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Eosinophilic Fasciitis (EF) or Shulman's Syndrome

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
What is Eosinophilic Fasciitis?
Scleroderma and EF
Symptoms of Eosinophilic Fasciits
Diagnosis of Eosinophilic Fasciitis
Research about Eosinophilic Fasciitis
Personal Stories of Eosinophilic Fasciitis

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

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.

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].

(Case Report) Eosinophilic fasciitis/generalized morphea overlap. A 50-year-old woman presented with a three-month history of violaceous, non-tender, indurated plaques on the chest, abdomen, breasts, and proximal portions of the arms and legs. An incisional biopsy specimen showed changes consistent with a diagnosis of inflammatory morphea. Heidary N. (PubMed) Dermatol Online J., [2009].

Symptoms of Eosinophilic Fasciitis

Eosinophilic fasciitis (Shulman's disease): A case series of 11 patients. Diagnosis of eosinophilic fasciitis should be considered in the presence myalgia and subcutaneous induration of limbs, blood eosinophilia and hypergammaglobulinemia. Treatment is based on systemic corticosteroids. Naoui A. (PubMed) Rev Med Interne, 2010 May 19.

Diagnosis

Unusual presentation of eosinophilic fasciitis: two case reports and a review of the literature. Eosinophilic fasciitis can present with various symptoms. When patients exhibit eosinophilia, arthralgia and myalgia, eosinophilic fasciitis should be considered as a possible diagnosis. Danis R. J Med Case Reports, 2010 Feb 8;4:46.

Research

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. (PubMed) Arch Dermatol. 2009 Sep;145(9):1017-22.

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. (PubMed) J Clin Rheumatol. 2009 Aug;15(5):247-9.

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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