Materiały źródłowe

• #1 Morphea – StatPearls – NCBI Bookshelf

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

  Morphea is a rare skin condition, and the incidence rates vary from 3.4 to 27 per 100,000, and women are more susceptible than men. Women have higher rates of morphea at 2.5 to 5:1 compared to men and are usually affected in the fourth decade of life. […] The peak incidence of morphea in children is reported to happen between 7 and 11 years. […] Morphea incidence is reported to be 2-3 per 100,000 in the United States. Morphea is reported to occur more frequently in Whites compared to the African American population.

• #2 Morphea: Background, Pathophysiology, Etiology

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

  The incidence of morphea has been estimated as approximately 0.4-2.7 cases per 100,000 people. The actual incidence is likely higher because many cases may not come to medical attention. […] Two thirds of adults with morphea present with plaque/superficial circumscribed lesions, with generalized, linear, and deep variants each accounting for approximately 10% of cases. Up to half of all cases of morphea occur in pediatric patients. In this group, linear morphea predominates (two thirds of cases), followed by the plaque/superficial circumscribed (25%) and generalized (5%) subtypes. Of note, as many as half of patients with linear morphea have coexistent plaque-type lesions. […] Although morphea occurs in persons of all races, it appears to be more common in White individuals, who comprise 73-82% of patients seen.

• #2 Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21238823/

  Morphea, also known as localized scleroderma, is a rare fibrosing disorder of the skin and underlying tissues. […] The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993.

• #2 Morphea: Background, Pathophysiology, Etiology

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

  Women are affected approximately three times as often as men for all forms of morphea except the linear subtype, which only has a slight female predominance. Morphea may appear or worsen during pregnancy. […] Linear morphea commonly manifests in children and adolescents, with two thirds of cases occurring before age 18 years. Other morphea subtypes have a peak incidence in the third and fourth decades of life.

• #3 Morphea – Wikipedia

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

  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. Adequate studies on the incidence and prevalence have not been performed. Morphea also may be under-reported, as physicians may be unaware of this disorder, and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist.

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

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

  Morphea is relatively uncommon, with an annual incidence of 2.7 per 100,000 population. […] It predominates in women, with a ratio of 2.6:1; and although it affects all ethnicities, it predominates in the Caucasian population. In 75 to 90 % of patients it occurs between the ages of 20 and 50 years. […] It is possible that due to the delay in diagnosis, the true prevalence of morphea is underestimated. […] Morphea etiology and pathogenesis are not yet fully understood. Autoimmune, environmental factors and various events that trigger the production of cytokines are thought to be involved, including infections, radiation or trauma. […] There is an unproven hypothesis that morphea may be due to post-cygotic mosaicism by the blaschkoid or dermatomic distribution of many lesions. […] Diagnosis and management of patients with morphea is not always easy and can be a challenge for the dermatologist.

• #3 Update on the Classification and Treatment of Localized Scleroderma | Actas Dermo-Sifiliográficas

  https://actasdermo.org/es-update-on-classification-treatment-localized-articulo-S157821901300173X

  Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. […] It is a rare disease, with an incidence that varies between 0.34 and 2.7 cases per 100,000 population per year. […] It is more common in white women (female to male ratio between 2.4:1 and 4.2:1) and its prevalence is similar in children and adults. […] Although serious systemic repercussions only develop in very rare cases of localized scleroderma, the disease can be a source of considerable morbidity as the lesions often affect the face and limbs and can lead to marked deformity and significant physical disability.

• #3 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. […] Laboratory tests are not considered necessary for the diagnosis or follow-up of patients with morphea. […] Due to the frequency of extracutaneous alterations, especially in patients with linear morphea, it is important to deliberately look for them through patient interrogation and consultation with the ophthalmology, neurology or orthopedics departments, in addition to relevant paraclinical tests. […] 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. […] 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. […] Approximately 30 % of patients with linear morphea may have extracutaneous, neurological, articular or ophthalmological lesions, which should be deliberately searched and discarded.

• #4 Update on the Classification and Treatment of Localized Scleroderma | Actas Dermo-Sifiliográficas

  https://actasdermo.org/es-update-on-classification-treatment-localized-articulo-S157821901300173X

  Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. […] It is a rare disease, with an incidence that varies between 0.34 and 2.7 cases per 100,000 population per year. […] It is more common in white women (female to male ratio between 2.4:1 and 4.2:1) and its prevalence is similar in children and adults. […] Although serious systemic repercussions only develop in very rare cases of localized scleroderma, the disease can be a source of considerable morbidity as the lesions often affect the face and limbs and can lead to marked deformity and significant physical disability.

