Eosinophilic fasciitis

Eosinophilic fasciitis
Eosinophilic fasciitis
Classification and external resources
ICD-10 M35.4
ICD-9 728.89
OMIM 226350
DiseasesDB 29427
eMedicine med/686

Eosinophilic fasciitis (pronounced /ˌiː.ɵsɪnɵˈfiːlɪk ˌfæʃiˈaɪtɪs/) (also known as "Shulman's syndrome"[1]) is a form of fasciitis. It is distinguished from scleroderma primarily because the affected area is the fascia, not the dermis as in scleroderma. Also, unlike scleroderma, Raynaud's phenomenon and telangiectasia are not observed.

It was first characterized in 1974,[2] and it is not yet known whether it is actually a distinct condition or just a different presentation. However, it remains used for diagnostic purposes.

Several cases have been reported after strenuous exercise.

Contents

Symptoms

As it is a rare disease, a clear set of symptoms is difficult to define. Usually, patients show severe pain and swelling are reported but clinical presentations vary. It can have a 'orange peel' like appearance.[3] less common features are joint pain and carpal tunnel syndrome.

Treatment

Common treatments include corticosteroids[4] such as prednisone, though other medications such as hydroxychloroquine[5] have also been used. The pronostic is usually good in the case of an early treatment if there is no visceral involvement.[3][6]

Diagnosis

The key to diagnosis is skin changes combined with blood eosinophilia but the most accurate test is a skin biopsy.

Epidemiology

It is more common in men (especially between 20 and 60) than in women.[7] It is also found in children.

See also

References

  1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
  2. ^ Shulman LE (1975). "Diffuse fasciitis with eosinophilia: a new syndrome?". Trans. Assoc. Am. Physicians 88: 70–86. PMID 1224441. 
  3. ^ a b Bischoff, Lindsay; Chris T. Derk (January 2008). "Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature". International journal of dermatology 47 (1): 29–35. doi:10.1111/j.1365-4632.2007.03544.x. PMID 18173597. 
  4. ^ Antic M, Lautenschlager S, Itin PH (2006). "Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature". Dermatology (Basel) 213 (2): 93–101. doi:10.1159/000093847. PMID 16902285. 
  5. ^ Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB (1988). "Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases". Semin. Arthritis Rheum. 17 (4): 221–31. doi:10.1016/0049-0172(88)90008-X. PMID 3232080. 
  6. ^ Valadares, Diana; Joao Neves, Isabel Almeida, Carlos Lopes, Carlos Vasconcelos (February 2011). "Iron Lady: A Case of Eosinophilic Fasciitis". Journal of Medical Cases 2: 34–36. doi:10.4021/jmc118w. http://www.journalmc.org/index.php/JMC/article/view/118/99. 
  7. ^ Wojas-Pelc A, Wielowieyska-Szybińska D, Lipko-Godlewska S (2004). "[Eosinophilic fasciitis--current database]" (in Polish). Pol. Merkur. Lekarski 16 (96): 585–8. PMID 15510903. 

External links



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