychildrens.org/health-info/childhood-sclerodermas-morpheas

  Scleroderma is a group of rare diseases that involves the tightening and hardening of the skin and connective tissues of the body. […] The main symptoms of localized scleroderma include: Swelling of the skin or fingers, Change in the color of the skin, especially when there is a change in air temperature, Thickening or scar-like changes in the skin, Tightness in the joints that can prevent normal movement of the arms, legs, hands and feet, Loss of hair and sweat glands in the affected areas over time. […] If your child has localized scleroderma, it is possible that the skin problems that are part of the condition will naturally fade away within three to five years. If your child has systemic scleroderma, the condition will likely become worse, and it is important to follow the prescribed treatment plan.

• #10 Morphea – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/symptoms-causes/syc-20375283

  Morphea is a rare skin condition characterized by small red or purple patches that develop firm white or ivory centers. The affected skin becomes tight and less flexible. […] Morphea (mor-FEE-uh) is a rare condition that causes painless, discolored patches on your skin. […] Typically, the skin changes appear on the belly, chest or back. But they might also appear on your face, arms and legs. Over time the patches may become firm, dry and smooth. Morphea tends to affect only the outer layers of your skin. But some forms of the condition also affect deeper tissues and may restrict movement in the joints. […] Signs and symptoms of morphea vary depending on the type and stage of the condition. They include: Reddish or purplish oval patches of skin, often on the belly, chest or back; Patches that gradually develop a lighter or whitish center; Linear patches, especially on the arms or legs and possibly the forehead or scalp; A gradual change in the affected skin, which becomes firm, thickened, dry and shiny.

• #11 Morphea: Symptoms, Causes & Treatment | Types of Morphea

  https://resources.healthgrades.com/right-care/skin-hair-and-nails/morphea

  Morphea causes discolored, usually oval-shaped skin patches that gradually develop a lighter center. The involved skin can become thick, hard and waxy. It may also have less hair and sweat less than other areas. The patches typically develop on the trunk and sometimes on the limbs or face. […] Morphea symptoms can vary depending on the type, severity and stage of the condition. […] The most common morphea symptoms include: Reddish or purplish discolored skin patches, which may develop a lighter center. Skin patches are generally oval-shaped unless it is linear morphea. Hardening or thickening of the skin in the area. Loss of hair and sweat glands in the area. Waxy or scar-like appearance with a fading of color to a brownish hyperpigmentation after the inflammation subsides. Lesions usually affect the trunk and limbs with circumscribed and generalized morphea. People with generalized morphea may also have fatigue and joint pain. Linear morphea most often occurs on the head, face, or upper body. People with pansclerotic morphea may have limited range of motion if joints are involved.

• #12 Morphea – Therapeutics in Dermatology

  https://www.therapeutique-dermatologique.org/spip.php?article1641

  Morphea tends to develop slowly. The size of the lesions varies and usually they are oval in shape. Initially, the affected areas can be itchy, uncomfortable or numb. The lesions usually appear as bruise-like pink areas in the skin. Sometimes the affected areas of skin turn slightly darker and beige coloured. Over time, the affected areas become thicker and turn pale and waxy in the middle and the skin feels firmer than normal when pinched. In some areas, such as over a joint, mobility can be reduced. Hair is usually lost in the affected areas and the skin feels dry because the sweat is no longer produced. Rarely, fatty tissue and muscle under the lesions disappear and the skin becomes stuck onto the underlying bone. […] Generalized morphea normally begins insidiously on the trunk as plaque morphea. An individual lesion is indistinguishable from classic plaque morphea, except that it does not stop expanding. Multiple plaques rapidly join and can affect nearly the entire trunk, often only sparing the nipples. As the disease progresses, it may result in disabling constrictions that even cause difficulty in breathing due to impaired thorax mobility and inflammation of the intercostal muscles. This variant is usually persistent given its often limited response to treatment. […] In adult-onset morphea, localized morphea involves one to three affected areas. It is the most common subtype in adults and is unlikely to involve the muscle or bone. Generalized morphea presents as four or more areas of skin and subcutaneous involvement. Adults can also get linear morphea.

• #13 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea may be asymptomatic, or symptoms may arise from the skin or deeper tissues, or they may due to extracutaneous manifestations. […] Some patients experience itch and/or pain from actively inflamed areas of morphoea. […] Deep involvement over joints causes joint pain or limited joint movement (contractures). […] Up to 30% of patients with more severe types of linear or generalised morphoea can have extracutaneous non-specific inflammatory symptoms. […] Morphoea can follow a protracted course, which can be relapsing and remitting, or chronically active. […] Relapse can occur after successful treatment, especially in morphoea that begins in childhood.

• #14 Morphea

  https://www.dermacompass.net/en/diseases/morphea

  Initially, there are centrifugally growing whitish, brownish or red plaques. An indurated, ivory-coloured atrophic zone is often visible centrally. The lesions are bordered by a blue-red erythema rim, the so-called Lilac Ring, in case of progression. […] Depending on the form, subcutaneous, fascia, muscle, periosteum and skeletal tissue are affected during progression. […] Severity: depends on the localisation and extent of the skin lesions. […] Most of the time, progression stops after about 7 years.

• #15 Morphea – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/symptoms-causes/syc-20375283

  Morphea is a rare skin condition characterized by small red or purple patches that develop firm white or ivory centers. The affected skin becomes tight and less flexible. […] Morphea (mor-FEE-uh) is a rare condition that causes painless, discolored patches on your skin. […] Typically, the skin changes appear on the belly, chest or back. But they might also appear on your face, arms and legs. Over time the patches may become firm, dry and smooth. Morphea tends to affect only the outer layers of your skin. But some forms of the condition also affect deeper tissues and may restrict movement in the joints. […] Signs and symptoms of morphea vary depending on the type and stage of the condition. They include: Reddish or purplish oval patches of skin, often on the belly, chest or back; Patches that gradually develop a lighter or whitish center; Linear patches, especially on the arms or legs and possibly the forehead or scalp; A gradual change in the affected skin, which becomes firm, thickened, dry and shiny.

• #16 Morphea | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/morphea?content_id=CON-20375265

  Morphea is a rare skin condition characterized by small red or purple patches that develop firm white or ivory centers. The affected skin becomes tight and less flexible. […] Morphea (mor-FEE-uh) is a rare condition that causes painless, discolored patches on your skin. […] Typically, the skin changes appear on the belly, chest or back. But they might also appear on your face, arms and legs. Over time the patches may become firm, dry and smooth. Morphea tends to affect only the outer layers of your skin. But some forms of the condition also affect deeper tissues and may restrict movement in the joints. […] Signs and symptoms of morphea vary depending on the type and stage of the condition. They include: Reddish or purplish oval patches of skin, often on the belly, chest or back; Patches that gradually develop a lighter or whitish center; Linear patches, especially on the arms or legs and possibly the forehead or scalp; A gradual change in the affected skin, which becomes firm, thickened, dry and shiny.

• #17 Morphea: progress to date and the road ahead

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8033330/

  Morphea is defined by periods of activity (inflammation and fibrosis) which leads to damage and atrophy. Activity in morphea is characterized histologically by an inflammatory dermal and subcutaneous lymphocytic infiltrate manifesting clinically as erythema, edema, and lesion extension, with patients reporting symptoms such as pain and pruritis. […] The pathological changes of morphea may affect the dermis, subcutis, soft tissue, and sometimes bone. Fibrosis and resultant atrophy of the dermis, soft tissue, and bone can cause significant deformity and functional impairment, such as contractures, limb length discrepancy, or limitations in range of motion. […] Current standard of care therapies for morphea are immunosuppressive agents that aim to shut down disease activity, and thus early and accurate assessment of activity is crucial in preventing permanent cosmetic and functional sequelae.

• #18 Morphea: progress to date and the road ahead

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8033330/

  Morphea is defined by periods of activity (inflammation and fibrosis) which leads to damage and atrophy. Activity in morphea is characterized histologically by an inflammatory dermal and subcutaneous lymphocytic infiltrate manifesting clinically as erythema, edema, and lesion extension, with patients reporting symptoms such as pain and pruritis. […] The pathological changes of morphea may affect the dermis, subcutis, soft tissue, and sometimes bone. Fibrosis and resultant atrophy of the dermis, soft tissue, and bone can cause significant deformity and functional impairment, such as contractures, limb length discrepancy, or limitations in range of motion. […] Current standard of care therapies for morphea are immunosuppressive agents that aim to shut down disease activity, and thus early and accurate assessment of activity is crucial in preventing permanent cosmetic and functional sequelae.

• #19 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Patients with generalized morphea are more likely to feel tired and to have joint pain than other variants of morphea. […] Patients with generalized morphea may experience fatigue and joint pain. Patients with generalized and pansclerotic morphea may experience difficulty with deep breathing, if all of the skin on their chest is involved. If a limb is involved, children with linear morphea may have shortening of the involved limb. Linear morphea involving the scalp and face may develop seizures and neurologic problems. Seizures and neurological deficits may also complicate progressive facial hemiatrophy. Children with linear morphea of the head may also have eye involvement and should see an ophthalmologist. […] For the vast majority of patients with morphea, the condition will burn out with or without treatment in 3 to 5 years. This means that no new lesions will develop, existing lesions will not grow, and any red or purple discoloration around the lesions will turn brown (postinflammatory hyperpigmentation). The vast majority of patients are responsive to therapy be it creams, light therapy or systemic immunosuppressive drugs such as methotrexate.

