Granular C3 Dermatosis.

Abstract:

:There has been no previous systematic study of bullous skin diseases with granular basement membrane zone deposition exclusively of C3. In this study we collected 20 such patients, none of whom showed cutaneous vasculitis histopathologically. Oral dapsone and topical steroids were effective. Various serological tests detected no autoantibodies or autoantigens. Direct immunofluorescence for various complement components revealed deposition only of C3 and C5-C9, indicating that no known complement pathways were involved. Studies of in situ hybridization and micro-dissection with quantitative RT-PCR revealed a slight reduction in expression of C3 in patient epidermis. These patients may represent a new disease entity, for which we propose the term "granular C3 dermatosis". The mechanism for granular C3 deposition in these patients is unknown, but it is possible that the condition is caused by autoantibodies to skin or aberrant C3 expression in epidermal keratinocytes.

journal_name

Acta Derm Venereol

authors

Hashimoto T,Tsuruta D,Yasukochi A,Imanishi H,Sekine H,Fujita T,Wanibuchi H,Gi M,Kárpáti S,Sitaru C,Zone JJ,Endo D,Abe S,Nishino T,Koji T,Ishii N

doi

10.2340/00015555-2379

subject

Has Abstract

pub_date

2016-08-23 00:00:00

pages

748-53

issue

6

eissn

0001-5555

issn

1651-2057

journal_volume

96

pub_type

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