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International Journal of Molecular... Jan 2023Eosinophilic fasciitis (EF), defined as diffuse fasciitis with eosinophilia by Shulman in 1974, is a disease with unknown etiology and whose pathogenesis is still being... (Review)
Review
Eosinophilic fasciitis (EF), defined as diffuse fasciitis with eosinophilia by Shulman in 1974, is a disease with unknown etiology and whose pathogenesis is still being researched. The diagnosis is based on the clinical aspects (skin induration with an "orange peel" appearance), the lab results (eosinophilia, increased inflammatory markers), the skin biopsy with the pathognomonic histopathological result, as well as the typical MRI changes. The treatment includes glucocorticoids and immunosuppressive drugs. Due to severe refractory cases, the treatment remains a challenge. EF is still a disease with potential for further research.
Topics: Humans; Fasciitis; Eosinophilia; Skin; Immunosuppressive Agents
PubMed: 36768300
DOI: 10.3390/ijms24031982 -
American Journal of Clinical Dermatology Aug 2017Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which... (Review)
Review
Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud's phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. 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. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.
Topics: Administration, Cutaneous; Administration, Oral; Algorithms; Biopsy; Calcitriol; Dermatologic Agents; Diagnosis, Differential; Disease Progression; Drug Therapy, Combination; Eosinophilia; Evidence-Based Medicine; Fasciitis; Glucocorticoids; Humans; Methotrexate; Phototherapy; Practice Guidelines as Topic; Randomized Controlled Trials as Topic; Scleroderma, Localized; Skin; Tacrolimus; Treatment Outcome; United States
PubMed: 28303481
DOI: 10.1007/s40257-017-0269-x -
Allergology International : Official... Oct 2019Eosinophilic fasciitis is a disease originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. The patients with this disease often have history of... (Review)
Review
Eosinophilic fasciitis is a disease originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. The patients with this disease often have history of strenuous exercise or labor a few days to 1-2 weeks before the onset. The chief symptoms are symmetrical, full-circumference swelling and plate-like hardness of the distal limbs. This is accompanied by redness and pain in the early stages, with many cases exhibiting systemic symptoms such as fever or generalized fatigue. The lesions have been observed extending to the proximal limbs, though never on the face or fingers. En bloc biopsies from the skin to the fascia show marked fascial thickening and inflammatory cell infiltration by the lymphocytes and plasma cells. Eosinophilic infiltration is useful for the diagnosis but is only seen in the early stages of the disease. Recently, "Diagnostic criteria, severity classification, and clinical guidelines for eosinophilic fasciitis" were published. This review article discusses about eosinophilic faciitis in detail, from its pathophysiology to the treatment.
Topics: Biopsy; Cytokines; Disease Management; Disease Susceptibility; Eosinophilia; Fasciitis; Humans; Phenotype; Skin
PubMed: 30910631
DOI: 10.1016/j.alit.2019.03.001 -
Actas Dermo-sifiliograficas Oct 2013Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes... (Review)
Review
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. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach.
Topics: Algorithms; Aminoquinolines; Clinical Trials as Topic; Eosinophilia; Fasciitis; Glucocorticoids; Humans; Imiquimod; Immunosuppressive Agents; Methotrexate; Photochemotherapy; Physical Therapy Modalities; Recurrence; Scleroderma, Localized; Severity of Illness Index
PubMed: 23948159
DOI: 10.1016/j.adengl.2012.10.012 -
Postepy Dermatologii I Alergologii Oct 2021Morphea, also known as localized scleroderma, is a chronic, autoimmune disease of connective tissue. It is characterized by a typical clinical feature. In morphea, there... (Review)
Review
Morphea, also known as localized scleroderma, is a chronic, autoimmune disease of connective tissue. It is characterized by a typical clinical feature. In morphea, there is no Raynaud's phenomenon, no sclerodactyly or no ulcerations on the fingertips. Although morphea and systemic sclerosis have been perceived as separate disease entities for years, they are still confused both by patients (which is a source of unnecessary stress) and doctors. This may be due to, in part, misunderstood terminology. The controversy around morphea also concerns the division of this disease entity, including its less common subtypes, such as eosinophilic fasciitis. Discussions also revolve around the diagnostic aspects and possible treatment options. The paper attempts to present the debatable aspects regarding nomenclature, classification, diagnosis and treatment of morphea.
