Medicine:Eosinophilic fasciitis

Eosinophilic fasciitis
Other names	Shulman syndrome

Eosinophilic fasciitis (/ˌiːəˌsɪnəˈfɪlɪk ˌfæʃiˈaɪtɪs, ˌiːoʊ-, -ˌfæsi-/), also known as Shulman's syndrome or diffuse fasciitis with eosinophilia, is an inflammatory disease that affects the fascia, other connective tissues, surrounding muscles, blood vessels and nerves. Unlike other forms of fasciitis, eosinophilic fasciitis is typically self-limited and confined to the arms and legs, although it can require treatment with corticosteroids, and some cases are associated with aplastic anemia.

The condition was first characterized by Shulman in 1974, but it is not yet known whether it is actually a distinct condition or merely a variant presentation of another syndrome. The presentation is similar to that of scleroderma or systemic sclerosis. However, unlike scleroderma, eosinophilic fasciitis affects the deeper fascial layers, rather than the dermis; the characteristic and severe effects of scleroderma and systemic sclerosis, such as Raynaud's syndrome, involvement of the extremities, prominent small blood vessels (telangiectasias), and visceral changes such as swallowing problems, are absent. Nevertheless, the term remains used for diagnostic purposes.

Epidemiology

This is a very rare disease with only about 100 cases reported. The typical age of onset is around 20 to 60 years of age, although cases in children have been observed. Its prevalence is slight more common in men compared to women with a 1.5:1 ratio, although more studies still need to be done.

Causes

Most cases are idiopathic, however some have been associated with:

• strenuous exercise,
• initiation of hemodialysis,
• infection with Borrelia burgdorferi,
• certain medications, such as statins, phenytoin, ramipril, and subcutaneous heparin
• other autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus, and Sjogren syndrome
• hematologic disorders such as aplastic anemia, multiple myeloma, chronic lymphocytic leukemia, other leukemias and lymphomas

Signs and symptoms

Because the disease is rare and clinical presentations vary, a clear set of symptoms is difficult to define. The main symptoms are pain and swelling to the distal extremities. Initial presentation usually involves symmetrical swelling and thickening of the skin to the forearms and lower legs, with associated pain and erythema. The hands and feet are typically spared. As the disease progresses, the skin texture starts puckering and gives an "orange peel" appearance. Systemic symptoms such as fever and generalized fatigue can also present. Other common features include fibrosis of the skin, joint pain, and secondary carpal tunnel syndrome due to compression of the median nerve.

Diagnosis

Since this condition has many similarities with other autoimmune diseases, the first step is to exclude other conditions such as systemic sclerosis. The key to diagnosis is the observation of skin changes in combination with eosinophilia, but the most accurate test is a biopsy of skin, fascia, and muscle.

Major criterion

• symmetrical "plate-like" sclerotic lesions in the distal extremities (with no signs of Raynaud phenomenon and exclusion of systemic sclerosis)

Minor criteria

• histology of a skin biopsy shows fibrosis of the subcutaneous connective tissue, with thickening of the fascia and eosinophilic infiltration
• Imaging tests such as magnetic resonance imaging (MRI) shows thickening of the fascia

Definitive diagnosis is made if the patient meets the major criterion and at least 1 of the 2 minor criteria.

Treatment

First-line treatment include oral corticosteroid therapy. Most patients have a favorable course as the dose is tapered gradually in 1–2 years. Second-line include therapy include disease-modifying anti-rheumatic drug (DMARD) such as hydroxychloroquine, methotrexate, cyclosporine, cyclophosphamide. Patients usually have good prognosis with early initiation of treatment if there is no visceral involvement.

See also

• Eosinophilia
• List of cutaneous conditions

Further reading

• "Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases". Seminars in Arthritis and Rheumatism 17 (4): 221–231. May 1988. doi:10.1016/0049-0172(88)90008-X. PMID 3232080. 
• "Eosinophilic fasciitis. A pathologic study of twenty cases". The American Journal of Pathology 96 (2): 493–518. August 1979. PMID 474708. 
• "Eosinophilic fasciitis". The Medical Clinics of North America 73 (5): 1157–1166. September 1989. doi:10.1016/S0025-7125(16)30625-3. PMID 2671538. 
• "Eosinophilic fasciitis: report of 15 cases". Mayo Clinic Proceedings 56 (1): 27–34. January 1981. PMID 7453247. 
• "Eosinophilic fasciitis. Case report and review of the literature". Annals of the Rheumatic Diseases 36 (4): 354–359. August 1977. doi:10.1136/ard.36.4.354. PMID 901033. 
• "Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis". Journal of the American Academy of Dermatology 17 (4): 648–656. October 1987. doi:10.1016/S0190-9622(87)70251-5. PMID 3668010. 
• "Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders". Current Opinion in Rheumatology 9 (6): 562–570. November 1997. doi:10.1097/00002281-199711000-00013. PMID 9375286. 
• "Eosinophilic fasciitis: clinical, laboratory, and microscopic considerations". Arthritis and Rheumatism 24 (5): 677–683. May 1981. doi:10.1002/art.1780240508. PMID 6972219. 
• "Eosinophilic fasciitis: spectrum of MRI findings". AJR. American Journal of Roentgenology 184 (3): 975–978. March 2005. doi:10.2214/ajr.184.3.01840975. PMID 15728627. 

External links

Classification	D ICD-10: M35.4 ICD-9-CM: 728.89 OMIM: 226350 DiseasesDB: 29427
External resources	MedlinePlus: 000447 eMedicine: med/686