Materiały źródłowe

• #1 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, also referred to as localized scleroderma, is a rare inflammatory disease of the skin and subcutaneous tissue. It has been reported in both kids and adults with equal frequency. Morphea encompasses a wide spectrum of clinical variants ranging from solitary skin lesions with minimal discomfort to severe subtypes like generalized or linear morphea. […] Morphea encompasses a wide variety of clinical phenotypes, which makes its classification challenging. The most commonly used classification was described by Laxer and Zulian, which includes circumscribed morphea, linear morphea, generalized morphea, pan-sclerotic and mixed subtype.

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

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

  Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. […] Diagnosis and management of patients with morphea is not always easy and can be a challenge for the dermatologist. In this article, the basic concepts of the disease are reviewed, with special focus on the diagnosis, classification and treatment. […] The diagnosis of morphea is mainly clinical, through history and careful physical examination of the skin. […] Laboratory tests are not considered necessary for the diagnosis or follow-up of patients with morphea. […] The presence of antinuclear antibodies or anti-double-stranded anti-DNA and anti-histone antibodies occurs in approximately 30% of patients, mainly in those with linear or generalized morphea.

• #1 Morphea | 5-Minute Clinical Consult

  https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/816329/0.4/Morphea

  A benign inflammatory skin disorder leading to excessive collagen deposition with thickening and sclerosis of the skin and subcutaneous tissues with characteristic plaque formation. […] Lesions are not associated with systemic sclerosis (scleroderma) based on lack of sclerosis of fingertips (sclerodactyly), Raynaud phenomenon, and nail fold capillary changes. […] Morphea is classified into five types (Mayo classification): Plaque: most common subtype especially in adults, well circumscribed, and confined to dermis. […] Three phases: active/edematous, inactive/sclerotic/fibrotic, and atrophic lesions. […] 50% of morphea cases present during childhood. […] Linear morphea can develop before age 10 years in 20% of patients. […] Sclerosis is limited to the skin and systemic sclerosis is absent.

• #1 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. It is also known as localised scleroderma. The term 'scleroderma’ covers various types of morphoea and systemic sclerosis. […] The precise cause of morphoea is unknown. […] The diagnosis of morphoea is often made clinically, without the need for further tests. Biopsy, blood tests and imaging may be undertaken if the diagnosis or extent of disease is unclear. […] A skin biopsy enables the skin, subcutaneous fat (and sometimes fascia) to be examined under the microscope. Classic findings in morphoea include sharply squared-off biopsy (cookie-cutter sign), atrophic epidermis, thickened, hyalinised collagen, with possible loss of appendageal structures (such as sweat glands and hair follicles), fat loss / entrapment, and inflammatory cell infiltrate (lymphocytes +/- plasma cells).

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

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

  The diagnosis of morphea is mainly clinical, through history and careful physical examination of the skin. […] Morphea histology depends on two factors: the stage of the disease and the depth to which it extends. […] Assessing the activity of the disease is extremely important for making therapeutic decisions; however, it is not always easy, since currently there are no objective, clear or objective standardized outcome measures. […] Morphea treatment can be a challenge, since there is no medication considered to be the gold standard. […] Treatment should be started early, before the appearance of complications in patients with risk associated with the morphea characteristics. […] Topical treatment is limited to the superficial and circumscribed varieties of morphea, without any movement or growth restriction.

• #1 Morphea: The 2023 update

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9969991/

  The diagnosis of morphea is mainly based on clinical features. […] A skin biopsy for histopathological evaluation is usually reserved for atypical, doubtful cases. […] However, there are no specific histopathology features for morphea and routine histopathology can neither differentiate among the various types nor to distinguish it from SSc. […] The correct assessment of disease severity is a crucial step in the evaluation of all patients with morphea, in particular in pediatric variants. […] Localized scleroderma cutaneous assessment tool (LoSCAT) is a scoring tool that assesses both disease activity and damage in morphea by combining the Physicians Global Assessment (PGA), localized scleroderma skin severity index (LoSSI) and localized scleroderma skin damage index (LoSDI) scores.

• #1 Morphea (Localized Scleroderma) – Dermatology Advisor

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

  Antinuclear Antibodies (ANA) are positive in 39% to 80% (especially in linear and generalized subtype); ssDNA antibodies have been associated with linear morphea and may correlate with increased severity and increased activity (although not fully confirmed). […] Physicians should also keep in mind that there is a greater risk for squamous cell carcinoma in patients with generalized/pansclerotic lesions due to chronic ulcerations. […] There is little understanding of underlying pathophysiology, especially on a molecular level. Most data are extrapolated from research on scleroderma patients. Autoimmune-mediated inflammation is likely the initial event in the formation of morphea lesions. Localized infiltration of immune cells and cytokine release then drives collagen deposition and extracellular matrix remodeling.

• #1 Morphea – Wikipedia

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

  Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies. […] 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.

• #1 Morphea

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

  A self-limited autoimmune disease belonging to the group of collagenoses, characterised by fibrosclerosis of the skin. Both the involvement of internal organs and the transition to systemic scleroderma are a rarity. […] Clinic (is sufficient in most cases for diagnosis) […] Biopsy […] There are no serological progression parameters! […] Laboratory (useful for differential diagnosis) […] blood count (sometimes eosinophilia in linear forms) […] creatinine kinase (e.g. of concomitant myositis) […] Autoantibodies (ANA (positive in up to 80% even without systemic involvement), anti-Scl-70) […] Borrelia serology (pseudoscleroderma in Borrelia infection) […] Modified Rodnan Skin Score (thickness of the skin is measured by palpation) […] Cutometer (elasticity measurement) […] Durometer (hardness test) […] Quantification of induration: […] 20 MHz sonography […] Laser Doppler […] Depth extension: […] CT […] MRI.

• #1 Orphanet: Localized scleroderma

  https://www.orpha.net/en/disease/detail/90289

  A rare skin disease characterized by inflammatory and sclerosing disease affecting the skin and underlying connective tissues (subcutaneous tissue, fascia, muscle or bone). It causes cutaneous patches or bands of skin inflammation and thickness affecting the head, neck region, trunk and extremities. […] Diagnosis is based on clinical presentation. A skin biopsy may help to confirm the diagnosis by revealing abnormal accumulation of collagen, thickening of the vessel walls, and the presence of inflammatory infiltrates in the papillary dermis and around the vessels. Blood tests are usually normal. Some patients may have antinuclear antibodies excluding systemic sclerosis-associated specificities (anti-topoisomerase, anti-centromere or anti-RNA polymerase III antibodies). […] Management is symptomatic and includes the use of topical steroids, tacrolimus, vitamin D derivates or imiquimod for morphea lesions, and systemic steroids, methotrexate or phototherapy for linear scleroderma of the neck and extremities with potential functional/esthetic consequences, or for generalized morphea. Treatment should be initiated promptly to prevent irreversible tissue damage. Physical therapy may be helpful to limit cutaneous indurations and muscle retraction.