• #20 Morphea | Altru Health System

  https://www.altru.org/health-library/conditions/morphea

  Morphea (mor-FEE-uh) is a rare condition that causes painless, discolored patches on your skin. […] Typically, the skin changes appear on the belly, chest or back. But they might also appear on your face, arms and legs. Over time the patches may become firm, dry and smooth. Morphea tends to affect only the outer layers of your skin. But some forms of the condition also affect deeper tissues and may restrict movement in the joints. […] Signs and symptoms of morphea vary depending on the type and stage of the condition. They include: Reddish or purplish oval patches of skin, often on the belly, chest or back. Patches that gradually develop a lighter or whitish center. Linear patches, especially on the arms or legs and possibly the forehead or scalp. A gradual change in the affected skin, which becomes firm, thickened, dry and shiny.

• #21 Morphea: Symptoms, Causes, and Treatments

  https://www.healthline.com/health/morphea

  Morphea predominately affects your skin. It doesnt involve your internal organs. In most cases, it resolves on its own, but you can experience a relapse. […] More severe forms can lead to cosmetic deformities, and it occasionally affects the muscle, joints, or bone. […] In general, morphea causes discolored, thickened patches of skin that are oval in shape. The outer edge of the lesion may be lilac, and the patch itself is usually reddish in color. It gradually becomes white or yellow in color toward the center of the oval. […] This involves multiple widespread lesions that are larger. Generalized morphea can affect deeper tissues, which might lead to disfigurement. The lesions can also join together. […] This is a rapidly progressive type of morphea with many plaques that can cover almost your entire body. It spares hands and feet. This type needs the most aggressive treatment.

• #22 Morphea – Therapeutics in Dermatology

  https://www.therapeutique-dermatologique.org/spip.php?article1641

  Morphea tends to develop slowly. The size of the lesions varies and usually they are oval in shape. Initially, the affected areas can be itchy, uncomfortable or numb. The lesions usually appear as bruise-like pink areas in the skin. Sometimes the affected areas of skin turn slightly darker and beige coloured. Over time, the affected areas become thicker and turn pale and waxy in the middle and the skin feels firmer than normal when pinched. In some areas, such as over a joint, mobility can be reduced. Hair is usually lost in the affected areas and the skin feels dry because the sweat is no longer produced. Rarely, fatty tissue and muscle under the lesions disappear and the skin becomes stuck onto the underlying bone. […] Generalized morphea normally begins insidiously on the trunk as plaque morphea. An individual lesion is indistinguishable from classic plaque morphea, except that it does not stop expanding. Multiple plaques rapidly join and can affect nearly the entire trunk, often only sparing the nipples. As the disease progresses, it may result in disabling constrictions that even cause difficulty in breathing due to impaired thorax mobility and inflammation of the intercostal muscles. This variant is usually persistent given its often limited response to treatment. […] In adult-onset morphea, localized morphea involves one to three affected areas. It is the most common subtype in adults and is unlikely to involve the muscle or bone. Generalized morphea presents as four or more areas of skin and subcutaneous involvement. Adults can also get linear morphea.

• #23 Morphea: progress to date and the road ahead

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8033330/

  Damage that results after active lesions progress to an inactive state include atrophy, pigment changes and functional impairment. Damage tends to remain stable or increase after successful management of active disease. Once lesions are clinically inactive, treatment centers around improving quality of life by addressing cosmetic and functional concerns. Sclerosis and atrophy due to morphea can lead to limb-length discrepancies, contractures, and limited range of motion. It is important to refer these patients to physical therapy, occupational therapy or specialties such as rheumatology or orthopedics early in order to reduce disability.

• #24 Morphea – Cleaver Dermatology

  https://www.cleaverdermatology.com/morph-ea

  Though your skin will likely soften and become less obviously discolored over time, some discoloration may last for years. […] Morphea that affects the arms and legs impairs joint mobility in up to one in five people with the condition. […] The pain may subside in the following months or years, even if discolored patches of skin remain. […] Sometimes new patches of hard, discolored skin making it appear that some patches have joined together. […] This more extensive condition is called generalized morphea. […] Rarely, morphea may be part of a more serious condition called progressive systemic scleroderma, which may affect your blood vessels and internal organs.

• #25 What Is New in Morphea—Narrative Review on Molecular Aspects and New Targeted Therapies

  https://www.mdpi.com/2077-0383/13/23/7134

  The only population-based study conducted by Peterson et al. in Olmsted County, Minnesota, USA, between 1960 and 1993 suggests that the incidence of morphea is approximately 0.4–2.7 cases/100,000. The disease is 2.6–6 times more common in women than in men. […] There are two peaks of morphea incidence, the first between 2 and 14 years of age and the second in adults between 40 and 50 years of age. […] Several forms of morphea have been described, each with different clinical manifestations and degrees of connective tissue involvement. […] In adults, the most common clinical form of morphea is the plaque-like variety (morphea en plaques, MEP), while in children it is the linear variety. […] The differential diagnosis of morphea is presented in Table 1. […] Depending on the clinical form of morphea, its activity or severity, the depth and extent of the lesions and the degree of tissue damage, local and systemic treatments are used in therapy. Decisions must take into account the chronic and recurrent nature of the disease and the presence of atrophy and extracutaneous complications, which can lead to significant disability.

• #26 Localised Scleroderma (Morphoea): Symptoms and treatment

  https://patient.info/skin-conditions/localised-scleroderma-morphoea-leaflet

  Localised scleroderma usually comes on gradually. Areas of the skin may be thickened and discoloured, and hair may be lost over the area of scleroderma. […] Plaque morphoea are oval patches that can range from 2-20 cm across. They start off mauve in colour, then gradually become white. Older patches may become brown. The surface is smooth, shiny and hairless. One to three plaques may develop in different areas of the skin. This type mainly affects adults and usually has no other symptoms or problems, although the affected skin can look unsightly. […] Superficial morphoea is similar to the plaque type. It is typically seen as symmetrical mauve-coloured patches, usually in skin folds of the groin, armpits or under breasts. This type is most commonly found in middle-aged women. […] Linear scleroderma usually occurs on an arm or leg of a child. It is a long, narrow area of thickened skin. In more severe cases the tissues just beneath the skin are affected which may cause scarring (contractures) underneath the skin.

• #27 Scleroderma

  https://www.nhs.uk/conditions/scleroderma/

  Localised scleroderma (morphoea) can affect both children and adults. […] It mostly affects the skin, causing 1 or more hard patches to develop. […] Exactly how the skin is affected depends on the type of localised scleroderma. […] There are 2 main types, called plaque and linear morphoea. […] Plaque morphoea causes 1 or more discoloured oval patches on the skin, which may be hairless and shiny. […] Linear morphoea causes thickened skin in lines mostly along the arms and legs of children, but also on the forehead and scalp. […] In systemic sclerosis, internal organs can be affected as well as the skin. […] Limited cutaneous systemic sclerosis can have other typical symptoms that include thickening of the skin over the hands, feet and face, red spots on the skin, hard lumps under the skin, heartburn and problems swallowing (dysphagia).

• #28 Localised Scleroderma (Morphoea): Symptoms and treatment

  https://patient.info/skin-conditions/localised-scleroderma-morphoea-leaflet

  En coup de sabre is a deep form of linear scleroderma affecting the scalp and temple. The name comes from its shape, meaning 'like a sword cut’. Hair is lost over the affected skin, and the skull bone may be shrunk underneath it. […] This is a rare type where there are more plaques spread around different areas of the body. […] Limited plaque morphoea tends to improve with time, often over 3-5 years, but some cases may last for many years with new lesions developing as old one’s fade. Some improvement in generalised plaque morphoea is usually seen over a few years, although the condition may last for many years. […] Linear-type localised scleroderma tends to persist for longer but may improve after several years. It may come and go and may flare up after a long time of being settled. However, linear-type localised scleroderma, especially the en coup de sabre subtype, may become more extensive with time. This may cause severe contractures that result in limited movement and permanent disability of an arm or leg. Damage to underlying brain and eye tissue is a potential, serious complication of the en coup de sabre subtype.

• #29 Morphea: Symptoms, Causes, and Treatments

  https://www.healthline.com/health/morphea

  Linear morphea features a single band of thickened, discolored skin. Usually, the indented band runs down an arm or leg, but it might also extend down your forehead. This is referred to as en coup de sabre because it makes the skin look as though its been struck by a sword. […] Linear morphea is the most common type of morphea found in school-aged children. The lesions may extend to the tissue under their skin, even to their muscles and bones, leading to deformities. If linear morphea occurs on their face, it might cause issues with their eyes or alignment of their teeth. […] Morphea with deep lesions, lesions on your face or neck, or widespread lesions can lead to restricted joint mobility, joint pain, cosmetic deformities, permanent eye damage in children, and hair loss. […] Most cases of morphea slowly go away on their own over time and dont alter a persons life expectancy. On average, a lesion lasts three to five years, but discoloration might persist for a few more years. Occasionally, people will develop new lesions later on. […] Linear and deep morphea can interfere with the growth of children and lead to further problems, including differences in limb length, stiff and weak limbs, eye damage, and even death, but this is considered quite rare.

