Eosinophilic fasciitis

Eosinophilic fasciitis (/ˌiːəˌsɪnəˈfɪlɪk ˌfæʃiˈaɪtɪs, ˌiːoʊ-, -ˌfæsi-/[2][3]), also known as "Shulman's syndrome",[4] is a form of fasciitis, the inflammatory diseases that affect the fascia, the connective tissues surrounding muscles, blood vessels and nerves. Unlike other diseases in that category, it is limited to the arms and legs, and usually resolves itself, although some cases require corticosteroids, and some cases are associated with aplastic anemia.[5]

Eosinophilic fasciitis
Other namesShulman syndrome[1]
SpecialtyRheumatology 

The presentation of eosinophilic fasciitis is similar to scleroderma or systemic sclerosis. However, unlike scleroderma, it affects the fascia, not the skin (dermis). The characteristic and severe effects of scleroderma and systemic sclerosis, such as Raynaud's syndrome, involvement of the extremities, prominent small blood vessels (telangiectasia), and visceral changes such as swallowing problems, are absent.

It was first characterized in 1974,[6] 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.

Signs and symptoms

As it is a rare disease, a clear set of symptoms is difficult to define. Usually, patients show severe pain and swelling is reported but clinical presentations vary. It can have an 'orange peel' like appearance.[7] Less common features are joint pain and carpal tunnel syndrome.

Cause

Most cases are idiopathic, but several triggers might related to the development of Eosinophilic fasciitis,[8] such as strenuous exercise,[6] initiation of hemodialysis,[9] infection with Borrelia burgdorferi,[10] some medications such as statins, phenytoin, ramipril, and subcutaneous heparin.[11]

Diagnosis

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

Treatment

Common treatments include corticosteroids[12] such as prednisone, though other medications such as hydroxychloroquine[13] have also been used. The prognosis is usually good in the case of an early treatment if there is no visceral involvement.[7][14]

Epidemiology

Typical age of onset is around 40 to 50 years. It is not clear whether it is more common in women than men - patient numbers are small and some studies report a preponderance of men and others women.[15] It is also found in children.

See also

References
  1. "Eosinophilic fasciitis: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 29 October 2019.
  2. "Eosinophilic". Oxford Dictionaries. Oxford University Press. Retrieved 2016-01-21. "fasciitis". Oxford Dictionaries. Oxford University Press. Retrieved 2016-01-21.
  3. "Eosinophilic". Merriam-Webster Dictionary. Retrieved 2016-01-21. "Fasciitis". Merriam-Webster Dictionary. Retrieved 2016-01-21.
  4. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  5. Merck Manual, Professional Edition, Eosinophilic Fasciitis
  6. Shulman, L. E (1975). "Diffuse fasciitis with eosinophilia: A new syndrome?". Transactions of the Association of American Physicians. 88: 70–86. PMID 1224441.
  7. 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.
  8. Pinal-Fernandez, I; Selva-O'Callaghan, A; Grau, J.M (2014). "Diagnosis and classification of eosinophilic fasciitis". Autoimmunity Reviews. 13 (4–5): 379–82. doi:10.1016/j.autrev.2014.01.019. PMID 24424187.
  9. Florell, Scott R; Egan, Conleth A; Gregory, Martin C; Zone, John J; Petersen, Marta J (2016). "Eosinophilic Fasciitis Occurring Four Weeks after the Onset of Dialysis in a Renal Failure Patient". Journal of Cutaneous Medicine and Surgery. 5 (1): 33–6. doi:10.1177/120347540100500108. PMID 11281432.
  10. Mosconi, Sandro; Streit, Markus; Brönimann, Marcel; Braathen, Lasse R (2002). "Eosinophilic Fasciitis (Shulman Syndrome)". Dermatology. 205 (2): 204–6. doi:10.1159/000063916. PMID 12218249.
  11. Long, Hai; Zhang, Guiying; Wang, Ling; Lu, Qianjin (2015). "Eosinophilic Skin Diseases: A Comprehensive Review". Clinical Reviews in Allergy & Immunology. 50 (2): 189–213. doi:10.1007/s12016-015-8485-8. PMID 25876839.
  12. 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. 213 (2): 93–101. doi:10.1159/000093847. PMID 16902285.
  13. 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.
  14. Valadares, Diana; Joao Neves; Isabel Almeida; Carlos Lopes; Carlos Vasconcelos (February 2011). "Iron Lady: A Case of Eosinophilic Fasciitis". Journal of Medical Case Reports. 2: 34–36. doi:10.4021/jmc118w.
  15. Wojas-Pelc, Anna; Wielowieyska-Szybińska, Dorota; Lipko-Godlewska, Sylwia (2004). "Eozynofilowe zapalenie powięzi - aktualny stan wiedzy" [Eosinophilic fasciitis--current database]. Polski Merkuriusz Lekarski (in Polish). 16 (96): 585–8. PMID 15510903.

Further reading
Classification
External resources
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