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2016 ; 10
(1
): 301
Nephropedia Template TP
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English Wikipedia
Recurrent purpura due to alcohol-related Schamberg s disease and its association
with serum immunoglobulins: a longitudinal observation of a heavy drinker
#MMPMID27799068
Bonnet U
; Selle C
; Isbruch K
; Isbruch K
J Med Case Rep
2016[Oct]; 10
(1
): 301
PMID27799068
show ga
BACKGROUND: It is unusual for purpura to emerge as a result of drinking alcohol.
Such a peculiarity was observed in a 55-year-old man with a 30-year history of
heavy alcohol use. CASE PRESENTATION: The Caucasian patient was studied for 11
years during several detoxification treatments. During the last 2 years of that
period, purpuric rashes were newly observed. The asymptomatic purpura was limited
to both lower limbs, self-limiting with abstinence, and reoccurring swiftly with
alcohol relapse. This sequence was observed six times, suggesting a causative
role of alcohol or its metabolites. A skin biopsy revealed histological features
of purpura pigmentosa progressiva (termed Schamberg's disease). Additionally,
alcoholic fatty liver disease markedly elevated serum immunoglobulins
(immunoglobulin A and immunoglobulin E), activated T-lymphocytes, and increased
C-reactive protein. In addition, moderate combined (cellular and humoral)
immunodeficiency was found. Unlike the patient's immunoglobulin A level, his
serum immunoglobulin E level fell in the first days of abstinence, which
corresponded to the time of purpura decline. Systemic vasculitis and clotting
disorders were excluded. The benign character of the purpura was supported by
missing circulating immune complexes or complement activation. An alcohol
provocation test with vinegar was followed by the development of fresh "cayenne
pepper" spots characteristic of Schamberg's disease. CONCLUSIONS: This case
report demonstrates that Schamberg's disease can be strongly related to alcohol
intake, in our patient most likely as a late complication of severe alcoholism
with alcoholic liver disease. Immunologic disturbances thereby acquired could
have constituted a basis for a hypersensitivity-like reaction after ingestion of
alcohol. Schamberg's disease induction by vinegar may point to an involvement of
acetate, a metabolite of ethanol.