• #30 Morphea (Scleroderma): Treatment, Causes, and More

  https://www.verywellhealth.com/morphea-5179411

  In linear morphea, you don’t see round areas but rather linear streaks of hardened tissue. This usually happens just on one side of the body, most commonly on the extremities, face, or scalp. […] Sometimes linear morphea just affects the outermost layer of skin, the epidermis. But sometimes linear extends into the deeper skin (dermis) and even deeper into a layer called fascia. Sometimes it even extends into the muscles and joints. Because of this, it can cause some additional health problems, like the following: Limiting joint movement (if the morphea connects with a joint), Muscle contractures (tightening of the muscles and joints that can cause deformity and pain), Differences in limb length (from constricted growth) and changes in gait, Arthritis and muscle aches. […] There is also a dangerous form of linear morphea in which areas of the head are affected, causing hair loss and a depressed groove. This is called linear morphea en coup de sabre, French for the blow of a sword. Depending on where this groove is, it can cause serious issues, such as seizures and problems with the teeth, jaw, nose, lips, and eyes.

• #31 Morphea: Symptoms, Causes, and Treatments

  https://www.healthline.com/health/morphea

  Linear morphea features a single band of thickened, discolored skin. Usually, the indented band runs down an arm or leg, but it might also extend down your forehead. This is referred to as en coup de sabre because it makes the skin look as though its been struck by a sword. […] Linear morphea is the most common type of morphea found in school-aged children. The lesions may extend to the tissue under their skin, even to their muscles and bones, leading to deformities. If linear morphea occurs on their face, it might cause issues with their eyes or alignment of their teeth. […] Morphea with deep lesions, lesions on your face or neck, or widespread lesions can lead to restricted joint mobility, joint pain, cosmetic deformities, permanent eye damage in children, and hair loss. […] Most cases of morphea slowly go away on their own over time and dont alter a persons life expectancy. On average, a lesion lasts three to five years, but discoloration might persist for a few more years. Occasionally, people will develop new lesions later on. […] Linear and deep morphea can interfere with the growth of children and lead to further problems, including differences in limb length, stiff and weak limbs, eye damage, and even death, but this is considered quite rare.

• #32 Morphea (Scleroderma): Treatment, Causes, and More

  https://www.verywellhealth.com/morphea-5179411

  In linear morphea, you don’t see round areas but rather linear streaks of hardened tissue. This usually happens just on one side of the body, most commonly on the extremities, face, or scalp. […] Sometimes linear morphea just affects the outermost layer of skin, the epidermis. But sometimes linear extends into the deeper skin (dermis) and even deeper into a layer called fascia. Sometimes it even extends into the muscles and joints. Because of this, it can cause some additional health problems, like the following: Limiting joint movement (if the morphea connects with a joint), Muscle contractures (tightening of the muscles and joints that can cause deformity and pain), Differences in limb length (from constricted growth) and changes in gait, Arthritis and muscle aches. […] There is also a dangerous form of linear morphea in which areas of the head are affected, causing hair loss and a depressed groove. This is called linear morphea en coup de sabre, French for the blow of a sword. Depending on where this groove is, it can cause serious issues, such as seizures and problems with the teeth, jaw, nose, lips, and eyes.

• #33 Morphea (Scleroderma): Treatment, Causes, and More

  https://www.verywellhealth.com/morphea-5179411

  In linear morphea, you don’t see round areas but rather linear streaks of hardened tissue. This usually happens just on one side of the body, most commonly on the extremities, face, or scalp. […] Sometimes linear morphea just affects the outermost layer of skin, the epidermis. But sometimes linear extends into the deeper skin (dermis) and even deeper into a layer called fascia. Sometimes it even extends into the muscles and joints. Because of this, it can cause some additional health problems, like the following: Limiting joint movement (if the morphea connects with a joint), Muscle contractures (tightening of the muscles and joints that can cause deformity and pain), Differences in limb length (from constricted growth) and changes in gait, Arthritis and muscle aches. […] There is also a dangerous form of linear morphea in which areas of the head are affected, causing hair loss and a depressed groove. This is called linear morphea en coup de sabre, French for the blow of a sword. Depending on where this groove is, it can cause serious issues, such as seizures and problems with the teeth, jaw, nose, lips, and eyes.

• #34 Morphea – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559010/

  Linear morphea is characterized by the presence of band-like lesions, which usually manifest over the head and neck or the limbs. The sclerosis is localized to the dermis but often has a protracted course and is the most common type in childhood-onset morphea. It can involve deeper tissues causing complications like muscle weakness, joint contractures, and limb deformities in children. […] Generalized morphea is a severe form of plaque morphea characterized by multiple lesions or coalescence of individual plaques. It is predominant in females, and the lesions are frequently deep and associated with limited range of motion in underlying joints. Generalized morphea is defined by the presence of more than four lesions in at least two different anatomic locations. […] Patches of morphea that are superficial may be relatively benign, and the prognosis is good. Still, some variants of morphea tend to involve subcutaneous tissue, muscle tissue, and bone, resulting in functional disabilities and cosmetic problems. Many children with linear morphea can develop joint contractures and limb length discrepancies.

• #35 Morphea (Scleroderma): Treatment, Causes, and More

  https://www.verywellhealth.com/morphea-5179411

  In linear morphea, you don’t see round areas but rather linear streaks of hardened tissue. This usually happens just on one side of the body, most commonly on the extremities, face, or scalp. […] Sometimes linear morphea just affects the outermost layer of skin, the epidermis. But sometimes linear extends into the deeper skin (dermis) and even deeper into a layer called fascia. Sometimes it even extends into the muscles and joints. Because of this, it can cause some additional health problems, like the following: Limiting joint movement (if the morphea connects with a joint), Muscle contractures (tightening of the muscles and joints that can cause deformity and pain), Differences in limb length (from constricted growth) and changes in gait, Arthritis and muscle aches. […] There is also a dangerous form of linear morphea in which areas of the head are affected, causing hair loss and a depressed groove. This is called linear morphea en coup de sabre, French for the blow of a sword. Depending on where this groove is, it can cause serious issues, such as seizures and problems with the teeth, jaw, nose, lips, and eyes.

• #36 Morphoea en coup de sabre

  https://dermnetnz.org/topics/morphoea-en-coup-de-sabre

  Morphoea en coup de sabre is a variant of linear morphoea (a localised form of scleroderma) restricted to the frontoparietal region (forehead). Morphoea en coup de sabre also can cut into the brain, causing neurological abnormalities and vision problems. […] Morphoea en coup de sabre mostly occurs in children (67%), with a slight predominance in girls (about 2:1). It usually first appears in the first two decades of life. […] The clinical features of morphoea en coup de sabre evolve over months to years. En coup de sabre begins with a slightly hyperpigmented or hypopigmented streak on the forehead. […] The complications of morphoea en coup de sabre include: Hair loss, Scalp atrophy or atrophy of the underlying bone, Progressive hemifacial atrophy (Parry-Romberg syndrome), Ipsilateral eye problems (structural and visual abnormalities), Neurological abnormalities (eg, seizures and headaches), Facial deformity, Gum, tongue, and other dental problems, Reduced self-esteem.

• #37 Morphea: a practical review of its diagnosis, classification and treatment

  http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S0016-38132019000500483

  Generalized morphea is a severe form of the disease, defined as four or more plaques larger than 3 cm that converge and involve more than two anatomical sites. It occurs in 7 to 9 % of patients with morphea and is characterized by rapid plaque spread. […] Pan-sclerotic morphea (also called childhood disabling morphea) is an aggressive and mutilating variant that most often affects children. Presentation is rapidly progressive and deteriorates deep structures such as muscles, tendons and bone by generating joint contractures, painful ulcerations and calcifications that lead to significant disability. […] The prognosis of circumscribed or superficial morphea is good, although the disease shows a tendency towards progression. Generally, the disease is inactivated in between three and five years, but approximately 30 % of patients will experience relapses, especially if morphea started in childhood and if treatment was not adequate. The linear and deep forms can leave important sequelae with functional and esthetic disability.

• #38 Morphea – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559010/

  Linear morphea is characterized by the presence of band-like lesions, which usually manifest over the head and neck or the limbs. The sclerosis is localized to the dermis but often has a protracted course and is the most common type in childhood-onset morphea. It can involve deeper tissues causing complications like muscle weakness, joint contractures, and limb deformities in children. […] Generalized morphea is a severe form of plaque morphea characterized by multiple lesions or coalescence of individual plaques. It is predominant in females, and the lesions are frequently deep and associated with limited range of motion in underlying joints. Generalized morphea is defined by the presence of more than four lesions in at least two different anatomic locations. […] Patches of morphea that are superficial may be relatively benign, and the prognosis is good. Still, some variants of morphea tend to involve subcutaneous tissue, muscle tissue, and bone, resulting in functional disabilities and cosmetic problems. Many children with linear morphea can develop joint contractures and limb length discrepancies.

• #39 Morphea: Symptoms, Causes, and Treatments

  https://www.healthline.com/health/morphea

  Morphea predominately affects your skin. It doesnt involve your internal organs. In most cases, it resolves on its own, but you can experience a relapse. […] More severe forms can lead to cosmetic deformities, and it occasionally affects the muscle, joints, or bone. […] In general, morphea causes discolored, thickened patches of skin that are oval in shape. The outer edge of the lesion may be lilac, and the patch itself is usually reddish in color. It gradually becomes white or yellow in color toward the center of the oval. […] This involves multiple widespread lesions that are larger. Generalized morphea can affect deeper tissues, which might lead to disfigurement. The lesions can also join together. […] This is a rapidly progressive type of morphea with many plaques that can cover almost your entire body. It spares hands and feet. This type needs the most aggressive treatment.

