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Eosinophilic Fasciitis (EF) or Shulman's Syndrome

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Scleroderma and EF
Symptoms
Diagnosis
Research
Treatment
Personal Stories

Overview: What is Eosinophilic Fasciitis (Shulman's Syndrome)?

Eosinophilic Fasciitis KaleidoscopeEosinophilic Fasciitis is a very rare scleroderma-like illness. It causes a symmetrical hardening of the muscles, fascia, and skin. Unlike scleroderma, it spares the fingers and toes, exhibits elevated blood eosinophils, often onsets after prolonged strenuous exercise, and responds to treatment with systemic corticosteroids.

Eosinophilic Fasciitis is a scleroderma-like disorder characterized by symmetric and painful inflammation, swelling, and induration involving arms and legs but not the most acral areas involved in scleroderma. Merck. (Also see What is Scleroderma?)

Eosinophilic Fasciitis is a syndrome characterized by tenderness and swelling of the extremities caused by inflammation of the fascia and muscles. Henry Ford Health System.

Eosinophilic Fasciitis (Shulman's Syndrome). Eosinophils are a particular type of white blood cells, usually representing a small percentage (less than 8% of the total white blood cell population). The number of these cells (eosinophil count) increases in certain illnesses, including allergies, asthma, Addison's disease, sarcoidosis, parasite infections, drug reactions, and connective tissue diseases (such as rheumatoid arthritis and scleroderma). MedicineNet.com

Scleroderma and Eosinophilic Fasciitis

Eosinophilic Fasciitis Since 1974, more than 200 patients with EF have been reported, and debates still are ongoing as to whether EF represents a variant of scleroderma. Medscape.

Eosinophilic fasciitis, morphea and vitiligo in a single patient. The association of morphea/fasciitis with eosinophilia is a classical finding; the presence of vitiligo raises the question of possible association between these different disorders. PubMed, Ann Dermatol Venereol, 2014 Oct;141(10):598-602. (Also see Conditions Associated with Morphea)

Unilateral Eosinophilic Fasciitis (EF): An Under-Recognized Subtype? It is possible that unilateral EF is under-recognized and can easily be misdiagnosed as another scleroderma variant if a full thickness biopsy is not reviewed by a dermatopathologist. R.S. Daniel, MD. Journal of Clinical Rheumatology, [2009].

Symptoms of Eosinophilic Fasciitis

Finger stiffness or edema as presenting symptoms of eosinophilic fasciitis. Finger symptoms of patients with EF might be caused by fasciitis of the forearms, which leads to dysfunction of the long finger flexors and extensors as well as slight edema of hands. PubMed, Rheumatol Int, 08/07/2015.

Eosinophilic fasciitis associated with myositis. Eosinophilic fasciitis is clinically characterized by symmetrical scleroderma-like indurations of the skin with pain. The histological features are fascial inflammation with lymphocytes and eosinophils as well as thickened and fibrotic fascia. PubMed, Case Rep Dermatol, 2015 Apr 30;7(1):79-83. (Also see Myopathy and Myositis)

Diagnosis

Eosinophilic fasciitis(EF) (Shulman's disease): Diagnostic and therapeutic review. The diagnosis of EF is often based on the association of characteristic skin or subcutaneous abnormalities and a thickened fascia with an inflammatory infiltration, mostly composed of lymphocytes and eosinophils. PubMed, Rev Med Interne, 09/15/2015.

Diagnosis and classification of eosinophilic fasciitis (EF). The diagnosis of EF can be established by clinical, laboratory and histological findings, but universally accepted international diagnostic criteria are lacking. PubMed, Autoimmun Rev. 2014 Apr-May;13(4-5):379-82.

Research

Overlapping gene expression profiles indicative of antigen processing and the interferon pathway characterize inflammatory fibrotic skin diseases. This review addresses whether there is evidence for different underlying molecular pathways in the various inflammatory fibrotic diseases such as localized scleroderma, pediatric lichen sclerosus, adult lichen sclerosus, eosinophilic fasciitis and systemic sclerosis. PubMed, Expert Rev Clin Immunol, 2014 Feb;10(2):231-41.

