title: 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.
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 830-0011 Kurume, Japan. hashimot@med.kurume-u.ac.jp.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Daisuke
authors.initials: D
authors.lastname: Tsuruta
authors.forename: Atsushi
authors.initials: A
authors.lastname: Yasukochi
authors.forename: Hisayoshi
authors.initials: H
authors.lastname: Imanishi
authors.forename: Hideharu
authors.initials: H
authors.lastname: Sekine
authors.forename: Teizo
authors.initials: T
authors.lastname: Fujita
authors.forename: Hideki
authors.initials: H
authors.lastname: Wanibuchi
authors.forename: Min
authors.initials: M
authors.lastname: Gi
authors.forename: Sarolta
authors.initials: S
authors.lastname: Kárpáti
authors.forename: Cassian
authors.initials: C
authors.lastname: Sitaru
authors.forename: John J
authors.initials: JJ
authors.lastname: Zone
authors.forename: Daisuke
authors.initials: D
authors.lastname: Endo
authors.forename: Shinichi
authors.initials: S
authors.lastname: Abe
authors.forename: Tomoya
authors.initials: T
authors.lastname: Nishino
authors.forename: Takehiko
authors.initials: T
authors.lastname: Koji
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000890:Anti-Infective Agents / Q000627:therapeutic use; D001485:Basement Membrane / Q000378:metabolism*; D002648:Child; D003176:Complement C3 / Q000378:metabolism*; D003622:Dapsone / Q000627:therapeutic use; D003874:Dermatitis Herpetiformis / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D015151:Immunoblotting; D017403:In Situ Hybridization; D007564:Japan; D015603:Keratinocytes / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D060888:Real-Time Polymerase Chain Reaction; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology; D013256:Steroids / Q000627:therapeutic use
chemical_list: D000890:Anti-Infective Agents; D003176:Complement C3; D013256:Steroids; D003622:Dapsone