Granular C3 Dermatosis.
Acta Derm Venereol
; 96(6): 748-53, 2016 Aug 23.
Article
in En
| MEDLINE
| ID: mdl-26912390
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Basement Membrane
/
Complement C3
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Skin Diseases, Vesiculobullous
/
Dermatitis Herpetiformis
Limits:
Adolescent
/
Adult
/
Aged
/
Aged80
/
Child
/
Female
/
Humans
/
Male
/
Middle aged
Country/Region as subject:
Asia
Language:
En
Journal:
Acta Derm Venereol
Year:
2016
Type:
Article
Affiliation country:
Japan