• #40 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Patients with generalized morphea are more likely to feel tired and to have joint pain than other variants of morphea. […] Patients with generalized morphea may experience fatigue and joint pain. Patients with generalized and pansclerotic morphea may experience difficulty with deep breathing, if all of the skin on their chest is involved. If a limb is involved, children with linear morphea may have shortening of the involved limb. Linear morphea involving the scalp and face may develop seizures and neurologic problems. Seizures and neurological deficits may also complicate progressive facial hemiatrophy. Children with linear morphea of the head may also have eye involvement and should see an ophthalmologist. […] For the vast majority of patients with morphea, the condition will burn out with or without treatment in 3 to 5 years. This means that no new lesions will develop, existing lesions will not grow, and any red or purple discoloration around the lesions will turn brown (postinflammatory hyperpigmentation). The vast majority of patients are responsive to therapy be it creams, light therapy or systemic immunosuppressive drugs such as methotrexate.

• #41 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Patients with generalized morphea are more likely to feel tired and to have joint pain than other variants of morphea. […] Patients with generalized morphea may experience fatigue and joint pain. Patients with generalized and pansclerotic morphea may experience difficulty with deep breathing, if all of the skin on their chest is involved. If a limb is involved, children with linear morphea may have shortening of the involved limb. Linear morphea involving the scalp and face may develop seizures and neurologic problems. Seizures and neurological deficits may also complicate progressive facial hemiatrophy. Children with linear morphea of the head may also have eye involvement and should see an ophthalmologist. […] For the vast majority of patients with morphea, the condition will burn out with or without treatment in 3 to 5 years. This means that no new lesions will develop, existing lesions will not grow, and any red or purple discoloration around the lesions will turn brown (postinflammatory hyperpigmentation). The vast majority of patients are responsive to therapy be it creams, light therapy or systemic immunosuppressive drugs such as methotrexate.

• #42 Morphea: a practical review of its diagnosis, classification and treatment

  http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S0016-38132019000500483

  Generalized morphea is a severe form of the disease, defined as four or more plaques larger than 3 cm that converge and involve more than two anatomical sites. It occurs in 7 to 9 % of patients with morphea and is characterized by rapid plaque spread. […] Pan-sclerotic morphea (also called childhood disabling morphea) is an aggressive and mutilating variant that most often affects children. Presentation is rapidly progressive and deteriorates deep structures such as muscles, tendons and bone by generating joint contractures, painful ulcerations and calcifications that lead to significant disability. […] The prognosis of circumscribed or superficial morphea is good, although the disease shows a tendency towards progression. Generally, the disease is inactivated in between three and five years, but approximately 30 % of patients will experience relapses, especially if morphea started in childhood and if treatment was not adequate. The linear and deep forms can leave important sequelae with functional and esthetic disability.

• #43 Morphea: a practical review of its diagnosis, classification and treatment

  http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S0016-38132019000500483

  Generalized morphea is a severe form of the disease, defined as four or more plaques larger than 3 cm that converge and involve more than two anatomical sites. It occurs in 7 to 9 % of patients with morphea and is characterized by rapid plaque spread. […] Pan-sclerotic morphea (also called childhood disabling morphea) is an aggressive and mutilating variant that most often affects children. Presentation is rapidly progressive and deteriorates deep structures such as muscles, tendons and bone by generating joint contractures, painful ulcerations and calcifications that lead to significant disability. […] The prognosis of circumscribed or superficial morphea is good, although the disease shows a tendency towards progression. Generally, the disease is inactivated in between three and five years, but approximately 30 % of patients will experience relapses, especially if morphea started in childhood and if treatment was not adequate. The linear and deep forms can leave important sequelae with functional and esthetic disability.

• #44 Morphea: Symptoms, Causes, and Treatments

  https://www.healthline.com/health/morphea

  Morphea predominately affects your skin. It doesnt involve your internal organs. In most cases, it resolves on its own, but you can experience a relapse. […] More severe forms can lead to cosmetic deformities, and it occasionally affects the muscle, joints, or bone. […] In general, morphea causes discolored, thickened patches of skin that are oval in shape. The outer edge of the lesion may be lilac, and the patch itself is usually reddish in color. It gradually becomes white or yellow in color toward the center of the oval. […] This involves multiple widespread lesions that are larger. Generalized morphea can affect deeper tissues, which might lead to disfigurement. The lesions can also join together. […] This is a rapidly progressive type of morphea with many plaques that can cover almost your entire body. It spares hands and feet. This type needs the most aggressive treatment.

• #45 Morphea: progress to date and the road ahead

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8033330/

  Morphea is a rare autoimmune condition causing inflammation and sclerosis of the skin and underlying soft tissue. It is characterized by periods of activity (inflammation admixed with fibrosis), ultimately resulting in permanent damage (pigment change and tissue loss). Damage resulting from unchecked activity can lead to devastating, permanent cosmetic and functional sequelae including hair loss; cutaneous, soft tissue and bony atrophy; joint contractures; and growth restriction of the affected body site in children. This makes the early identification of activity and initiation of appropriate treatment crucial to limiting damage in morphea. […] Morphea is characterized by relapsing and remitting periods of activity, marked by inflammation and fibrosis, and damage which produces atrophy. Unchecked disease activity in morphea can lead to permanent deformity and functional impairment, and thus early diagnosis and treatment are imperative to minimize damage.

• #46 Morphea: progress to date and the road ahead

  https://atm.amegroups.org/article/view/61306/html

  Morphea is a rare autoimmune condition causing inflammation and sclerosis of the skin and underlying soft tissue. It is characterized by periods of activity (inflammation admixed with fibrosis), ultimately resulting in permanent damage (pigment change and tissue loss). […] Damage resulting from unchecked activity can lead to devastating, permanent cosmetic and functional sequelae including hair loss; cutaneous, soft tissue and bony atrophy; joint contractures; and growth restriction of the affected body site in children. This makes the early identification of activity and initiation of appropriate treatment crucial to limiting damage in morphea. […] Morphea is characterized by relapsing and remitting periods of activity, marked by inflammation and fibrosis, and damage which produces atrophy. Unchecked disease activity in morphea can lead to permanent deformity and functional impairment, and thus early diagnosis and treatment are imperative to minimize damage.

• #47

  https://www.aboutkidshealth.ca/morphea

  Morphea is an uncommon condition that causes skin to harden and look bruised. […] The active inflammation of morphea stops after several years. […] Morphea appears as patches called „plaques” or „lesions” on the skin. […] As morphea progresses, the affected skin may change. The veins under the skin may show more clearly. The affected skin may look thinner and may look raised or sunken compared to the skin around it. […] Morphea is unpredictable. Most children have the disease for months or years before it is recognized or diagnosed. Even without treatment, morphea stops being active and usually „burns out” after three to five years. […] The disease has three phases: an early inflammatory phase that presents as nonspecific redness, a second inflammatory phase, which typically presents with a hardened middle of white discolouration with a red-purple border, and a burnt-out phase, appearing as increased colour, thinning of the skin with more visible vessels or sunken skin layers (due to loss of fat).

• #48 Morphea – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/symptoms-causes/syc-20375283

  Morphea affects the skin and underlying tissue and sometimes bone. The condition generally lasts several years and then improves or at times disappears by itself. It may leave scars or areas of darkened or discolored skin. It is possible for morphea to recur. […] Morphea can cause a number of complications, including: Movement problems. Morphea that affects the arms or legs can impair joint mobility. […] The condition generally lasts several years and then improves or at times disappears by itself.

• #49

  https://www.aboutkidshealth.ca/morphea

  Morphea is an uncommon condition that causes skin to harden and look bruised. […] The active inflammation of morphea stops after several years. […] Morphea appears as patches called „plaques” or „lesions” on the skin. […] As morphea progresses, the affected skin may change. The veins under the skin may show more clearly. The affected skin may look thinner and may look raised or sunken compared to the skin around it. […] Morphea is unpredictable. Most children have the disease for months or years before it is recognized or diagnosed. Even without treatment, morphea stops being active and usually „burns out” after three to five years. […] The disease has three phases: an early inflammatory phase that presents as nonspecific redness, a second inflammatory phase, which typically presents with a hardened middle of white discolouration with a red-purple border, and a burnt-out phase, appearing as increased colour, thinning of the skin with more visible vessels or sunken skin layers (due to loss of fat).

• #50 Morphoea en coup de sabre

  https://dermnetnz.org/topics/morphoea-en-coup-de-sabre

  The goal of treatment is to prevent progression of the en coup de sabre. […] The natural history of morphoea en coup de sabre is to progress slowly over many years, then to be self-limiting. There are three phases. […] The active phase of morphoea en coup de sabre features areas of active sclerosis with erythematous or violaceous borders, with or without expansion of the plaque. […] The regression phase of morphoea en coup de sabre retains persistent sclerosis without erythema or a violaceous border. […] The burnt-out phase of morphoea en coup de sabre demonstrates no sclerosis and no erythema. The burnt-out phase signals that the course of en coup de sabre is complete, and no further damage will occur.

• #51

  https://www.aboutkidshealth.ca/morphea

  Some patients may have disease activity beyond the three to five years that requires longer-term treatment. […] When the disease becomes inactive, the affected skin may become darker than the rest of the skin. Additionally, hardening of the skin may leave permanent scarring. Loss of fat may make the skin appear thinner with more visible vessels. […] The active inflammation of morphea may last three to five years, even if not treated. Most children recover very well.

• #52 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Patients with generalized morphea are more likely to feel tired and to have joint pain than other variants of morphea. […] Patients with generalized morphea may experience fatigue and joint pain. Patients with generalized and pansclerotic morphea may experience difficulty with deep breathing, if all of the skin on their chest is involved. If a limb is involved, children with linear morphea may have shortening of the involved limb. Linear morphea involving the scalp and face may develop seizures and neurologic problems. Seizures and neurological deficits may also complicate progressive facial hemiatrophy. Children with linear morphea of the head may also have eye involvement and should see an ophthalmologist. […] For the vast majority of patients with morphea, the condition will burn out with or without treatment in 3 to 5 years. This means that no new lesions will develop, existing lesions will not grow, and any red or purple discoloration around the lesions will turn brown (postinflammatory hyperpigmentation). The vast majority of patients are responsive to therapy be it creams, light therapy or systemic immunosuppressive drugs such as methotrexate.