• #1

  https://link.springer.com/article/10.1007/s11926-012-0308-9

  There is huge variation in the evaluation, diagnosis, and treatment of patients with morphea (localized scleroderma). […] The purpose of this review is to summarize the results of these studies and to synthesize the information into a rational approach to the diagnosis and assessment of patients with morphea. […] This cross sectional survey of patients enrolled in the Morphea in Adults and Children Cohort underscores often significant delays in diagnosis and treatment of patients with morphea. […] The results of this study support the need for treatment plans accepted across specialties. […] This study reports ultrasound features associated with disease activity in morphea. […] This cross-sectional study of patients with morphea documents the typical finding on MR imaging of morphea lesions with suspected musculoskeletal involvement.

• #1 Ultrasound Monitoring of Childhood Linear Morphea | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-ultrasound-monitoring-childhood-linear-morphea-articulo-S1578219015000670

  Morphea is a form of localized scleroderma that differs from systemic forms of the disease by the presence of well-characterized morphological variants and the absence of any clinically detectable extracutaneous involvement. Many clinical classifications are employed, but there are few management guidelines for use in daily clinical practice. The most recently published guideline includes the following clinical variants: circumscribed morphea, generalized morphea, and the linear variant, which is more typical in childhood. The linear variant is subdivided into 3 subtypes: the purely linear form, coup de sabre morphea, and progressive facial hemiatrophy (or Parry-Romberg syndrome. […] The possibility of performing a diagnostic biopsy was discussed with the family, but, given the cosmetic repercussion, it was decided to use skin ultrasound to confirm the clinical diagnostic suspicion.

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

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

  Pulmonary function test. Your child’s doctor may perform breathing tests to check the function of your child’s lungs, which can be affected by the condition. […] Computed tomography (CT) scan. Your child’s doctor may run this type of scan to analyze areas of the body that may be affected by scleroderma. […] Echocardiogram (EKG). Your child’s doctor may order an EKG, which checks the function of your child’s heart. […] If your child is diagnosed with scleroderma, it is important to closely follow the treatment plan of the pediatric rheumatologist.

• #1 Usefulness of Dermoscopy in Localized Scleroderma (LoS, Morphea) Diagnosis and Assessment-Monocentric Cross-Sectional Study

  https://www.mdpi.com/2077-0383/11/3/764

  Morphea, also known as localized scleroderma (LoS), is a chronic autoimmune disease of the connective tissue. The clinical picture of LoS distinguishes between active and inactive lesions. […] Our study aimed to demonstrate the characteristic dermoscopic features in morphea skin lesions, focusing on demonstrating features in active and inactive lesions. […] We showed that within the active lesions (clinically and with high mLoSSI), white clouds and linear branching vessels had the highest severity. […] We have shown dermoscopy is a valuable tool to assess the activity or inactivity of lesions, which translates into appropriate therapeutic decisions and the possibility of monitoring the patient during and after therapy for possible relapse. […] The clinical picture, although varied, is usually significant enough to establish the diagnosis in the majority of cases.

• #1 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  There is no cure for morphoea. Treatment is aimed at halting ongoing disease activity and progression. […] Specific treatment decisions in morphoea are guided by the subtype of morphoea and its severity. […] Disease progression and treatment response can be monitored using photographs, the localized scleroderma cutaneous assessment tool(LoSCAT) and other highly specialised tests, such as infrared thermography. […] Morphoea can follow a protracted course, which can be relapsing and remitting, or chronically active. Milder forms of the disease tend to become inactive within 35 years.

• #1 Pathology Outlines – Morphea and systemic sclerosis

  https://www.pathologyoutlines.com/topic/skinnontumorscleroderma.html

  For systemic sclerosis, the clinical presentation includes physical examination, capillaroscopy, and modified Rodnan skin score. […] Autoantibodies such as antinuclear antibody (ANA) are commonly present in systemic sclerosis. […] Diagnosis may involve an organ-oriented diagnostic workup, including laboratory tests and imaging.

• #1 Morphea – StatPearls – NCBI Bookshelf

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

  It is vital to differentiate morphea from systemic sclerosis due to its systemic organ involvement and bad prognosis with delay in diagnosis. […] Morphea is a rare skin disorder that can affect both children and adults with equal frequency. Autoimmune dysregulation plays a role in the etiology of morphea, but patients should be educated about external factors like trauma, radiation, and friction in morphea. […] Morphea encompasses a wide variety of phenotypes that range from benign isolated lesions to deep forms of morphea, which can have a poor prognosis. Unfortunately, this makes diagnosis challenging, and there is no consensus in classification, which can result in delayed diagnosis and initiation of treatment.

• #1 Morphea (Localized Scleroderma) – Dermatology Advisor

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

  Morphea may present at any age. In children, the linear subtype predominates while in adults plaque and generalized subtypes are most common. Morphea occurs more frequently in women, especially in adults where female predominance is marked (5:1). […] Morphea is not the same as scleroderma (systemic sclerosis), and it does not become scleroderma. Key differences include presence of the following in scleroderma, NOT morphea: acrosclerosis/sclerodactyly; Raynauds phenomenon; antibodies such as anti-centromere, Scl-70, and RNA polymerase; and characteristic internal organ involvement. […] Diagnosis is usually based on clinical findings as described above. There are no widely accepted markers for diagnosis, prognosis, or disease activity. […] Skin histopathology (for confirmation, if other diagnoses are in consideration): 4mm punch biopsy (skin and subcutaneous fat) from inflammatory or indurated border and sclerotic center (indicate on pathology requisition). Subtle changes in skin architecture may be detected by comparing lesional with site-matched unaffected skin biopsies.