• #5 Morphea: Background, Pathophysiology, Etiology

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

  The incidence of morphea has been estimated as approximately 0.4-2.7 cases per 100,000 people. The actual incidence is likely higher because many cases may not come to medical attention. […] Two thirds of adults with morphea present with plaque/superficial circumscribed lesions, with generalized, linear, and deep variants each accounting for approximately 10% of cases. Up to half of all cases of morphea occur in pediatric patients. In this group, linear morphea predominates (two thirds of cases), followed by the plaque/superficial circumscribed (25%) and generalized (5%) subtypes. Of note, as many as half of patients with linear morphea have coexistent plaque-type lesions. […] Although morphea occurs in persons of all races, it appears to be more common in White individuals, who comprise 73-82% of patients seen.

• #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 is relatively uncommon, with an annual incidence of 2.7 per 100,000 population. […] It predominates in women, with a ratio of 2.6:1; and although it affects all ethnicities, it predominates in the Caucasian population. In 75 to 90 % of patients it occurs between the ages of 20 and 50 years. […] It is possible that due to the delay in diagnosis, the true prevalence of morphea is underestimated. […] Morphea etiology and pathogenesis are not yet fully understood. Autoimmune, environmental factors and various events that trigger the production of cytokines are thought to be involved, including infections, radiation or trauma. […] There is an unproven hypothesis that morphea may be due to post-cygotic mosaicism by the blaschkoid or dermatomic distribution of many lesions. […] Diagnosis and management of patients with morphea is not always easy and can be a challenge for the dermatologist.

• #6

  https://link.springer.com/article/10.1007/s40257-017-0269-x

  The rarity of morphea is reflected in the annual incidence rates which are reported to be between 3.4 and 27 cases per 1,000,000. Females are more frequently affected than males (ratio: 2.4-5.0 to 1). The peak incidence is bimodal with peaks between 7 and 11 years for pediatric-onset disease and 44-47 years for adult-onset disease. The incidence and prevalence of EF is unknown. The disease predominantly affects patients in their fourth and fifth decade of life. Only a few case reports describe childhood-onset disease. […] Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. […] Disease monitoring by reliable and sensitive outcome measures is improved by the localized scleroderma cutaneous assessment tool (LoSCAT); however, especially for deep involvement, additional validated outcome measures are required.

• #7 Morphea and a case with fasciitis in the hands – Journal of Turkish Society for Rheumatology

  https://www.raeddergisi.org/articles/morfea-ve-ellerde-fasiit-gelisen-bir-olgu/doi/raed.galenos.2020.18480

  The epidemiology of morphoea (localised scleroderma) in Olmsted County 1960-1993. J Rheum 1997;24:73-80. […] The epidemiology of morphoea (localised scleroderma) in Olmsted County 1960-1993. J Rheum 1997;24:73-80.

• #8 Morfea: revisión práctica de su diagnóstico, clasificación y tratamiento

  https://www.medigraphic.com/cgi-bin/new/resumen.cgi?IDARTICULO=90083

  La morfea o esclerodermia localizada es una enfermedad poco comn del tejido conectivo que se manifiesta con esclerosis localizada de la piel y, en algunos casos, con lesiones extracutneas. […] Su etiologa no se comprende por completo, pero se cree que hay predisposicin gentica, adems de factores ambientales desencadenantes. […] En esta revisin resumimos los aspectos prcticos ms importantes de la clasificacin, mtodos diagnsticos y de evaluacin de actividad en morfea, as como las opciones teraputicas disponibles, con nfasis en la evidencia clnica existente respecto a su eficacia y seguridad. […] Peterson L, Nelson A, Su W, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol. 1997; 24:73-80. […] Zulian F, Athreya BH, Laxer RM, Nelson AM, Feitosa-De Oliveira SK, Punaro MG, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45:614-620. […] Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006; 18:606-613.