• #53 Morphea: a practical review of its diagnosis, classification and treatment

  http://www.scielo.org.mx/scielo.php?script=sci_arttext&pid=S0016-38132019000500483

  Generalized morphea is a severe form of the disease, defined as four or more plaques larger than 3 cm that converge and involve more than two anatomical sites. It occurs in 7 to 9 % of patients with morphea and is characterized by rapid plaque spread. […] Pan-sclerotic morphea (also called childhood disabling morphea) is an aggressive and mutilating variant that most often affects children. Presentation is rapidly progressive and deteriorates deep structures such as muscles, tendons and bone by generating joint contractures, painful ulcerations and calcifications that lead to significant disability. […] The prognosis of circumscribed or superficial morphea is good, although the disease shows a tendency towards progression. Generally, the disease is inactivated in between three and five years, but approximately 30 % of patients will experience relapses, especially if morphea started in childhood and if treatment was not adequate. The linear and deep forms can leave important sequelae with functional and esthetic disability.

• #54 Morphea: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1065782-overview

  Morphea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissues, or both. Morphea can present with extracutaneous manifestations, including fever, lymphadenopathy, arthralgias, fatigue, and central nervous system involvement, as well as laboratory abnormalities, including eosinophilia, polyclonal hypergammaglobulinemia, and positive antinuclear antibody (ANA) titers. […] Initial presentation with generalized morphea and/or positive baseline ANA titer is associated with a poorer prognosis, worsening of disease, or a relapsing-remitting course. […] Linear lesions tend to persist for longer than plaque-type lesions, but they often improve over the years. However, linear morphea, including the en coup de sabre subtype, may remit and reactivate, remain unchanged, or become more extensive with time. Linear morphea also has a higher rate of relapse compared with other variants. In addition, patients with linear lesions may develop limb atrophy and contractures that result in limited movement and permanent disability.

• #55 Morphea: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1065782-overview

  Pansclerotic morphea of children is a rare, aggressive, and mutilating variant of deep morphea that begins before age 14 years and has a disease course of relentless progression and severe disability. […] Disease activity in patients with morphea tends to improve with treatment; however, long-term monitoring is necessary, as the disease course can wax and wane, particularly in those with the generalized subtype. […] Morphea typically has a benign, self-limited course. However, linear and deep morphea subtypes can cause considerable morbidity, especially in children, when they interfere with growth. Joint contractures, limb-length discrepancy, and prominent facial atrophy result in substantial disability and deformity in a quarter to half of all patients with linear or deep morphea.

• #56 Morphea in Childhood: An Update | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-morphea-in-childhood-an-update-articulo-S157821901830088X

  The classification proposed by PReS also includes the term mixed morphea (MM), a variant that appears to be more common than previously believed. This decision indicates a shift towards viewing morphea as a term encompassing a spectrum of clinical variants characterized by inflammation and fibrosis occurring at different depths. […] Extracutaneous manifestations are observed in up to 40% of patients with morphea. […] Although prognosis in patients with morphea has been little studied and long-term studies are particularly scarce, the available evidence suggests that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae. In the short and medium term, systemic treatment with MTX and/or corticosteroid therapy appears to stop the progression of the disease and/or improve lesions. However, the condition recurs in between 28% and 46% of patients within 20 months of cessation of treatment. Activity persists in between 28% and 89% of patients in the long term. In a survey of patients with LM conducted after follow-up of up to 20 years, all but one of the respondents reported having residual damage. This included cosmetic sequelae in 66% and functional limitations in 38%. In total, 51% of those surveyed considered that the treatment they had received had been effective. Finally, individuals who develop morphea in early childhood may have decreased quality of life and be more likely to develop autoimmune conditions in later life.

• #57 Morphea in Childhood: An Update | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-morphea-in-childhood-an-update-articulo-S157821901830088X

  The classification proposed by PReS also includes the term mixed morphea (MM), a variant that appears to be more common than previously believed. This decision indicates a shift towards viewing morphea as a term encompassing a spectrum of clinical variants characterized by inflammation and fibrosis occurring at different depths. […] Extracutaneous manifestations are observed in up to 40% of patients with morphea. […] Although prognosis in patients with morphea has been little studied and long-term studies are particularly scarce, the available evidence suggests that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae. In the short and medium term, systemic treatment with MTX and/or corticosteroid therapy appears to stop the progression of the disease and/or improve lesions. However, the condition recurs in between 28% and 46% of patients within 20 months of cessation of treatment. Activity persists in between 28% and 89% of patients in the long term. In a survey of patients with LM conducted after follow-up of up to 20 years, all but one of the respondents reported having residual damage. This included cosmetic sequelae in 66% and functional limitations in 38%. In total, 51% of those surveyed considered that the treatment they had received had been effective. Finally, individuals who develop morphea in early childhood may have decreased quality of life and be more likely to develop autoimmune conditions in later life.

• #58 Morphea in Childhood: An Update | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-morphea-in-childhood-an-update-articulo-S157821901830088X

  The classification proposed by PReS also includes the term mixed morphea (MM), a variant that appears to be more common than previously believed. This decision indicates a shift towards viewing morphea as a term encompassing a spectrum of clinical variants characterized by inflammation and fibrosis occurring at different depths. […] Extracutaneous manifestations are observed in up to 40% of patients with morphea. […] Although prognosis in patients with morphea has been little studied and long-term studies are particularly scarce, the available evidence suggests that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae. In the short and medium term, systemic treatment with MTX and/or corticosteroid therapy appears to stop the progression of the disease and/or improve lesions. However, the condition recurs in between 28% and 46% of patients within 20 months of cessation of treatment. Activity persists in between 28% and 89% of patients in the long term. In a survey of patients with LM conducted after follow-up of up to 20 years, all but one of the respondents reported having residual damage. This included cosmetic sequelae in 66% and functional limitations in 38%. In total, 51% of those surveyed considered that the treatment they had received had been effective. Finally, individuals who develop morphea in early childhood may have decreased quality of life and be more likely to develop autoimmune conditions in later life.

• #59

  https://link.springer.com/article/10.1007/s00403-022-02478-1

  The average time on treatment was 22.2 months18.8 months. The median time from onset of disease to treatment was 2.5 years, ranging from 0 to 25 years. Of the 24 patients who underwent systemic treatment, 17 (70.8%) had improved or stable disease upon completion of any systemic treatment, 3 (12.5%) had progressive disease, and 4 (16.7%) were intolerant of the initial treatment agent and discontinued systemic treatment. […] Among patients who received MTX (n=16), 11 patients (68.8%) had improved or stable disease upon completion of treatment, 1 patient (6.3%) had progressive disease, and 4 patients (25.0%) did not tolerate MTX. […] Of all 34 patients, 25 (73.5%) patients were stable or in remission at their most recent clinic visit. […] Similar to prior findings, a significant portion (41.7%) of patients who underwent systemic treatment ultimately had recurrence of disease activity.

• #60 Coexistence of Recurrent Generalized Morphea and Systemic Sclerosis | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-0645

  Systemic sclerosis (SSc) is a generalized disorder of the connective tissue in which there is thickening of dermal collagen bundles, fibrosis and vascular abnormalities in the visceral organs. Generalized morphea (GM) is characterized by indurated plaques with widespread pigmentary change. […] However, there have been several reports that suggest a relationship between the two disorders, e.g. coexistence of morphea and SSc, and the progression from morphea to SSc. […] We diagnosed the patient with a coexistence of morphea and SSc. […] His symptoms responded well to this therapy and the dosage of prednisolone was tapered to 5 mg/day, taken for a period of 24 months. […] In January 2002 he experienced sclerodermatous plaques on his chest and abdomen. […] A recurrence of morphea was diagnosed.

• #61 Coexistence of Recurrent Generalized Morphea and Systemic Sclerosis | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-0645

  The disease activity was evaluated as similar to the previous episode, and he was subsequently treated with oral prednisolone 30 mg/day. […] Although no recurrence was found during a follow-up 24 months later, a recurrence of morphea on his trunk appeared suddenly in May 2008. […] An unusual manifestation of this case is the recurrence of morphea lesions. […] The recurrence of morphea in our case was less frequent than in the reported cases. […] These findings suggest that the prevention of palindromic or recurrent morphea requires a maintenance dose of steroids for an extended period of time.

• #62 Morphea – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559010/

  Morphea lesions can cause cosmetic sequelae if untreated, but internal organ involvement is rare. Linear morphea in children can cause severe atrophy of the extremities with joint contractures and limb length discrepancies. In the en coup de sabre variant, the inflammation and sclerosis involve deeper tissues and cause hemiatrophy of the face and facial deformity.

• #63 Morphea – Cleaver Dermatology

  https://www.cleaverdermatology.com/morph-ea

  Morphea usually affects only the uppermost layers of your skin, but in some cases may involve fatty or connective tissue below your skin. Morphea is usually chronic or recurrent. […] Hardening of the skin […] Thickening of the skin […] Discoloration of the affected skin to look lighter or darker than the surrounding area. Most people develop one or two oval-shaped patches that start out red, purple (lilac-colored) or yellowish and gradually develop a whitish center. Over time, the patches may turn brownish in color and eventually white. […] The most common complication of morphea is a possible negative effect on your self-esteem and body image if discolored patches of skin appear on your arms, legs and face. […] If your face is involved, your skin may appear depressed and is colored.

