Cutaneous vasculitis and glomerulonephritis associated with C4 deficiency.

Abstract:

:Complete deficiency of the fourth component of complement (C4) is an extremely rare condition. However, it has been reported that partial C4 deficiency can occur in normal subjects, and is associated with several immune diseases. We report a 44-year-old woman who developed slight oedema and punctate purpura on her lower legs after a common cold. She was noted to have persistent microscopic haematuria and proteinuria, and her C4 level was undetectable. On histological examination of a skin biopsy specimen, leucocytoclastic vasculitis was seen, with granular deposition of IgG, IgM, C3 and C1q on the vessel walls in the upper dermis. A renal biopsy showed mild mesangial proliferative glomerulonephritis with slight damage to the capillary loops, and granular deposits of IgM and C4 mainly in the mesangium. The patient was systemically well and needed no medication. The C4 level remained low during the observation period, but neither genotyping nor allotyping analysis identified a C4 deficiency.

journal_name

Clin Exp Dermatol

authors

Kosaka S,Osada S,Kaneko T,Nishimura S,Kawana S

doi

10.1111/j.1365-2230.2012.04423.x

subject

Has Abstract

pub_date

2013-07-01 00:00:00

pages

492-5

issue

5

eissn

0307-6938

issn

1365-2230

journal_volume

38

pub_type

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