• #9 Pathology Outlines – Morphea and systemic sclerosis

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

  Localized scleroderma […] Incidence 0.4 – 2.7 cases per 100,000 persons (J Rheumatol 1997;24:73, Rheum Dis Clin North Am 2002;28:603) […] F:M = 2.6 – 6:1, except in linear morphea (no sex predominance) (Br J Rheumatol 1988;27:286) […] Systemic sclerosis […] Incidence: 0.3 – 2.8 per 100,000 per year (J Eur Acad Dermatol Venereol 2017;31:1401) […] African Americans > Caucasians (G Ital Dermatol Venereol 2018;153:208) […] F:M = 3:1 (J Eur Acad Dermatol Venereol 2017;31:1401) […] Positive family history increases the relative risk twelvefold (J Eur Acad Dermatol Venereol 2017;31:1401) […] Higher severity has been suggested in African American women (G Ital Dermatol Venereol 2018;153:208) […] Older age and male gender are associated with poor outcomes (Semin Arthritis Rheum 2014;44:208)

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

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

  Morphea is a rare autoimmune connective tissue disease, and its incidence appears to be underestimated. 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. Based on retrospective studies, morphea appears to be more common in Caucasians. The incidence is similar in children and adults. 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. […] Imaging methods can be used to monitor morphea. Ultrasonography is useful for assessing the extent and depth of the disease process and enables measurement of the thickness of the dermis and adipose tissue. High-frequency ultrasonography (HF-USG) can be used to monitor the dynamics of the disease process. In the sclerosis phase, an increase in echogenicity and thickening of the skin is observed. In the inflammatory phase, an echogenicity decrease is observed. Salgueiro et al. observed an ultrasound symptom called the “sun sign”. This is a hyperechoic halo surrounding the superficial subcutaneous veins of the limbs in cross-section. There are interesting anecdotal data on the use of several other noninvasive methods like infrared thermography (IRT, skin temperature), laser Doppler imaging (LDI, skin blood flow) and multispectral imaging (MSI, oxygenation) in morphea, although their use requires further research.

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

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

  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.

• #11 Orphanet: Localized scleroderma

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

  The incidence of localized scleroderma has been estimated at 1/37,000-625,000 people per year depending on the country. Women are predominantly affected (F/M sex ratio around 3:1). The mean age at diagnosis ranges from 8 to 47 years. In two-thirds of cases, it occurs in adults. Localized scleroderma is called ”juvenile” when occurring in childhood (peak incidence between 7 and 11 years). […] Prognosis is usually favorable within 5 years but some patients may present with chronic disease and recurrences. Localized scleroderma is only rarely life threatening but can severely affect quality of life, particularly in children. Linear lesions tend to persist longer than plaque lesions.

• #12 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

  The registry was established in 2007 to learn more about morphea. Little is known about morphea since it is a rare, poorly understood, and understudied condition. This registry will provide a resource for investigators to make discoveries in regard to both the clinical and genetic aspects of the disease. […] 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.

• #13 Scleroderma and Raynaud Phenomenon | Clinical Gate

  https://clinicalgate.com/scleroderma-and-raynaud-phenomenon/

  Juvenile scleroderma is rare, with an estimated prevalence of 1/100,000. Localized scleroderma is far more common than SSc in children, by a 10 : 1 ratio, with plaque morphea and linear scleroderma being the most common subtypes. Linear scleroderma is predominantly a pediatric condition, with 65% of patients diagnosed before age 18. After age 8 yr the female : male ratio for both LS and SSc is approximately 3 : 1, whereas in patients younger than 8 yr there is no sex predilection. […] The epidemiology of morphea (localized scleroderma) in Olmsted county 1960-1993.

• #14 Morphea (Localized Scleroderma) | 5-Minute Pediatric Consult

  https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618251/all/Morphea__Localized_Scleroderma_?q=methotrexate

  Linear morphea is the most frequent subtype (~5065%), followed by circumscribed morphea (~25%), generalized morphea (58%), and pansclerotic (12%) morphea. […] Many patients (1520%) present with more than one subtype of morphea (mixed morphea). […] Localized scleroderma is approximately 10 times more common than systemic sclerosis in childhood. The annual age and sex adjusted incidence is 2.7 cases per 100,000.

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

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

  Morphea is defined by periods of activity (inflammation and fibrosis) which leads to damage and atrophy. 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. […] Given that unchecked disease activity in morphea can lead to severe cosmetic and functional sequelae, it is crucial to identify activity and initiate treatment early. Thus, recent progress has been made in developing and refining outcome measures in morphea. Clinical, biomarker, imaging, and histologic outcomes have all been studied in order to allow for more accurate assessment of disease activity and severity. Despite promising results in this arena, further work remains to thoroughly validate these measures before use in the clinical setting as well as for research purposes.