• #64 Morphea – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/morphea/diagnosis-treatment/drc-20375290

  Your doctor may diagnose morphea by examining the affected skin and asking about your signs and symptoms. […] Morphea usually lasts several years and then goes away without treatment. It may leave scars or areas of darkened or discolored skin. […] You might undergo ultrasound and magnetic resonance imaging to monitor disease progression and your response to treatment. […] Because morphea affects your appearance, it can be an especially difficult condition to live with. You may also be concerned that it will get worse before it goes away.

• #65 Morphea: Evidence-based recommendations for treatment – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/morphea-evidence-based-recommendations-for-treatment/

  Morphea is a rare fibrosing disorder of the skin that may also involve the underlying muscle, connective tissue, bone and brain. […] Morphea typically goes through two stages: an active (inflammatory) stage and a „burnt out” stage. Treatment is targeted at the active phase, in the hope of stabilizing the size of current lesions and preventing the occurrence of new lesions. Anti-inflammatory and immunosuppressive agents are unlikely to improve the burnt-out phase of morphea and, therefore, should not be used for this phase of the disease. […] Patients with linear morphea are at risk for facial deformity, limb length discrepancy and contractures. […] Patients need to recognize that the involved skin will never look completely normal. They must be counseled that treatment is aimed at active disease in the hopes of preventing enlargement of already-present lesions and the development of new lesions. Burnt-out disease is unlikely to improve with immunosuppression and, therefore, the risks of these medications are not warranted in burnt-out disease.

• #66 Morphea (Localized Scleroderma) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/morphea-localized-scleroderma/

  Morphea causes functional, psychologic, and neurologic complications. Morphea in children may cause growth retardation and muscular atrophy of affected limbs as well as limitation of range of motion and contracture, especially in deep lesions spanning joints or affecting the majority of the surface area of an extremity. […] Pain, sometimes severe, is reported in patients with deep involvement or circumferential involvement of an extremity. […] Pruritus, numbness, and tingling are common complaints and may be a marker of disease activity. […] Lesions often appear on cosmetically sensitive sites and may produce permanent damage manifested by cutaneous or subcutaneous atrophy or pigmentary change. […] Facial morphea linear lesions (eg, en coup de sabre lesions) may be associated with behavioral changes, learning disabilities and epilepsy.

• #67 Morphea – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559010/

  Morphea lesions can cause cosmetic sequelae if untreated, but internal organ involvement is rare. Linear morphea in children can cause severe atrophy of the extremities with joint contractures and limb length discrepancies. In the en coup de sabre variant, the inflammation and sclerosis involve deeper tissues and cause hemiatrophy of the face and facial deformity.

• #68 Morphea | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25270

  Linear morphea is characterized by the presence of band-like lesions, which usually manifest over the head and neck or the limbs. The sclerosis is localized to the dermis but often has a protracted course and is the most common type in childhood-onset morphea. It can involve deeper tissues causing complications like muscle weakness, joint contractures, and limb deformities in children. […] Generalized morphea is a severe form of plaque morphea characterized by multiple lesions or coalescence of individual plaques. It is predominant in females, and the lesions are frequently deep and associated with limited range of motion in underlying joints. Generalized morphea is defined by the presence of more than four lesions in at least two different anatomic locations. […] Morphea lesions can cause cosmetic sequelae if untreated, but internal organ involvement is rare. Linear morphea in children can cause severe atrophy of the extremities with joint contractures and limb length discrepancies. In the en coup de sabre variant, the inflammation and sclerosis involve deeper tissues and cause hemiatrophy of the face and facial deformity.

• #69 Morphea – Cleaver Dermatology

  https://www.cleaverdermatology.com/morph-ea

  Though your skin will likely soften and become less obviously discolored over time, some discoloration may last for years. […] Morphea that affects the arms and legs impairs joint mobility in up to one in five people with the condition. […] The pain may subside in the following months or years, even if discolored patches of skin remain. […] Sometimes new patches of hard, discolored skin making it appear that some patches have joined together. […] This more extensive condition is called generalized morphea. […] Rarely, morphea may be part of a more serious condition called progressive systemic scleroderma, which may affect your blood vessels and internal organs.

• #70 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Patients with generalized morphea are more likely to feel tired and to have joint pain than other variants of morphea. […] Patients with generalized morphea may experience fatigue and joint pain. Patients with generalized and pansclerotic morphea may experience difficulty with deep breathing, if all of the skin on their chest is involved. If a limb is involved, children with linear morphea may have shortening of the involved limb. Linear morphea involving the scalp and face may develop seizures and neurologic problems. Seizures and neurological deficits may also complicate progressive facial hemiatrophy. Children with linear morphea of the head may also have eye involvement and should see an ophthalmologist. […] For the vast majority of patients with morphea, the condition will burn out with or without treatment in 3 to 5 years. This means that no new lesions will develop, existing lesions will not grow, and any red or purple discoloration around the lesions will turn brown (postinflammatory hyperpigmentation). The vast majority of patients are responsive to therapy be it creams, light therapy or systemic immunosuppressive drugs such as methotrexate.

• #71 Morphea – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK559010/

  Linear morphea is characterized by the presence of band-like lesions, which usually manifest over the head and neck or the limbs. The sclerosis is localized to the dermis but often has a protracted course and is the most common type in childhood-onset morphea. It can involve deeper tissues causing complications like muscle weakness, joint contractures, and limb deformities in children. […] Generalized morphea is a severe form of plaque morphea characterized by multiple lesions or coalescence of individual plaques. It is predominant in females, and the lesions are frequently deep and associated with limited range of motion in underlying joints. Generalized morphea is defined by the presence of more than four lesions in at least two different anatomic locations. […] Patches of morphea that are superficial may be relatively benign, and the prognosis is good. Still, some variants of morphea tend to involve subcutaneous tissue, muscle tissue, and bone, resulting in functional disabilities and cosmetic problems. Many children with linear morphea can develop joint contractures and limb length discrepancies.

• #72 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Autoimmune diseases occur when the immune system, which normally protects you from infections and cancer, makes a mistake and starts to damage your bodys own tissues. Morphea (syn. localized scleroderma) occurs as a result of autoimmune fibrosing (leather-like) injury to the skin and sometimes the underlying fat, muscle and bone. Morphea is sometimes referred to as localized scleroderma. However, despite their similar names, patients with morphea are not at increased risk of developing internal organ scleroderma involvement (systemic sclerosis). […] In morphea, the immune system mistakenly causes inflammation of the skin. This inflammation then causes fibrosis (hardening) of the skin and underlying tissues. There are several different types of morphea some of which characteristically begin in adults while others characteristically start in childhood.

• #73

  https://link.springer.com/article/10.1007/s00403-022-02478-1

  Parry Romberg Syndrome (PRS) and en coup de sabre (ECDS) are head variants of linear morphea with functional and structural implications. […] Twenty-six patients (76.5%) reported ocular, oral, and/or neurologic symptoms, and 8 (23.5%) had concomitant autoimmune/inflammatory conditions. […] In the head variant cohort, 26 patients (76.5%) had oral, ocular, or neurologic clinical symptoms or exam findings related to PRS/ECDS. Fifteen patients (44.1%) reported neurologic symptoms, such as headache, migraine, paresthesia, dysphagia, and contralateral extremity numbness. Ipsilateral ophthalmologic symptoms, including enophthalmos, retinal hemorrhage, and vision loss, were observed in 13 patients (38.2%). Ipsilateral oral findings were noted in 16 patients (47.1%), most commonly tongue hemiatrophy and gingival recession.

• #74

  https://link.springer.com/article/10.1007/s00403-022-02478-1

  Parry Romberg Syndrome (PRS) and en coup de sabre (ECDS) are head variants of linear morphea with functional and structural implications. […] Twenty-six patients (76.5%) reported ocular, oral, and/or neurologic symptoms, and 8 (23.5%) had concomitant autoimmune/inflammatory conditions. […] In the head variant cohort, 26 patients (76.5%) had oral, ocular, or neurologic clinical symptoms or exam findings related to PRS/ECDS. Fifteen patients (44.1%) reported neurologic symptoms, such as headache, migraine, paresthesia, dysphagia, and contralateral extremity numbness. Ipsilateral ophthalmologic symptoms, including enophthalmos, retinal hemorrhage, and vision loss, were observed in 13 patients (38.2%). Ipsilateral oral findings were noted in 16 patients (47.1%), most commonly tongue hemiatrophy and gingival recession.

• #75 Morphoea en coup de sabre

  https://dermnetnz.org/topics/morphoea-en-coup-de-sabre

  Morphoea en coup de sabre is a variant of linear morphoea (a localised form of scleroderma) restricted to the frontoparietal region (forehead). Morphoea en coup de sabre also can cut into the brain, causing neurological abnormalities and vision problems. […] Morphoea en coup de sabre mostly occurs in children (67%), with a slight predominance in girls (about 2:1). It usually first appears in the first two decades of life. […] The clinical features of morphoea en coup de sabre evolve over months to years. En coup de sabre begins with a slightly hyperpigmented or hypopigmented streak on the forehead. […] The complications of morphoea en coup de sabre include: Hair loss, Scalp atrophy or atrophy of the underlying bone, Progressive hemifacial atrophy (Parry-Romberg syndrome), Ipsilateral eye problems (structural and visual abnormalities), Neurological abnormalities (eg, seizures and headaches), Facial deformity, Gum, tongue, and other dental problems, Reduced self-esteem.