• #1 Morphea Differential Diagnoses

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

  Also consider the following: […] Some of the entities in the differential diagnosis above often manifest with a sclerodermoid (ie, diffuse sclerosis) rather than morpheaform (ie, discrete areas of sclerosis) morphology. […] Atrophoderma of Pasini and Pierini and eosinophilic fasciitis are generally viewed as part of the morphea spectrum. […] Lichen sclerosus and morphea can coexist, with clinical and histologic findings of both conditions present in the same patient and even within the same lesion. […] The differential diagnosis for early, erythematous lesions of plaque-type morphea may include the following:

• #1 Delays in diagnosis, variations in treatment for morphea

  https://medicalxpress.com/news/2012-10-diagnosis-variations-treatment-morphea.html

  Patients with localized scleroderma (morphea) often experience delay in diagnosis and variability in treatment that is based more on the specialty of the provider than disease characteristics, according to a study published in the November issue of the Journal of the American Academy of Dermatology. […] The researchers found that 63 percent of patients received a diagnosis more than six months after morphea onset. […] „In summary, we identified several factors that impact the care of patients with morphea,” the authors write. „These include delay in diagnosis and treatment, large variation in evaluation and therapy based on the specialty of the provider, and widespread use of evaluations and therapy with little evidence for their efficacy.”

• #1 Frequently Asked Questions about Morphea: Department of Dermatology – UT Southwestern, Dallas, Texas

  https://www.utsouthwestern.edu/education/medical-school/departments/dermatology/research/morphea-registry/disease-faq.html

  Morphea is an autoimmune disorder (such as type I diabetes, lupus, vitiligo, or multiple sclerosis, among others). While morphea does not affect lifespan, it can significantly affect a patients appearance or have symptoms including itching and pain. Often patients are not diagnosed in the early stages as it can initially look like a bruise. […] The cause of morphea is unknown, but it is thought that the collagen-producing cells become overactive and overproduce collagen. This can result in areas of sclerosis or skin hardening. Researchers are trying to determine what triggers the disease and whether it is genetic in origin. […] Morphea is more common in women than in men and about half of all patients first show signs before the age of 18. There is very little information about who are at highest risk for getting morphea as there are no known predictors for the disease. Data from our registry indicates that other autoimmune diseases are more common in patients with morphea and possibly their relatives as well.

• #1 Morphea – StatPearls – NCBI Bookshelf

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

  The etiology of morphea is unclear, but genetic predisposition, autoimmune dysregulation, and environmental factors play a role in the pathogenesis of morphea. […] The pathophysiology of morphea is vague, but there is evidence supporting the role of autoimmune dysregulation with abnormal cytokine production and vascular dysfunction in morphea. […] Histopathologic findings are helpful to confirm a diagnosis and correlate with the clinical state of morphea. […] Morphea is usually a clinical diagnosis and can be confirmed by physical examination. However, some patients need a skin biopsy, including subcutaneous fat, to confirm the diagnosis of morphea. […] Clinicians should review the subtype of morphea, the depth of involvement, and disease activity, which play a significant role in treatment decisions. Early diagnosis and treatment are necessary to minimize damage such as cosmetic sequelae and joint contractures or limb deformities in patients with severe forms of morphea.

• #1 Morphoea – Tests and Diagnosis | SRUK

  https://www.sruk.co.uk/scleroderma/scleroderma-getting-diagnosed/morphea-tests-and-diagnosis/

  Your doctor may diagnose morphoea by examining the affected skin and asking you about your signs and symptoms. He or she may take a small sample of the affected skin (skin biopsy) for examination in the laboratory. This may reveal changes in your skin, such as thickening of the collagen in the second layer of skin (dermis). […] It’s important to distinguish morphoea from systemic scleroderma, so if you have morphoea, your doctor will likely refer you to a specialist in skin disorders (dermatologist) or diseases of the joints, bones and muscles (rheumatologist). […] Ultrasound and magnetic resonance imaging (MRI) may be useful in monitoring disease progression and how it is responding to treatment.

• #1

  https://journals.lww.com/idoj/fulltext/2020/11020/update_on_management_of_morphea__localized.1.aspx

  Juvenile localized scleroderma (morphea) is the predominant scleroderma in childhood which affects the skin and may extend to the underlying fascia, muscle, joints and bone. […] The assessment of activity and damage can be done with a validated instrument like LoSCAT. […] Disease classified as low severity which includes superficial plaque morphea can be treated with topical mid potent- potent steroids, tacrolimus, calcipotriol or imiquimod in combination with phototherapy. […] Methotrexate is recommended for linear, deep and generalized morphea. […] Steroids are effective in the early inflammatory stage and used in combination with methotrexate. […] Methotrexate is continued for at least 12 months after adequate response is achieved. […] Mycophenolate mofetil is given in cases where methotrexate is contraindicated or for those who do not respond to methotrexate.

• #1

  https://journals.lww.com/idoj/fulltext/2020/11020/update_on_management_of_morphea__localized.1.aspx

  There are also reports of improvement of disease with ciclosporine and hydroxychloroquine. […] In severe cases, recalcitrant to standard therapy there may be a role for biologics, JAK inhibitors, and IVIG. […] Supportive measures like physiotherapy and psychiatric counseling are also important in the management of morphea. […] Orthopedic surgery and other measures like autologous fat transfer may be advocated once the disease is inactive.

• #1 Morphea (Localized Scleroderma) – Dermatology Advisor

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

  Currently there is no curative therapeutic approach available, and there is general agreement among morphea experts that the systemic therapies suppress disease activity, as evidenced by reports of disease reactivation after cessation of therapy. Further, some patients experience a remitting/relapsing course. Thus management encompasses a complex interplay of risk vs benefit, minimizing side effects, and close patient follow-up. Patients also benefit from a multidisciplinary approach when severe disease is present.

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

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

  Systemic treatment should be used in linear morphea in the head and extremities with potential functional or cosmetic disability, in generalized or rapidly progressive lesions and in pan-sclerotic morphea. […] The prognosis of circumscribed or superficial morphea is good, although the disease shows a tendency towards progression.

• #1

  https://www.aboutkidshealth.ca/morphea

  Morphea scleroderma is a rare condition in which the skin becomes thick and hard due to excess collagen. Learn about possible treatments for morphea. […] Morphea is diagnosed by history and examination. […] The doctor might take a small part of the skin to send to the lab for tests. This is called a biopsy. There is no blood test to confirm the diagnosis for morphea, although the doctors will ask for tests to look for functioning of other organs. […] There are several treatments for morphea. […] Morphea can be treated, but it cannot be cured. Treatment can reduce the inflammation, stop lesions from spreading and prevent new lesions from appearing. […] The active inflammation of morphea may last three to five years, even if not treated. Most children recover very well.