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

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

  Morphea, also known as localized scleroderma, is in an autoimmune disorder characterized by inflammation and sclerosis of the skin and underlying soft tissues. The estimated incidence of the disease is 0.4 to 2.7 per 100,000 people, although population based studies are lacking. Morphea affects adults and children equally, with females more susceptible to the disease than males. The pathogenesis of morphea is incompletely understood and is an evolving area of research. Studies suggest a multifactorial etiology involving dysregulated immune and fibrotic pathways, with additional contributing factors including genetic predisposition, traumatic or environmental factors, and vascular dysregulation. After many years of neglect, substantial progress has been made in morphea research. The purpose of this review is to summarize new developments in understanding the clinical manifestations of morphea and their evaluation as well as management. Despite these promising developments, further work is needed to better define clinical subtypes, extracutaneous manifestations, outcome measures and pathogenesis in order to better evaluate and treat patients with morphea.

• #16 Acquired Strabismus in Linear Scleroderma of the Face | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3238

  In general, the poor recognition of JLS clinical picture by primary care physicians accounts for a significant delay between disease onset and diagnosis and subsequent start of treatment and this probably negatively influences the course of disease. […] In conclusion, patients with LiS should be evaluated promptly as they can develop extracutaneous manifestations, such as in the current case, and clinicians should address proper investigations and prompt immunosuppressive treatment.

• #16 Acquired Strabismus in Linear Scleroderma of the Face | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3238

  LiS is a rare disease, with an estimated annual incidence of 0.42.7 per 100,000 individuals. […] The overall incidence of JLS is not known, but there is evidence that the majority of cases occur during childhood (mean age at onset 7.9 years). […] In a large study of 750 patients with JLS, 22.4% presented extracutaneous involvement, such as articular, neurological, vascular, ocular, gastrointestinal, respiratory, cardiac, and renal manifestations. […] Ocular involvement in JLS is rare (3.2%), mostly associated with linear subtype, and generally related to extension of the lesions to annexes, resulting in abnormalities of eyelashes, eyebrows and eyelids, xerophthalmia and enophthalmus; more rarely inflammatory manifestations, such as episcleritis and anterior uveitis, as well as glaucoma and papilledema have been observed.

• #17 Morphea – wikidoc

  https://www.wikidoc.org/index.php/Morphea

  Morphea is an uncommon condition that is thought to affect 1 in 100,000 people. […] Adequate studies on the incidence and prevalence have not been performed. […] Morphea also may be under-reported as physicians may be unaware of this disorder and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist. […] As in many other connective tissue or autoimmune disorders, morphea mainly involves women with a W:M ratio of 3:1.

• #18 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  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.

• #18 Morphoea (localised scleroderma, morphea)

  https://dermnetnz.org/topics/morphoea

  Morphoea is rare and is estimated to have an incidence of 13 per 100,000 children. It is three times more common in females compared to males and often begins in childhood. Although not an inherited disorder, certain HLA subtypes (HLA-DRB1*04:04 and HLA-B*37) are associated with an increased risk of morphoea. […] Up to 30% of patients with more severe types of linear or generalised morphoea can have extracutaneous non-specific inflammatory symptoms. These include: fatigue, lethargy, non-specific joint pain and/or inflammation (arthralgia, arthritis), muscle pain, reflux/heartburn, Raynaud phenomenon (cold hands with red/white/blue colour changes), eye dryness, irritation or blurred vision due to ocular involvement (most commonly episcleritis, anterior uveitis, keratitis) related or unrelated to the site of morphoea.

• #19 High-frequency ultrasonography (HFUS) as a useful tool in differentiating between plaque morphea and extragenital lichen sclerosus lesions

  https://www.termedia.pl/High-frequency-ultrasonography-HFUS-as-a-useful-tool-in-differentiating-between-plaque-morphea-and-extragenital-lichen-sclerosus-lesions,7,30910,1,1.html

  Morphea (localized scleroderma) is a rare, chronic inflammatory disease of the skin and subcutaneous tissues that progresses to sclerosis. […] Up to 38% of patients diagnosed with morphea suffer from genital LS as well, whereas extragenital LS was present in approximately 1.7% of patients with morphea. […] The association between morphea and LS remains controversial. […] High-frequency ultrasonography is a useful diagnostic modality in dermatology, which complements the diagnosis and monitoring of various disorders. […] Our preliminary study implies that HFUS may be useful in differentiating between plaque morphea and extragenital LS. Hyperechogenic, polycyclic entrance echo seems to be a characteristic ultrasonographic phenomenon in extragenital LS lesions, although further studies concerning this issue are necessary.

• #20 Morphea in Saudi Arabia, a Clinical Study of 64 Patients

  https://symbiosisonlinepublishing.com/dermatology/dermatology67.php

  The presence of ANA or antibodies to ssDNA and histones are unusual in patients with plaque-type morphea. They are more frequent in linear and generalized morphea, where ANA can be found in high titers in 40–80% of patients. About 40% of children and adolescents with morphea have elevated ANA titers. […] In conclusion, Morphea is an inflammatory disease characterized by excessive dermal and subcutaneous collagen production. This is the first study investigating the clinical types of morphea in Saudi patients.