• #76

  https://link.springer.com/article/10.1007/s00403-022-02478-1

  Parry Romberg Syndrome (PRS) and en coup de sabre (ECDS) are head variants of linear morphea with functional and structural implications. […] Twenty-six patients (76.5%) reported ocular, oral, and/or neurologic symptoms, and 8 (23.5%) had concomitant autoimmune/inflammatory conditions. […] In the head variant cohort, 26 patients (76.5%) had oral, ocular, or neurologic clinical symptoms or exam findings related to PRS/ECDS. Fifteen patients (44.1%) reported neurologic symptoms, such as headache, migraine, paresthesia, dysphagia, and contralateral extremity numbness. Ipsilateral ophthalmologic symptoms, including enophthalmos, retinal hemorrhage, and vision loss, were observed in 13 patients (38.2%). Ipsilateral oral findings were noted in 16 patients (47.1%), most commonly tongue hemiatrophy and gingival recession.

• #77 Morphea symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/morphea

  Morphea, also known as localized scleroderma, is a rare disorder characterized by excessive collagen deposition leading to thickening of the skin, subcutaneous tissues, or both. It is classified into plaque, generalized, linear, and deep subtypes. […] Common symptom Fatigue […] 9 morphea patients report severe fatigue (33%) […] 13 morphea patients report moderate fatigue (48%) […] 4 morphea patients report mild fatigue (14%) […] 1 a morphea patient reports no fatigue (3%) […] Common symptom Stress […] 3 morphea patients report severe stress (33%) […] 1 a morphea patient reports moderate stress (11%) […] 5 morphea patients report mild stress (55%) […] 0 morphea patients report no stress (0%) […] Common symptom Pain […] 5 morphea patients report severe pain (18%) […] 12 morphea patients report moderate pain (44%)

• #78 Morphea symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/morphea

  7 morphea patients report mild pain (25%) […] 3 morphea patients report no pain (11%) […] Common symptom Depressed mood […] 2 morphea patients report severe depressed mood (8%) […] 5 morphea patients report moderate depressed mood (20%) […] 16 morphea patients report mild depressed mood (64%) […] 2 morphea patients report no depressed mood (8%) […] Common symptom Anxious mood […] 1 a morphea patient reports severe anxious mood (4%) […] 10 morphea patients report moderate anxious mood (41%) […] 8 morphea patients report mild anxious mood (33%) […] 5 morphea patients report no anxious mood (20%).

• #79 Morphea in Childhood: An Update | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-morphea-in-childhood-an-update-articulo-S157821901830088X

  The classification proposed by PReS also includes the term mixed morphea (MM), a variant that appears to be more common than previously believed. This decision indicates a shift towards viewing morphea as a term encompassing a spectrum of clinical variants characterized by inflammation and fibrosis occurring at different depths. […] Extracutaneous manifestations are observed in up to 40% of patients with morphea. […] Although prognosis in patients with morphea has been little studied and long-term studies are particularly scarce, the available evidence suggests that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae. In the short and medium term, systemic treatment with MTX and/or corticosteroid therapy appears to stop the progression of the disease and/or improve lesions. However, the condition recurs in between 28% and 46% of patients within 20 months of cessation of treatment. Activity persists in between 28% and 89% of patients in the long term. In a survey of patients with LM conducted after follow-up of up to 20 years, all but one of the respondents reported having residual damage. This included cosmetic sequelae in 66% and functional limitations in 38%. In total, 51% of those surveyed considered that the treatment they had received had been effective. Finally, individuals who develop morphea in early childhood may have decreased quality of life and be more likely to develop autoimmune conditions in later life.

• #80 Morphea: Symptoms, causes, and treatment

  https://www.medicalnewstoday.com/articles/320708

  Morphea is usually painless and will disappear on its own, but this may take several years. […] The symptoms of morphea differ depending on the stage and severity of the condition. […] Some common signs and symptoms include: patches of reddish or lilac skin, most commonly on the torso; linear patches of discolored skin on the arms or legs; patches of skin that gradually turn lighter or have a whitish center; patches of skin that gradually harden and have a thick, shiny appearance; hair loss in the affected area; loss of sweat glands in the affected area. […] The appearance of morphea usually only affects the skin and its underlying tissue, although it can also affect the bone and muscle. […] Morphea is a rare skin condition that will usually only affect the appearance of the skin and will go away without treatment. […] However, in more severe cases, morphea can cause mobility issues or deformities. […] In children, morphea can cause eye damage and problems with limb growth and movement.

• #81 Morphea: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1065782-overview

  Pansclerotic morphea of children is a rare, aggressive, and mutilating variant of deep morphea that begins before age 14 years and has a disease course of relentless progression and severe disability. […] Disease activity in patients with morphea tends to improve with treatment; however, long-term monitoring is necessary, as the disease course can wax and wane, particularly in those with the generalized subtype. […] Morphea typically has a benign, self-limited course. However, linear and deep morphea subtypes can cause considerable morbidity, especially in children, when they interfere with growth. Joint contractures, limb-length discrepancy, and prominent facial atrophy result in substantial disability and deformity in a quarter to half of all patients with linear or deep morphea.

• #82 Localised Scleroderma (Morphoea): Symptoms and treatment

  https://patient.info/skin-conditions/localised-scleroderma-morphoea-leaflet

  En coup de sabre is a deep form of linear scleroderma affecting the scalp and temple. The name comes from its shape, meaning 'like a sword cut’. Hair is lost over the affected skin, and the skull bone may be shrunk underneath it. […] This is a rare type where there are more plaques spread around different areas of the body. […] Limited plaque morphoea tends to improve with time, often over 3-5 years, but some cases may last for many years with new lesions developing as old one’s fade. Some improvement in generalised plaque morphoea is usually seen over a few years, although the condition may last for many years. […] Linear-type localised scleroderma tends to persist for longer but may improve after several years. It may come and go and may flare up after a long time of being settled. However, linear-type localised scleroderma, especially the en coup de sabre subtype, may become more extensive with time. This may cause severe contractures that result in limited movement and permanent disability of an arm or leg. Damage to underlying brain and eye tissue is a potential, serious complication of the en coup de sabre subtype.

• #83 Morphea: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1065782-overview

  Pansclerotic morphea of children is a rare, aggressive, and mutilating variant of deep morphea that begins before age 14 years and has a disease course of relentless progression and severe disability. […] Disease activity in patients with morphea tends to improve with treatment; however, long-term monitoring is necessary, as the disease course can wax and wane, particularly in those with the generalized subtype. […] Morphea typically has a benign, self-limited course. However, linear and deep morphea subtypes can cause considerable morbidity, especially in children, when they interfere with growth. Joint contractures, limb-length discrepancy, and prominent facial atrophy result in substantial disability and deformity in a quarter to half of all patients with linear or deep morphea.

• #84 Localised Scleroderma (Morphoea): Symptoms and treatment

  https://patient.info/skin-conditions/localised-scleroderma-morphoea-leaflet

  En coup de sabre is a deep form of linear scleroderma affecting the scalp and temple. The name comes from its shape, meaning 'like a sword cut’. Hair is lost over the affected skin, and the skull bone may be shrunk underneath it. […] This is a rare type where there are more plaques spread around different areas of the body. […] Limited plaque morphoea tends to improve with time, often over 3-5 years, but some cases may last for many years with new lesions developing as old one’s fade. Some improvement in generalised plaque morphoea is usually seen over a few years, although the condition may last for many years. […] Linear-type localised scleroderma tends to persist for longer but may improve after several years. It may come and go and may flare up after a long time of being settled. However, linear-type localised scleroderma, especially the en coup de sabre subtype, may become more extensive with time. This may cause severe contractures that result in limited movement and permanent disability of an arm or leg. Damage to underlying brain and eye tissue is a potential, serious complication of the en coup de sabre subtype.

• #85 Morphea: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1065782-overview

  Pansclerotic morphea of children is a rare, aggressive, and mutilating variant of deep morphea that begins before age 14 years and has a disease course of relentless progression and severe disability. […] Disease activity in patients with morphea tends to improve with treatment; however, long-term monitoring is necessary, as the disease course can wax and wane, particularly in those with the generalized subtype. […] Morphea typically has a benign, self-limited course. However, linear and deep morphea subtypes can cause considerable morbidity, especially in children, when they interfere with growth. Joint contractures, limb-length discrepancy, and prominent facial atrophy result in substantial disability and deformity in a quarter to half of all patients with linear or deep morphea.

• #86 Systemic lupus erythematosus

  https://diseases.autoimmuneregistry.org/disease_profile/morphea.aspx

  Morphea is a disorder primarily affecting the skin. There are several types of morphea: Localized or circumscribed morphea causes patients to develop oval-shaped areas of redness. Irritated skin may be limited to a single patch or to several patches covering a smaller area. Generalized morphea is more widespread, affecting a number of skin regions throughout the body. In linear morphea, skin irritation appears in a linear, rather than an oval shape. Damage can penetrate to the tissues below the skin, including muscle and bone. Pansclerotic morphea, the rarest and most severe form, usually affect children and involves the entire body, also penetrating through the skin into the bone. The cause of morphea is unknown but is believed to be autoimmune. There is no cure for morphea, but treatments are available to manage symptoms. Patches of irritation caused by mild types of morphea often improve in 3-5 years, though new patches may develop. However, damage affecting both the skin and the muscles/bones, as in linear morphea, may have a longer duration and occur in cycles of flares and remission, while pansclerotic morphea tends to worsen significantly with time. Both linear and pansclerotic morphea may cause severe disability. […] Symptoms: Pain, Oval-shaped patches, Facial pain, Red patches with light center, Headache, Hair loss, Vision problems, Decreased sweating, Dry eyes, Fatigue, Eye pain, Carpal tunnel syndrome, Joint pain, Difficulty swallowing, Muscle pain, Shortness of breath, Weakness, Seizures.