• #1 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. […] Morphea usually improves on its own over time, though recurrences are common. In the meantime, medications and therapies are available to help treat the skin discoloration and other effects. […] Signs and symptoms of morphea vary depending on the type and stage of the condition. […] 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.

• #1 Morphea: The 2023 update

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9969991/

  Disease activity assessment based on current validated clinical scores is a crucial step in the initial evaluation of patients with morphea. […] A late diagnosis or an incorrect severity assessment may lead to a delay of appropriate treatment, and thus to physical and functional disabilities as well as decreased quality of life.

• #2 Morphea: The 2023 update

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9969991/

  Morphea, also known as localized scleroderma, is a chronic inflammatory connective tissue disorder with variable clinical presentations, that affects both adults and children. […] It is crucial to properly assess the disease activity and to initiate promptly the adequate treatment, thus preventing subsequent damage. […] The mainstay of treatment is based on corticosteroids and methotrexate. […] This review presents the current understanding of morphea by discussing its epidemiology, diagnosis, management and prognosis. […] To date, there is no cure for morphea and therapy remains a major clinical challenge. […] Herein, we review the various clinical presentations of morphea, the most recent advances regarding its pathogenesis, as well as the many challenges that the clinicians encounter in disease diagnosis, severity assessment and appropriate treatment selection.

• #2 Morphea – StatPearls – NCBI Bookshelf

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

  The etiology of morphea is unclear, but genetic predisposition, autoimmune dysregulation, and environmental factors play a role in the pathogenesis of morphea. […] The pathophysiology of morphea is vague, but there is evidence supporting the role of autoimmune dysregulation with abnormal cytokine production and vascular dysfunction in morphea. […] Histopathologic findings are helpful to confirm a diagnosis and correlate with the clinical state of morphea. […] Morphea is usually a clinical diagnosis and can be confirmed by physical examination. However, some patients need a skin biopsy, including subcutaneous fat, to confirm the diagnosis of morphea. […] Clinicians should review the subtype of morphea, the depth of involvement, and disease activity, which play a significant role in treatment decisions. Early diagnosis and treatment are necessary to minimize damage such as cosmetic sequelae and joint contractures or limb deformities in patients with severe forms of morphea.

• #2 Morphea: The 2023 update

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9969991/

  Disease activity assessment based on current validated clinical scores is a crucial step in the initial evaluation of patients with morphea. […] A late diagnosis or an incorrect severity assessment may lead to a delay of appropriate treatment, and thus to physical and functional disabilities as well as decreased quality of life.

• #2 Morphea Workup: Laboratory Studies, Imaging Studies, Other Tests

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

  Laboratory tests have a limited role in the evaluation of patients with morphea. The studies below can be considered on a case-by-case basis (eg, to monitor disease activity) but generally are not required. […] Although a presumptive diagnosis of morphea can frequently be made based on clinical findings, a biopsy can be used to confirm the diagnosis and delineate the depth of involvement. […] The histologic findings of morphea and systemic sclerosis are similar, with a fundamental process of thickening and homogenization of collagen bundles. The depth of involvement is important for categorization into the morphea subtypes.

• #2 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. It is also known as localised scleroderma. The term 'scleroderma’ covers various types of morphoea and systemic sclerosis. […] The precise cause of morphoea is unknown. […] The diagnosis of morphoea is often made clinically, without the need for further tests. Biopsy, blood tests and imaging may be undertaken if the diagnosis or extent of disease is unclear. […] A skin biopsy enables the skin, subcutaneous fat (and sometimes fascia) to be examined under the microscope. Classic findings in morphoea include sharply squared-off biopsy (cookie-cutter sign), atrophic epidermis, thickened, hyalinised collagen, with possible loss of appendageal structures (such as sweat glands and hair follicles), fat loss / entrapment, and inflammatory cell infiltrate (lymphocytes +/- plasma cells).

• #2 Histopathologic study of morphea – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/histopathologic-study-of-morphea/

  Morphea (localised scleroderma) is a rare inflammatory fibrosing disease of the skin. Patterns of sclerosis and degree of inflammation are known to have clinical implications such as increased functional limitations. Histopathology of morphea has received little attention in India. […] Cases diagnosed based on clinical suspicion and histopathologic confirmation from 2012 to 2020 were included. Skin biopsies were studied for histopathologic features. Patterns of arrangement of lymphocytes were categorised as scattered interstitial, Indian file and lymphoid aggregate. […] The most common clinical subtype was the plaque type (n = 32, 43%). Dermal sclerosis, which was present in all cases, was either top-heavy, bottom-heavy, full thickness or rarely patchy or focal. […] The presence of top-heavy sclerosis supports the concept that morphea may be limited to the superficial reticular dermis, known as superficial morphea. Diminished skin adnexal structures were consistent findings.

• #2 Pathology Outlines – Morphea and systemic sclerosis

  https://www.pathologyoutlines.com/topic/skinnontumorscleroderma.html

  Morphea and systemic sclerosis are rare autoimmune fibrosing entities subtyped based on clinical presentation. […] Dysregulation of collagen synthesis and degradation causes vascular and immune dysfunction, leading to organ damaging fibrosis. […] Localized scleroderma (morphea) is divided into limited/circumscribed, generalized, linear, deep, and mixed types. […] Systemic sclerosis includes limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, and systemic sclerosis sine scleroderma. […] The clinical presentation of localized scleroderma does not require a routine systemic workup, and diagnosis is primarily based on physical examination and clinical assessment. […] Histopathology is not able to differentiate between localized scleroderma and systemic sclerosis.

• #2 Morphea (Localized Scleroderma) – Dermatology Advisor

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

  Antinuclear Antibodies (ANA) are positive in 39% to 80% (especially in linear and generalized subtype); ssDNA antibodies have been associated with linear morphea and may correlate with increased severity and increased activity (although not fully confirmed). […] Physicians should also keep in mind that there is a greater risk for squamous cell carcinoma in patients with generalized/pansclerotic lesions due to chronic ulcerations. […] There is little understanding of underlying pathophysiology, especially on a molecular level. Most data are extrapolated from research on scleroderma patients. Autoimmune-mediated inflammation is likely the initial event in the formation of morphea lesions. Localized infiltration of immune cells and cytokine release then drives collagen deposition and extracellular matrix remodeling.