Psychological distress in patients with morphea and eosinophilic fasciitis. Physical and psychosocial aspects play a substantial role in the quality of life for patients with morphea. Physicians should be encouraged to assess the physical and psychosocial factors when treating patients with sclerotic skin diseases. This approach could improve quality of life and ultimately lead to improved dermatological treatment outcomes. Kroft EB, Arch Dermatol, [2009].

Unilateral eosinophilic fasciitis: an under-recognized subtype? Symmetric skin thickening of the limbs with deep fascial inflammation is the hallmark of eosinophilic fasciitis. We describe a woman who presented with unilateral progressive skin thickening. Daniel RS, J Clin Rheumatol, [2009].

Treatment

Dermatologic Manifestations of Eosinophilic Fasciitis Treatment & Management. Many eosinophilic fasciitis cases respond to corticosteroids (88%, with 25% obtaining complete recovery), although spontaneous resolution is possible. Complete recovery may take up to 1-3 years. This article includes adjunctive and newer therapies for EF. WebMD.

Efficacy of Tocilizumab in the treatment of Eosinophilic fasciitis: Report of one case. This report describes for the first time the efficacy of an anti interleukin-6 agent in Eosinophilic fasciitis treatment. PubMed, Joint Bone Spine, 07/07/2015. (Also see Clinical Trials)

Eosinophilic fasciitis (EF): clinical characteristics and response to methotrexate (MTX). MTX represents an effective treatment option for EF although the rarity of this disease would make a double-blind controlled trial study difficult to perform. PubMed, Int J Rheum Dis, 2015 Jan;18(1):91-8. (Also see Immunosuppressants)

Personal Stories

Beth R: Eosinophilic Fasciitis My arms and legs are still tight and I still cannot make a fist…

Claude: Eosinophilic Fasciitis I am a forty-four-year-old male from Ottawa, Canada. In the late spring, early summer of 2003, my wife noticed some red splotches on my lower legs…

Gerry Fitzpatrick: Eosinophilic Fasciitis My hands have become very weak and with all my other medical problems, this one is really depressing me the most…

Gillian: Eosinophilia-Myalgia Syndrome or Eosinophilic Fasciitis My arms and legs still feel really hard and are very inflexible. I can't fully open my hands or point my toes…

Henry: Eosinophilic Fasciitis The disease then grabbed hold and started spreading like wild fire…

Jennifer Foster: Eosinophilic Fasciitis (Ontario, Canada) My husband has been extraordinarily accepting of the rapid deterioration of my once charming and petite figure…

Jenny H: Eosinophilic Fasciitis My legs started changing appearance to a mottled red and white color, and looked like they were made of plastic…

Jessica N: Eosinophilic Fasciitis Sometimes I still worry about me and how my parents are dealing with this…

John: Eosinophilic Fasciitis or Scleroderma-Like Condition I have knee contractures that prevent me from walking, very tight skin in the legs…

Jon T: Eosinophilic Fasciitis I am stumped as to the possibility for me to return to normal duties at work, and what the normal prognosis for someone with this disease can expect?

Kathleen S: Eosinophilic Fasciitis I noticed that winter during gym, that when we did push-ups, I had to do knuckle push-ups because I could not straighten my fingers…

Karen H: Eosinophilic Fasciitis I am experiencing extreme fatigue and muscle pain on a daily basis…

Keith H: Eosinophilic Fasciitis (EF) I have a rare skin disease called eosinophilic fascitiis, a skin and muscle disease which causes tightness of the skin, and sometimes a reddish coloration of the skin in areas…

Lindsey: Eosinophilic Fasciitis (EF) My skin remains very thick and constricted…

Lori S: Mother of Eosinophilic Fasciitis Patient On the second biopsy, they went further into the fascia and confirmed the diagnosis of eosinophilic fasciitis (EF). I would like to talk to other parents or patients with EF…

Susan H: Eosinophilic Fasciitis While I was in the hospital and starting dialysis, my right arm became hard as a rock and swollen…

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