Post-streptococcal vasculopathy with evolution to Degos' disease
Institution:
Barrow Neurological Institute, 350 W Thomas Road, Phoenix, AZ 85013, United States
Correspondance Name:
Sandipan Pati, Corresponding author. Dept. of Neurology, 350 W Thomas Road, Phoenix, AZ 85013, United States. Tel.: + 1 480 652 3615.
Correspondance Email:
sandipan.pati@chw.edu
Publication date:
Tuesday, 5th October 2010
Link:
http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T06-51BPDV6-2&_user=10&_coverDate=10/29/2010&_rdoc=1&_fmt=high&_orig=search&_origin=search&_sort=d&_docanchor=&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=930a35712dc1c43ff90d50b1a2329e83&searchtype=a Degos’ disease or malignant atrophic papulosis is a rare disseminated occlusive vasculopathy affecting the skin, gastrointestinal tract, central nervous system, and less often other organ systems. The exact etiology of this vasculopathy has not been established. Infections, autoimmune disease and coagulation defects have been proposed as underlying pathogenic mechanisms, but none have been confirmed. Here, we report the clinical, radiological and histopathologic features of Degos’ disease in a 41-year-old man following streptococcal throat infection. Prior postulated hypothesis as post-infectious immunologic mechanism may be further supported by this case.
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1 comment
This is an interesting observation
This is an interesting observation indicating an immune reaction like the one we already know for leukocytoclastic vasculitis, erythema nodosum, acute febrile neutrophilic dermatosis (Sweet syndrome), pityriasis lichenoides chronica et variolipormis acuta (PLEVA), reactive arthritis etc. The major question arising is why the majority of the patients develop eukocytoclastic vasculitis and/or reactive arthritis and other rare syndromes, such as malignant atrophic papulosis. A genetic predisposition? Different streptococci genres? Other factors?
With best regards
Prof. Dr. Christos C. Zouboulis