• #2 Morphea

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

  A self-limited autoimmune disease belonging to the group of collagenoses, characterised by fibrosclerosis of the skin. Both the involvement of internal organs and the transition to systemic scleroderma are a rarity. […] Clinic (is sufficient in most cases for diagnosis) […] Biopsy […] There are no serological progression parameters! […] Laboratory (useful for differential diagnosis) […] blood count (sometimes eosinophilia in linear forms) […] creatinine kinase (e.g. of concomitant myositis) […] Autoantibodies (ANA (positive in up to 80% even without systemic involvement), anti-Scl-70) […] Borrelia serology (pseudoscleroderma in Borrelia infection) […] Modified Rodnan Skin Score (thickness of the skin is measured by palpation) […] Cutometer (elasticity measurement) […] Durometer (hardness test) […] Quantification of induration: […] 20 MHz sonography […] Laser Doppler […] Depth extension: […] CT […] MRI.

• #2 Morphea (Localized Scleroderma) – Dermatology Advisor

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

  Morphea may present at any age. In children, the linear subtype predominates while in adults plaque and generalized subtypes are most common. Morphea occurs more frequently in women, especially in adults where female predominance is marked (5:1). […] Morphea is not the same as scleroderma (systemic sclerosis), and it does not become scleroderma. Key differences include presence of the following in scleroderma, NOT morphea: acrosclerosis/sclerodactyly; Raynauds phenomenon; antibodies such as anti-centromere, Scl-70, and RNA polymerase; and characteristic internal organ involvement. […] Diagnosis is usually based on clinical findings as described above. There are no widely accepted markers for diagnosis, prognosis, or disease activity. […] Skin histopathology (for confirmation, if other diagnoses are in consideration): 4mm punch biopsy (skin and subcutaneous fat) from inflammatory or indurated border and sclerotic center (indicate on pathology requisition). Subtle changes in skin architecture may be detected by comparing lesional with site-matched unaffected skin biopsies.

• #2

  https://link.springer.com/article/10.1007/s11926-012-0308-9

  The clinical significance of these occult changes is uncertain, but the results demonstrate the utility of MRI when musculoskeletal involvement is suspected. […] The localized scleroderma skin severity index and physician global assessment of disease activity: a work in progress toward development of localized scleroderma outcome measures. […] Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof-of-concept study.

• #2 Ultrasound Monitoring of Childhood Linear Morphea | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-ultrasound-monitoring-childhood-linear-morphea-articulo-S1578219015000670

  A diagnosis of inactive linear morphea was made on the basis of the clinical appearance and a compatible ultrasound image, and it was decided to take a wait-and-see approach. […] Ultrasound is a useful method for the diagnosis and, in particular, for the follow-up of diseases that affect the dermis and subcutaneous cellular tissue. […] However, the progression of morphea can initially affect deeper layers and be clinically undetectable. This can delay the early initiation of treatment. […] The difference in thickness between affected skin and normal skin does not correlate with disease activity. […] In conclusion, skin ultrasound is a non-invasive technique that can reduce the need for skin biopsy, particularly when the clinical findings are highly suggestive, as in our patient, or when histology has already been performed, avoiding cosmetic repercussions in visible areas. The dermatologist must learn to perform ultrasound studies, as they enable plaques of morphea to be monitored and contribute not only to the ability to take a wait-and-see approach but also to monitoring the efficacy of treatments.

• #2 Usefulness of Dermoscopy in Localized Scleroderma (LoS, Morphea) Diagnosis and Assessment-Monocentric Cross-Sectional Study

  https://www.mdpi.com/2077-0383/11/3/764

  Further diagnostic workup should be undertaken, especially in the inflammatory and atrophic phases, as well as in atypical or discrete lesions. […] Dermoscopy is a non-invasive, inexpensive, and accessible method that allows for the rapid diagnosis of skin lesions, also in an outpatient setting. […] Its application facilitates the diagnosis of subclinical or discrete lesions because of the clear dermoscopic-histopathologic correlation. […] Our study aimed to demonstrate the characteristic dermoscopic features in morphea skin lesions. […] Dermoscopy allows for differential diagnosis of LoS lesions, especially with the extragenital variety of lichen sclerosus. […] Moreover, as we have shown, it is a valuable tool to assess the activity or inactivity of lesions, which translates into appropriate therapeutic decisions and the possibility of monitoring the patient during and after therapy for possible relapse.

• #2

  https://journals.lww.com/idoj/fulltext/2020/11020/update_on_management_of_morphea__localized.1.aspx

  Juvenile localized scleroderma (morphea) is the predominant scleroderma in childhood which affects the skin and may extend to the underlying fascia, muscle, joints and bone. […] The assessment of activity and damage can be done with a validated instrument like LoSCAT. […] Disease classified as low severity which includes superficial plaque morphea can be treated with topical mid potent- potent steroids, tacrolimus, calcipotriol or imiquimod in combination with phototherapy. […] Methotrexate is recommended for linear, deep and generalized morphea. […] Steroids are effective in the early inflammatory stage and used in combination with methotrexate. […] Methotrexate is continued for at least 12 months after adequate response is achieved. […] Mycophenolate mofetil is given in cases where methotrexate is contraindicated or for those who do not respond to methotrexate.

• #2 Pathology Outlines – Morphea and systemic sclerosis

  https://www.pathologyoutlines.com/topic/skinnontumorscleroderma.html

  For systemic sclerosis, the clinical presentation includes physical examination, capillaroscopy, and modified Rodnan skin score. […] Autoantibodies such as antinuclear antibody (ANA) are commonly present in systemic sclerosis. […] Diagnosis may involve an organ-oriented diagnostic workup, including laboratory tests and imaging.

• #2 Morphoea – Tests and Diagnosis | SRUK

  https://www.sruk.co.uk/scleroderma/scleroderma-getting-diagnosed/morphea-tests-and-diagnosis/

  Your doctor may diagnose morphoea by examining the affected skin and asking you about your signs and symptoms. He or she may take a small sample of the affected skin (skin biopsy) for examination in the laboratory. This may reveal changes in your skin, such as thickening of the collagen in the second layer of skin (dermis). […] It’s important to distinguish morphoea from systemic scleroderma, so if you have morphoea, your doctor will likely refer you to a specialist in skin disorders (dermatologist) or diseases of the joints, bones and muscles (rheumatologist). […] Ultrasound and magnetic resonance imaging (MRI) may be useful in monitoring disease progression and how it is responding to treatment.

