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A child with localized vulval pemphigoid and IgG autoantibodies targeting the C‐terminus of collagen XVII/BP180
Author(s) -
Schumann,
Amann,
Kaisa Tasanen,
Martin Anton Müller,
Zillikens,
Metze,
Luger,
Bruckner-Tuderman,
Bonsmann
Publication year - 1999
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1999.02891.x
Subject(s) - bullous pemphigoid , autoantibody , pemphigoid , cicatricial pemphigoid , medicine , pathology , epitope , ectodomain , antibody , immunology , receptor
Localized vulval pemphigoid of childhood (LVPC) has previously been reported in six girls. Clinical features and immunopathological data have suggested it to be a morphological variant of bullous pemphigoid. Epitope targets of the autoantibodies of these patients have not been defined in detail. We describe a 9‐year‐old girl with possible cicatricial LVPC and circulating IgG antibodies directed against native collagen XVII/BP180, its 120‐kDa soluble ectodomain and against the C‐terminus of collagen XVII/BP180. No reactivity was detected towards the NC16A domain of collagen XVII/BP180. Linear IgG and C3 deposits were found along the cutaneous basement membrane zone. On 1 mol/L salt‐split skin, IgG autoantibodies were shown to bind to the epidermis, and the HLA type II allele DQB1*0301, a marker with significantly increased occurrence in patients with ocular and oral cicatricial pemphigoid, was identified in this patient. Our data suggest that LVPC is a variant of bullous pemphigoid in which direct immunofluorescence microscopy combined with immunoblot analysis can deliver valuable diagnostic information for differential diagnosis. However, differentiation between the scarring and non‐scarring course of the disease cannot be made with the present diagnostic markers and therefore careful follow‐up of patients with LVPC is required.