Medicine:Eosinophilic fasciitis
Eosinophilic fasciitis | |
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Other names | Shulman syndrome[1] |
Eosinophilic fasciitis (/ˌiːəˌsɪnəˈfɪlɪk ˌfæʃiˈaɪtɪs, ˌiːoʊ-, -ˌfæsi-/[2][3]), also known as Shulman's syndrome,[4] 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.[5]
The condition was first characterized in 1974,[6] 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.[citation needed]
Signs and symptoms
Because the disease is rare and clinical presentations vary, a clear set of symptoms is difficult to define. Severe pain and swelling are often reported, and skin can resemble orange peel in appearance.[7] Less common features include joint pain and carpal tunnel syndrome.[citation needed]
Cause
Most cases are idiopathic, but strenuous exercise,[6] initiation of hemodialysis,[8] infection with Borrelia burgdorferi,[9] and certain medications, such as statins, phenytoin, ramipril, and subcutaneous heparin,[10] may trigger the condition.[11]
Diagnosis
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.[citation needed]
Treatment
Common treatments include corticosteroids[12] such as prednisone, although medications such as hydroxychloroquine[13] have also been used. Early initiation of treatment usually portends a good prognosis if there is no visceral involvement.[7][14]
Epidemiology
Typical age of onset is around forty to fifty years, although cases in children have been observed. It remains unclear whether the condition is more common in women or men; patient numbers are small, and studies conflict in their reports of preponderance.[15]
See also
- Eosinophilia
- List of cutaneous conditions
References
- ↑ "Eosinophilic fasciitis: MedlinePlus Medical Encyclopedia" (in en). https://medlineplus.gov/ency/article/000447.htm.
- ↑ "Eosinophilic". Eosinophilic. Oxford University Press. http://www.lexico.com/definition/Eosinophilic. "fasciitis". fasciitis. Oxford University Press. http://www.lexico.com/definition/fasciitis.[|permanent dead link|dead link}}]
- ↑ "Eosinophilic". Merriam-Webster Dictionary. https://www.merriam-webster.com/dictionary/Eosinophilic. Retrieved 2016-01-21. "Fasciitis". Merriam-Webster Dictionary. https://www.merriam-webster.com/dictionary/fasciitis. Retrieved 2016-01-21.
- ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.[page needed]
- ↑ Merck Manual, Professional Edition, Eosinophilic Fasciitis
- ↑ 6.0 6.1 Shulman, L. E (1975). "Diffuse fasciitis with eosinophilia: A new syndrome?". Transactions of the Association of American Physicians 88: 70–86. PMID 1224441.
- ↑ 7.0 7.1 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. https://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1029&context=medfp.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ "Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature". Dermatology 213 (2): 93–101. 2006. doi:10.1159/000093847. PMID 16902285.
- ↑ "Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases". Semin. Arthritis Rheum. 17 (4): 221–31. 1988. doi:10.1016/0049-0172(88)90008-X. PMID 3232080.
- ↑ 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.
- ↑ Wojas-Pelc, Anna; Wielowieyska-Szybińska, Dorota; Lipko-Godlewska, Sylwia (2004). "Eozynofilowe zapalenie powięzi - aktualny stan wiedzy" (in pl). Polski Merkuriusz Lekarski 16 (96): 585–8. PMID 15510903.
Further reading
- Lakhanpal, Sharad; Ginsburg, William W.; Michet, Clement J.; Doyle, John A.; Moore, S.Breanndan (1988). "Eosinophilic fasciitis: Clinical spectrum and therapeutic response in 52 cases". Seminars in Arthritis and Rheumatism 17 (4): 221–231. doi:10.1016/0049-0172(88)90008-X. ISSN 0049-0172. 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): 1157–66. 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.
- Bennett, R M; Herron, A; Keogh, L (1977). "Eosinophilic fasciitis. Case report and review of the literature.". Annals of the Rheumatic Diseases 36 (4): 354–359. doi:10.1136/ard.36.4.354. ISSN 0003-4967. PMID 901033.
- Falanga, Vincent; Medsger, Thomas A. (1987). "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. doi:10.1016/S0190-9622(87)70251-5. ISSN 0190-9622. PMID 3668010.
- "Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders". Current Opinion in Rheumatology 9 (6): 562–70. November 1997. doi:10.1097/00002281-199711000-00013. PMID 9375286.
- Kent, Lawrence T.; Cramer, Stewart F.; Moskowitz, Roland W. (1981). "Eosinophilic fasciitis". Arthritis & Rheumatism 24 (5): 677–683. doi:10.1002/art.1780240508. ISSN 0004-3591. PMID 6972219.
- "Eosinophilic fasciitis: spectrum of MRI findings". AJR. American Journal of Roentgenology 184 (3): 975–8. March 2005. doi:10.2214/ajr.184.3.01840975. PMID 15728627.
External links
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External resources |
Original source: https://en.wikipedia.org/wiki/Eosinophilic fasciitis.
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