• #87 Juvenile Localized Scleroderma Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/14857-juvenile-localized-scleroderma

  Patches that form on your childs skin can decrease over time but usually need treatment. Patches can return (flare up) after theyve gone away. […] Juvenile localized scleroderma often affects skin tissues and doesnt harm major organs. At some point, your childs hard skin may soften and only hyperpigmented (darkened) areas may have symptoms. Old patches of skin affected by scleroderma can recur (come back), or new skin patches can develop. […] Some children may end up with hardened skin, while others may experience damage to their skin or limbs. Your child might need surgery if the condition didnt respond well to treatment. […] Your child will have the most positive outlook if their condition receives treatment early, when they first get their diagnosis.

• #88 What Is New in Morphea—Narrative Review on Molecular Aspects and New Targeted Therapies

  https://www.mdpi.com/2077-0383/13/23/7134

  The treatment of morphea remains a challenge for clinicians because of the poor clinical response to treatment. The main therapeutic options, depending on the type of morphea, include topical steroids, topical calcineurin inhibitors, imiquimod, and in more advanced cases methotrexate, systemic steroids, mycophenolate mofetil, and phototherapy (PUVA, UVA1, UVB). […] The earlier treatment starts, the less damage, such as limb deformity, will occur. […] For very active, widespread lesions, systemic treatment with corticosteroids (intravenous or oral) or methotrexate is required. The combination of systemic corticosteroids and methotrexate has the best effect in this group of patients and is the first-line treatment.

• #89 Morphea: Evidence-based recommendations for treatment – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/morphea-evidence-based-recommendations-for-treatment/

  Morphea is a rare fibrosing disorder of the skin that may also involve the underlying muscle, connective tissue, bone and brain. […] Morphea typically goes through two stages: an active (inflammatory) stage and a „burnt out” stage. Treatment is targeted at the active phase, in the hope of stabilizing the size of current lesions and preventing the occurrence of new lesions. Anti-inflammatory and immunosuppressive agents are unlikely to improve the burnt-out phase of morphea and, therefore, should not be used for this phase of the disease. […] Patients with linear morphea are at risk for facial deformity, limb length discrepancy and contractures. […] Patients need to recognize that the involved skin will never look completely normal. They must be counseled that treatment is aimed at active disease in the hopes of preventing enlargement of already-present lesions and the development of new lesions. Burnt-out disease is unlikely to improve with immunosuppression and, therefore, the risks of these medications are not warranted in burnt-out disease.

• #90 What Is New in Morphea—Narrative Review on Molecular Aspects and New Targeted Therapies

  https://www.mdpi.com/2077-0383/13/23/7134

  The treatment of morphea remains a challenge for clinicians because of the poor clinical response to treatment. The main therapeutic options, depending on the type of morphea, include topical steroids, topical calcineurin inhibitors, imiquimod, and in more advanced cases methotrexate, systemic steroids, mycophenolate mofetil, and phototherapy (PUVA, UVA1, UVB). […] The earlier treatment starts, the less damage, such as limb deformity, will occur. […] For very active, widespread lesions, systemic treatment with corticosteroids (intravenous or oral) or methotrexate is required. The combination of systemic corticosteroids and methotrexate has the best effect in this group of patients and is the first-line treatment.

• #91 Morphea: Evidence-based recommendations for treatment – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/morphea-evidence-based-recommendations-for-treatment/

  Morphea is a rare fibrosing disorder of the skin that may also involve the underlying muscle, connective tissue, bone and brain. […] Morphea typically goes through two stages: an active (inflammatory) stage and a „burnt out” stage. Treatment is targeted at the active phase, in the hope of stabilizing the size of current lesions and preventing the occurrence of new lesions. Anti-inflammatory and immunosuppressive agents are unlikely to improve the burnt-out phase of morphea and, therefore, should not be used for this phase of the disease. […] Patients with linear morphea are at risk for facial deformity, limb length discrepancy and contractures. […] Patients need to recognize that the involved skin will never look completely normal. They must be counseled that treatment is aimed at active disease in the hopes of preventing enlargement of already-present lesions and the development of new lesions. Burnt-out disease is unlikely to improve with immunosuppression and, therefore, the risks of these medications are not warranted in burnt-out disease.

• #92 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Typically, morphea does not change the length of your life span. However, the rare severe forms of morphea discussed above can produce long-term disability and on extremely rare occasion, death. […] Most patients with circumscribed morphea will do very well with medicated creams. Patients with generalized, linear, mixed and pansclerotic morphea may need light therapy or pills to suppress the inflammation. […] All patients will have less itching and symptoms if the morphea lesions are kept moisturized. It is important to find a thick moisturizing cream that you are comfortable applying twice a day to decrease symptoms. […] Morphea does not tend to run in families; however, if you have morphea your family has a slightly increased risk of having other autoimmune diseases such as vitiligo, psoriasis, lupus, and thyroiditis.

• #93 Morphea Information for Patients | RDS

  https://www.rheumaderm-society.org/morphea-information-for-patients/

  Typically, morphea does not change the length of your life span. However, the rare severe forms of morphea discussed above can produce long-term disability and on extremely rare occasion, death. […] Most patients with circumscribed morphea will do very well with medicated creams. Patients with generalized, linear, mixed and pansclerotic morphea may need light therapy or pills to suppress the inflammation. […] All patients will have less itching and symptoms if the morphea lesions are kept moisturized. It is important to find a thick moisturizing cream that you are comfortable applying twice a day to decrease symptoms. […] Morphea does not tend to run in families; however, if you have morphea your family has a slightly increased risk of having other autoimmune diseases such as vitiligo, psoriasis, lupus, and thyroiditis.

• #94 Morphea: Symptoms, Causes & Treatment | Types of Morphea

  https://resources.healthgrades.com/right-care/skin-hair-and-nails/morphea

  In most cases, morphea resolves on its own over a few years. Doctors may recommend treatment to help manage symptoms, but it won’t shorten the duration of the condition. Morphea treatment may include medicated creams, light therapy, and oral medicines to suppress the immune system. […] Morphea is usually not serious. However, the skin damage may cause self-esteem issues. Pansclerotic morphea can cause long-term disability and even death in very rare cases. See your doctor if you notice unusual skin patches.

• #95 Morphea in Childhood: An Update | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-morphea-in-childhood-an-update-articulo-S157821901830088X

  The classification proposed by PReS also includes the term mixed morphea (MM), a variant that appears to be more common than previously believed. This decision indicates a shift towards viewing morphea as a term encompassing a spectrum of clinical variants characterized by inflammation and fibrosis occurring at different depths. […] Extracutaneous manifestations are observed in up to 40% of patients with morphea. […] Although prognosis in patients with morphea has been little studied and long-term studies are particularly scarce, the available evidence suggests that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae. In the short and medium term, systemic treatment with MTX and/or corticosteroid therapy appears to stop the progression of the disease and/or improve lesions. However, the condition recurs in between 28% and 46% of patients within 20 months of cessation of treatment. Activity persists in between 28% and 89% of patients in the long term. In a survey of patients with LM conducted after follow-up of up to 20 years, all but one of the respondents reported having residual damage. This included cosmetic sequelae in 66% and functional limitations in 38%. In total, 51% of those surveyed considered that the treatment they had received had been effective. Finally, individuals who develop morphea in early childhood may have decreased quality of life and be more likely to develop autoimmune conditions in later life.

• #96 Morphea: Symptoms, Causes, and Treatments

  https://www.healthline.com/health/morphea

  Linear morphea features a single band of thickened, discolored skin. Usually, the indented band runs down an arm or leg, but it might also extend down your forehead. This is referred to as en coup de sabre because it makes the skin look as though its been struck by a sword. […] Linear morphea is the most common type of morphea found in school-aged children. The lesions may extend to the tissue under their skin, even to their muscles and bones, leading to deformities. If linear morphea occurs on their face, it might cause issues with their eyes or alignment of their teeth. […] Morphea with deep lesions, lesions on your face or neck, or widespread lesions can lead to restricted joint mobility, joint pain, cosmetic deformities, permanent eye damage in children, and hair loss. […] Most cases of morphea slowly go away on their own over time and dont alter a persons life expectancy. On average, a lesion lasts three to five years, but discoloration might persist for a few more years. Occasionally, people will develop new lesions later on. […] Linear and deep morphea can interfere with the growth of children and lead to further problems, including differences in limb length, stiff and weak limbs, eye damage, and even death, but this is considered quite rare.

• #97 Morphoea | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/morphoea/

  The main symptom of morphoea is the distinctive patches of hardened and shiny skin. However, these patches can have knock-on effects depending on their position on the body. […] If the patch is on the face, this can cause dental problems as the bone underneath the patch shrinks and becomes thinner. If the patch is on a limb, particularly over a joint, this can cause the joint to stiffen making it difficult to bend and straighten. It could also cause limb length discrepancy where one limb is shorter than the other. […] The outlook for children with morphoea is generally good as it responds well to treatment. In most cases, the condition burns out after a few years of treatment, but there is a small chance it could recur. The child will receive long-term follow up treatment once the morphoea has burnt out to check whether it is recurring and to restart treatment if this is the case.

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