PubMed: 34849114
DOI: 10.5114/ada.2021.106242 -
Anais Brasileiros de Dermatologia 2016Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with...
Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with a variable infiltration of eosinophils. Peripheral eosinophilia, poly or monoclonal hypergammaglobulinemia and increased erythrocyte sedimentation rate can be seen. Clinical features begin acutely, with local edema and a painful and symmetrical stiffening of the limbs, progressing rapidly to fibrosis, which can limit joint movements. Some cases have a history of strenuous physical exercise or trauma. The diagnosis is confirmed by a deep skin biopsy. Glucocorticoids in high doses is the treatment of choice. We report a typical eosinophilic fasciitis case with peripheral eosinophilia and dramatic response to pulse therapy with methylprednisolone.
Topics: Adult; Biopsy; Eosinophilia; Extremities; Fasciitis; Glucocorticoids; Humans; Magnetic Resonance Imaging; Male; Methylprednisolone; Pulse Therapy, Drug; Skin; Syndrome
PubMed: 28300895
DOI: 10.1590/abd1806-4841.20164683 -
British Medical Journal Feb 1980
Topics: Eosinophils; Fascia; Female; Humans; Male; Scleroderma, Localized; Scleroderma, Systemic
PubMed: 7370561
DOI: No ID Found -
Postepy Higieny I Medycyny... Jan 2015Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is... (Review)
Review
Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is characterized by fibrosis of the skin and subcutaneous tissues with significant thickening of fascia. Visceral involvement is rare. Characteristic feature in laboratory tests is peripheral blood eosinophilia. Differential diagnosis should be performed, including ruling out systemic sclerosis, nephrogenic systemic fibrosis, eosinophilia-myalgia syndrome, scleromyxedema, hypereosinophilic syndrome or Churg-Strauss syndrome. Final diagnosis is confirmed by histopathological examination. In treatment of the disease corticosteroids and/or immunosuppressive drugs are used. Some other drugs showed activity in this disease e.g. dapsone, infiximab or rituximab. Prognosis is rather good but sometimes a long-term treatment is necessary. In this paper we summarized the current knowledge on eosinophilic fasciitis.
Topics: Diagnosis, Differential; Eosinophilia; Fasciitis; Glucocorticoids; Humans; Immunosuppressive Agents; Prognosis
PubMed: 25897110
DOI: 10.5604/17322693.1149872 -
Current Treatment Options in... Mar 2016Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin...
Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated.
PubMed: 28473954
DOI: 10.1007/s40674-016-0038-7 -
Cureus Jun 2022Eosinophilic fasciitis (EF) is a rare ailment that affects the immune system. Due to the rarity of this condition, there are few clear diagnostic criteria for...
Eosinophilic fasciitis (EF) is a rare ailment that affects the immune system. Due to the rarity of this condition, there are few clear diagnostic criteria for clinicians to focus on. This may lead to significant delays in reaching a diagnosis and offering proper treatment, and patients may end up seeing multiple different specialists. This is especially true in a free clinic setting where continuity of care, follow-up, and specialist access are usually lacking. In this report, we describe a case of a 24-year-old white male who presented with sudden onset of redness, swelling, burning, and pain in the bilateral upper and lower extremities. Through arduous workup and testing, he was found to have increased eosinophils in peripheral blood, elevated levels of white blood cell count, increased C-reactive protein, and pathological changes in the tissue showing eosinophil and lymphocyte infiltration. We shed light on the relative rarity of this condition and its similar clinical characteristics to various dermatological/rheumatological disease processes. We also highlight how a free clinic can provide high-quality healthcare to bridge gaps in access to care by providing high-quality and broad specialist access while ensuring continuity of care.
PubMed: 35812539
DOI: 10.7759/cureus.25668