• #2 Morphea | 5-Minute Clinical Consult

  https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/816329/all/Morphea?q=Telangiectasia

  A benign inflammatory skin disorder leading to excessive collagen deposition with thickening and sclerosis of the skin and subcutaneous tissues with characteristic plaque formation. […] Lesions are not associated with systemic sclerosis (scleroderma) based on lack of sclerosis of fingertips (sclerodactyly), Raynaud phenomenon, and nail fold capillary changes. […] Morphea is classified into five types (Mayo classification): Plaque: most common subtype especially in adults, well circumscribed, and confined to dermis. […] 50% of morphea cases present during childhood. […] Linear morphea can develop before age 10 years in 20% of patients. […] Sclerosis is limited to the skin and systemic sclerosis is absent. […] Biopsy shows lymphocytic perivascular infiltration of the dermis, decreased number of blood vessels, and eccrine (sweat) glands.

• #2 Morphea Differential Diagnoses

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

  Also consider the following: […] Some of the entities in the differential diagnosis above often manifest with a sclerodermoid (ie, diffuse sclerosis) rather than morpheaform (ie, discrete areas of sclerosis) morphology. […] Atrophoderma of Pasini and Pierini and eosinophilic fasciitis are generally viewed as part of the morphea spectrum. […] Lichen sclerosus and morphea can coexist, with clinical and histologic findings of both conditions present in the same patient and even within the same lesion. […] The differential diagnosis for early, erythematous lesions of plaque-type morphea may include the following:

• #2 Delays in Diagnosis, Variations in Treatment for Morphea – MPR

  https://www.empr.com/home/news/drug-news/delays-in-diagnosis-variations-in-treatment-for-morphea/

  Patients with localized scleroderma (morphea) often experience delay in diagnosis and variability in treatment that is based more on the specialty of the provider than disease characteristics, according to a study published in the November issue of the Journal of the American Academy of Dermatology. […] The researchers found that 63 percent of patients received a diagnosis more than six months after morphea onset. […] In summary, we identified several factors that impact the care of patients with morphea, the authors write. These include delay in diagnosis and treatment, large variation in evaluation and therapy based on the specialty of the provider, and widespread use of evaluations and therapy with little evidence for their efficacy.

• #2 Morphea: Symptoms, Causes, and Treatments

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

  Morphea is a skin condition that involves a patch or patches of discolored or hardened skin on the face, neck, hands, torso, or feet. The condition is rare and thought to affect less than 3 out of 100,000 people. […] 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. […] If you have unexplained hard or discolored patches of skin, your doctor may refer you to a dermatologist (a doctor who specializes in skin problems) or a rheumatologist (a doctor who specializes in diseases of the joints, bones, and muscles). […] There is no test for diagnosing morphea. Your doctor will examine your skin and, though not usually necessary, might take a small sample to have analyzed by a lab. This is called a skin biopsy. […] They may also order some tests to help distinguish morphea from something called systemic scleroderma. This type of scleroderma is similar to morphea at first. But it can later affect internal organs and requires more aggressive treatment.

• #2 Morphea – Therapeutics in Dermatology

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

  Morphea is a rare, autoimmune skin disease characterized by inflammation and sclerosis (hardening) of the skin and soft tissues and may lead to scar-like lesions. […] Diagnosis can be usually made through skin examination. A skin biopsy (a small piece of the skin removed under a local anesthesia) can be taken from the affected area if there is uncertainty or to rule out other skin diseases. Blood tests may also be helpful. […] The treatment for morphea depends on the type (linear, plaque or generalized), the age of the patients and whether it has spread underneath the skin. Plaque morphea does not always need treatment as untreated disease normally progresses over 35 years and then regresses very slowly over years. However, strong steroid creams or ointments, non-steroid agents such as tacrolimus or imiquimod are sometimes indicated. Injections with steroid into the affected skin can also help. Early treatment is especially important for linear and generalized morphea to prevent further consequences, like loss of mobility. Options include phototherapy or intravenous treatment with steroids and methotrexate. Other treatments include penicillin and its derivatives or vitamin A derivatives, such as acitretin or isotretinoin. In addition to care from a dermatologist, expert treatment may be needed from a plastic surgeon or orthopaedic surgeon; in the case of persistent contractures, reconstructive surgery may be needed, and surgical revision may also be required in linear or en coup de sabre morphea. Finally, expert and longstanding physiotherapy is obligatory for patients in whom morphea threatens to impair mobility.

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

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

  Morphea, also sometimes called localized scleroderma, is a rare autoimmune disorder that causes skin thickening, hardening, and color changes. […] Morphea can occur in distinct patterns of symptoms. […] Morphea and systemic sclerosis have some similarities in their symptoms and underlying causes. They both involve underlying inflammation that leads to scarring and thickening of the skin. […] However, even though they are both kinds of scleroderma, morphea and systemic sclerosis are quite different. […] The medical history and medical exam are key parts of diagnosis. […] A skin biopsy may also be important to rule out other possibilities and confirm a diagnosis. […] A generalist physician can diagnose morphea, but the expertise of a dermatologist or rheumatologist is sometimes helpful.

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

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

  The diagnosis of morphea is mainly clinical, through history and careful physical examination of the skin. […] Morphea histology depends on two factors: the stage of the disease and the depth to which it extends. […] Assessing the activity of the disease is extremely important for making therapeutic decisions; however, it is not always easy, since currently there are no objective, clear or objective standardized outcome measures. […] Morphea treatment can be a challenge, since there is no medication considered to be the gold standard. […] Treatment should be started early, before the appearance of complications in patients with risk associated with the morphea characteristics. […] Topical treatment is limited to the superficial and circumscribed varieties of morphea, without any movement or growth restriction.

• #2 Morphea: Clinical Considerations and Management – Journal of the Turkish Academy of Dermatology

  https://jtad.org/articles/morphea-clinical-considerations-and-management/doi/jtad.galenos.2020.70299

  Patients with severe linear scleroderma of the face, especially the Parry Romberg variant may have associated neurologic disturbances. […] There is a wide range of therapeutic options available for the treatment of morphea. […] Topical steroids are the most commonly preferred first line treatment for cases of morphea with limited involvement. […] Phototherapy is the first-line treatment option for extensive or rapidly progressing disease without deep involvement and can also be considered for patients with limited involvement that are unresponsive to topical treatment. […] Morphea lesions with deep involvement or lesions causing functional impairments should be treated with systemic therapies. […] Autologous fat grafting is currently considered as ideal treatment for inactive linear morphea of the face.