• #21

  https://journals.lww.com/ijpm/fulltext/2020/63040/histopathology_of_morphea__sensitivity_of_various.18.aspx

  Morphea or localized scleroderma is characterized histopathologically by sclerosis, fibrosis, and atrophy of the skin and subcutaneous tissue. Its incidence varies between 427 per million per year. […] The present study is being undertaken to assess the sensitivity, specificity, positive predictive value, and negative predictive value of these tests in the diagnosis of morphea. […] The sensitivity of these tests in accurately diagnosing morphea was assessed. […] The presence of HEG in our study had the highest sensitivity, positive predictive value as well as negative predictive value. […] Thus, knowledge of these signs will help in the quick diagnosis of morphea and should be taught to dermatologists as well as pathologists.

• #22 Update on the Classification and Treatment of Localized Scleroderma | Actas Dermo-Sifiliográficas

  https://actasdermo.org/es-update-on-classification-treatment-localized-articulo-S157821901300173X

  Linear morphea, especially when the lesions are located on the head, is commonly associated with neurological complications (in almost 20% of cases) and ophthalmological complications (15%). […] Whatever the therapeutic regimen used, all types of morphea tend to progress and recur, particularly when onset occurs during childhood.

• #22 Update on the Classification and Treatment of Localized Scleroderma | Actas Dermo-Sifiliográficas

  https://actasdermo.org/es-update-on-classification-treatment-localized-articulo-S157821901300173X

  The most common systemic complications are joint pain and, when lesions affect the head, neurological and ocular manifestations. […] The cutaneous and systemic manifestations of generalized morphea overlap to a great extent with another disorder, eosinophilic fasciitis. […] The inclusion of eosinophilic fasciitis in the deep morphea group of Peterson et al.’s classification has been and continues to be a subject of debate in the medical literature. […] Most of the literature describing the clinical and microscopic characteristics of eosinophilic fasciitis has been published by rheumatologists, the specialists who mainly use this term. However, their descriptions include many of the cutaneous and extracutaneous features of the disease described as generalized or deep morphea in the dermatological literature.

• #23 Update on the Classification and Treatment of Localized Scleroderma | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-update-on-classification-treatment-localized-articulo-S157821901300173X

  Relatives of patients with morphea are also at higher risk of developing autoimmune diseases. […] The cutaneous and systemic manifestations of generalized morphea overlap to a great extent with another disorder, eosinophilic fasciitis. […] The inclusion of eosinophilic fasciitis in the deep morphea group of Peterson et al.’s classification has been and continues to be a subject of debate in the medical literature. […] Most of the literature describing the clinical and microscopic characteristics of eosinophilic fasciitis has been published by rheumatologists, the specialists who mainly use this term. However, their descriptions include many of the cutaneous and extracutaneous features of the disease described as generalized or deep morphea in the dermatological literature. […] It would appear likely that all these terms (generalized morphea, deep morphea, and eosinophilic fasciitis) in actual fact refer to the same clinical entity. […] Whatever the therapeutic regimen used, all types of morphea tend to progress and recur, particularly when onset occurs during childhood.

• #23 Update on the Classification and Treatment of Localized Scleroderma | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-update-on-classification-treatment-localized-articulo-S157821901300173X

  Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. […] It is a rare disease, with an incidence that varies between 0.34 and 2.7 cases per 100,000 population per year. […] It is more common in white women (female to male ratio between 2.4:1 and 4.2:1) and its prevalence is similar in children and adults. […] Although serious systemic repercussions only develop in very rare cases of localized scleroderma, the disease can be a source of considerable morbidity as the lesions often affect the face and limbs and can lead to marked deformity and significant physical disability.

• #24 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). […] The disease is likely more common in Caucasians (population-based studies are lacking) with an overall incidence of 2.7 per 100,000 people. […] Individuals with morphea tend to have a higher risk for other autoimmune diseases as well as increased prevalence of familial autoimmunity. […] The prevalence of morphea in childhood is not well established, but past reports suggest approximately 2/3 of linear morphea begins before age 18, and 20-30% of morphea overall begins in childhood. […] High prevalences of concomitant and familial autoimmune disease, systemic manifestations, and antinuclear antibody positivity in the generalized and possibly mixed subtypes suggest that these are systemic autoimmune syndromes and not skin-only phenomena.

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