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- Title
Non-thrombocytopenic purpura in familial Mediterranean fever--comorbidity with Henoch-Schonlein purpura or an additional rare manifestation of familial Mediterranean fever?
- Authors
Ben-Chetrit, Eldad; Yazici, Hasan
- Abstract
Henoch-Schönlein purpura is a relatively common vasculitis mainly affecting children. It is characterized by purpuric skin rash, abdominal cramping, and haematuria. Skin biopsies taken from Henoch-Schönlein purpura lesions disclose perivascular IgA deposits. FMF is an autoinflammatory disease characterized by recurrent attacks of fever lasting 2-3 days which resolve spontaneously. Typical manifestations of the disease are peritonitis, pleuritis, pericarditis, arthritis and erysipelas-like erythema usually affecting the lower limbs. Over the years many reviews emphasized the clinical impression that Henoch-Schönlein purpura is more common among FMF patients than in healthy control population. In this review we summarize these reports and show that sometimes Henoch-Schönlein purpura associated with FMF differs from typical isolated Henoch-Schönlein purpura, and this is also the case with polyarteritis nodosa and SpA associated with FMF. It is suggested that these clinical manifestations (polyarteritis nodosa, Henoch-Schönlein purpura and SpA) should be considered to be associated with FMF as part of what we call FMF rather than as co-existing additional separate clinical entities.
- Subjects
CUTANEOUS manifestations of general diseases; GENETIC disorders; INFLAMMATION; POLYARTERITIS nodosa; TERMS &; phrases; VASCULITIS; COMORBIDITY; SCHOENLEIN-Henoch purpura
- Publication
Rheumatology, 2016, Vol 55, Issue 7, p1153
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/kev378