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

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

  Systemic treatment should be used in linear morphea in the head and extremities with potential functional or cosmetic disability, in generalized or rapidly progressive lesions and in pan-sclerotic morphea. […] The prognosis of circumscribed or superficial morphea is good, although the disease shows a tendency towards progression.

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

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

  Unfortunately, we dont have a cure for morphea, but a variety of treatments can help lessen symptoms. […] Specific treatment will depend on the type and severity of morphea involved. […] Phototherapy is also an option for these people. […] Physical therapy and occupational therapy can be key for people who have joint contractures or deeper muscle and joint involvement. […] Morphea can have a significant psychological impact.

• #2 Scleroderma: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/scleroderma-treatment

  If the diagnosis is scleroderma, treatment is most effective when started shortly after the disease has begun. […] The type of treatment you receive will depend on how deeply the scleroderma goes and many other factors, including the type of scleroderma you have and what skin problems the scleroderma is causing. […] While there are many treatment options, the U.S. Food and Drug Administration (FDA) has not approved any drug to treat scleroderma on the skin. […] If you have only a few patches of morphea (a type of scleroderma) on your skin, the following medicines can be effective: Calcipotriene, Calcipotriene + a strong corticosteroid, Imiquimod, Tacrolimus ointment. […] A type of light treatment called narrowband UVB treatment can be helpful. […] When the morphea goes deeper, UVA-1 phototherapy may be prescribed.

• #2 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. […] Morphea usually improves on its own over time, though recurrences are common. In the meantime, medications and therapies are available to help treat the skin discoloration and other effects. […] Signs and symptoms of morphea vary depending on the type and stage of the condition. […] 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.

• #2 Morphea – Cleaver Dermatology

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

  Morphea is a form of Scleroderma that literally means hard skin. […] Morphea is usually chronic or recurrent. […] An early diagnosis allows for the most successful treatment of affected skin, may help slow the development of new patches and allows us to identify and treat complications before they progress. […] Dr. Cleaver may confirm a diagnosis of morphea based on: Physical examination. Discoloration combined with skin firmness is a good indicator of morphea. […] Dr. Cleaver may remove a small tissue sample (biopsy) of your affected skin to be examined in the laboratory for abnormalities that allow for diagnosis, such as thickening of the collagen in the second layer of skin (dermis). […] Morphea has no known cure. Treatment of morphea focuses on controlling the signs and symptoms and slowing the spread of the disease.

• #2 Delays in diagnosis, variations in treatment for morphea

  https://medicalxpress.com/news/2012-10-diagnosis-variations-treatment-morphea.html

  Patients with localized scleroderma (morphea) often experience delay in diagnosis and variability in treatment that is based more on the specialty of the provider than disease characteristics, according to a study published in the November issue of the Journal of the American Academy of Dermatology. […] The researchers found that 63 percent of patients received a diagnosis more than six months after morphea onset. […] „In summary, we identified several factors that impact the care of patients with morphea,” the authors write. „These include delay in diagnosis and treatment, large variation in evaluation and therapy based on the specialty of the provider, and widespread use of evaluations and therapy with little evidence for their efficacy.”

• #3 Morphea (Localized Scleroderma) – Dermatology Advisor

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

  Morphea may present at any age. In children, the linear subtype predominates while in adults plaque and generalized subtypes are most common. Morphea occurs more frequently in women, especially in adults where female predominance is marked (5:1). […] Morphea is not the same as scleroderma (systemic sclerosis), and it does not become scleroderma. Key differences include presence of the following in scleroderma, NOT morphea: acrosclerosis/sclerodactyly; Raynauds phenomenon; antibodies such as anti-centromere, Scl-70, and RNA polymerase; and characteristic internal organ involvement. […] Diagnosis is usually based on clinical findings as described above. There are no widely accepted markers for diagnosis, prognosis, or disease activity. […] Skin histopathology (for confirmation, if other diagnoses are in consideration): 4mm punch biopsy (skin and subcutaneous fat) from inflammatory or indurated border and sclerotic center (indicate on pathology requisition). Subtle changes in skin architecture may be detected by comparing lesional with site-matched unaffected skin biopsies.

• #3 Morphea: The 2023 update

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9969991/

  The diagnosis of morphea is mainly based on clinical features. […] A skin biopsy for histopathological evaluation is usually reserved for atypical, doubtful cases. […] However, there are no specific histopathology features for morphea and routine histopathology can neither differentiate among the various types nor to distinguish it from SSc. […] The correct assessment of disease severity is a crucial step in the evaluation of all patients with morphea, in particular in pediatric variants. […] Localized scleroderma cutaneous assessment tool (LoSCAT) is a scoring tool that assesses both disease activity and damage in morphea by combining the Physicians Global Assessment (PGA), localized scleroderma skin severity index (LoSSI) and localized scleroderma skin damage index (LoSDI) scores.

• #3 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. […] This makes the early identification of activity and initiation of appropriate treatment crucial to limiting damage in morphea. […] The diagnosis of morphea can typically be made based on clinical findings, however biopsy of the lesions and imaging can help confirm the diagnosis or exclude other diagnoses. […] Treatment of morphea depends on clinical activity, depth of lesion involvement, and extent of disease, and primarily centers around limiting disease activity. […] Active lesions that are isolated to a limited surface area can be treated by topical or intralesional steroids, as well as calcineurin inhibitors such as tacrolimus. […] Systemic therapy in morphea is indicated for those with moderate-severe disease, large body surface area involvement, deep involvement, or for lesions that may impact function/cosmesis (facial lesions).

• #3 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. It is also known as localised scleroderma. The term 'scleroderma’ covers various types of morphoea and systemic sclerosis. […] The precise cause of morphoea is unknown. […] The diagnosis of morphoea is often made clinically, without the need for further tests. Biopsy, blood tests and imaging may be undertaken if the diagnosis or extent of disease is unclear. […] A skin biopsy enables the skin, subcutaneous fat (and sometimes fascia) to be examined under the microscope. Classic findings in morphoea include sharply squared-off biopsy (cookie-cutter sign), atrophic epidermis, thickened, hyalinised collagen, with possible loss of appendageal structures (such as sweat glands and hair follicles), fat loss / entrapment, and inflammatory cell infiltrate (lymphocytes +/- plasma cells).

• #3 Morphea

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

  A self-limited autoimmune disease belonging to the group of collagenoses, characterised by fibrosclerosis of the skin. Both the involvement of internal organs and the transition to systemic scleroderma are a rarity. […] Clinic (is sufficient in most cases for diagnosis) […] Biopsy […] There are no serological progression parameters! […] Laboratory (useful for differential diagnosis) […] blood count (sometimes eosinophilia in linear forms) […] creatinine kinase (e.g. of concomitant myositis) […] Autoantibodies (ANA (positive in up to 80% even without systemic involvement), anti-Scl-70) […] Borrelia serology (pseudoscleroderma in Borrelia infection) […] Modified Rodnan Skin Score (thickness of the skin is measured by palpation) […] Cutometer (elasticity measurement) […] Durometer (hardness test) […] Quantification of induration: […] 20 MHz sonography […] Laser Doppler […] Depth extension: […] CT […] MRI.

• #3 Morphoea – Tests and Diagnosis | SRUK

  https://www.sruk.co.uk/scleroderma/scleroderma-getting-diagnosed/morphea-tests-and-diagnosis/

  Your doctor may diagnose morphoea by examining the affected skin and asking you about your signs and symptoms. He or she may take a small sample of the affected skin (skin biopsy) for examination in the laboratory. This may reveal changes in your skin, such as thickening of the collagen in the second layer of skin (dermis). […] It’s important to distinguish morphoea from systemic scleroderma, so if you have morphoea, your doctor will likely refer you to a specialist in skin disorders (dermatologist) or diseases of the joints, bones and muscles (rheumatologist). […] Ultrasound and magnetic resonance imaging (MRI) may be useful in monitoring disease progression and how it is responding to treatment.

• #3 Usefulness of Dermoscopy in Localized Scleroderma (LoS, Morphea) Diagnosis and Assessment-Monocentric Cross-Sectional Study

  https://www.mdpi.com/2077-0383/11/3/764

  Further diagnostic workup should be undertaken, especially in the inflammatory and atrophic phases, as well as in atypical or discrete lesions. […] Dermoscopy is a non-invasive, inexpensive, and accessible method that allows for the rapid diagnosis of skin lesions, also in an outpatient setting. […] Its application facilitates the diagnosis of subclinical or discrete lesions because of the clear dermoscopic-histopathologic correlation. […] Our study aimed to demonstrate the characteristic dermoscopic features in morphea skin lesions. […] Dermoscopy allows for differential diagnosis of LoS lesions, especially with the extragenital variety of lichen sclerosus. […] Moreover, as we have shown, it is a valuable tool to assess the activity or inactivity of lesions, which translates into appropriate therapeutic decisions and the possibility of monitoring the patient during and after therapy for possible relapse.

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

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

  The most widely investigated systemic therapies include combinations of methotrexate and corticosteroids. […] The current understanding of morphea pathogenesis is incomplete but points primarily towards the role of dysregulated immune and fibrotic pathways, with environmental triggers, genetic predisposition, and vascular dysfunction also playing a role. […] Further study using these state of the art approaches are necessary to gain a detailed, unbiased picture of upstream and downstream pathways in human skin that are likely implicated in morphea pathogenesis, particularly dysregulated IFN gamma mediated pathways, which appear particularly promising.

• #4 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. It is also known as localised scleroderma. The term 'scleroderma’ covers various types of morphoea and systemic sclerosis. […] The precise cause of morphoea is unknown. […] The diagnosis of morphoea is often made clinically, without the need for further tests. Biopsy, blood tests and imaging may be undertaken if the diagnosis or extent of disease is unclear. […] A skin biopsy enables the skin, subcutaneous fat (and sometimes fascia) to be examined under the microscope. Classic findings in morphoea include sharply squared-off biopsy (cookie-cutter sign), atrophic epidermis, thickened, hyalinised collagen, with possible loss of appendageal structures (such as sweat glands and hair follicles), fat loss / entrapment, and inflammatory cell infiltrate (lymphocytes +/- plasma cells).

• #5 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. It is also known as localised scleroderma. The term 'scleroderma’ covers various types of morphoea and systemic sclerosis. […] The precise cause of morphoea is unknown. […] The diagnosis of morphoea is often made clinically, without the need for further tests. Biopsy, blood tests and imaging may be undertaken if the diagnosis or extent of disease is unclear. […] A skin biopsy enables the skin, subcutaneous fat (and sometimes fascia) to be examined under the microscope. Classic findings in morphoea include sharply squared-off biopsy (cookie-cutter sign), atrophic epidermis, thickened, hyalinised collagen, with possible loss of appendageal structures (such as sweat glands and hair follicles), fat loss / entrapment, and inflammatory cell infiltrate (lymphocytes +/- plasma cells).

• #6 Histopathologic study of morphea – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/histopathologic-study-of-morphea/

  Morphea (localised scleroderma) is a rare inflammatory fibrosing disease of the skin. Patterns of sclerosis and degree of inflammation are known to have clinical implications such as increased functional limitations. Histopathology of morphea has received little attention in India. […] Cases diagnosed based on clinical suspicion and histopathologic confirmation from 2012 to 2020 were included. Skin biopsies were studied for histopathologic features. Patterns of arrangement of lymphocytes were categorised as scattered interstitial, Indian file and lymphoid aggregate. […] The most common clinical subtype was the plaque type (n = 32, 43%). Dermal sclerosis, which was present in all cases, was either top-heavy, bottom-heavy, full thickness or rarely patchy or focal. […] The presence of top-heavy sclerosis supports the concept that morphea may be limited to the superficial reticular dermis, known as superficial morphea. Diminished skin adnexal structures were consistent findings.

• #7 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea is characterised by an area of inflammation and fibrosis (thickening and hardening) of the skin due to increased collagen deposition. It is also known as localised scleroderma. The term 'scleroderma’ covers various types of morphoea and systemic sclerosis. […] The precise cause of morphoea is unknown. […] The diagnosis of morphoea is often made clinically, without the need for further tests. Biopsy, blood tests and imaging may be undertaken if the diagnosis or extent of disease is unclear. […] A skin biopsy enables the skin, subcutaneous fat (and sometimes fascia) to be examined under the microscope. Classic findings in morphoea include sharply squared-off biopsy (cookie-cutter sign), atrophic epidermis, thickened, hyalinised collagen, with possible loss of appendageal structures (such as sweat glands and hair follicles), fat loss / entrapment, and inflammatory cell infiltrate (lymphocytes +/- plasma cells).

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