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Childhood vulval pemphigoid: a clinical and immunopathological study of five patients
Author(s) -
' Farrell,
Kirtschig,
Dalziel,
Matthew R. Allen,
Dootson,
; Edwards,
Wojnarowska
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.1365-2133.1999.02668.x
Subject(s) - cicatricial pemphigoid , hemidesmosome , immunoelectron microscopy , medicine , lamina densa , dapsone , pemphigoid , lamina lucida , pathology , bullous pemphigoid , dermatology , vulva , azathioprine , basement membrane , antibody , immunohistochemistry , immunology , basal lamina , disease , ultrastructure
We describe five girls with vulval pemphigoid: two had bullous pemphigoid confined to the vulva and three had cicatricial pemphigoid. They demonstrate a spectrum of severity from localized disease to extensive vulval scarring necessitating long‐term immunosuppressive therapy and surgical correction. The age at onset of their disease ranged between 6 and 13 years. All presented with vulval discomfort and erosions. Three had oral lesions, two perianal and one eye and cutaneous involvement. Two girls with only vulval lesions and one with vulval and oral lesions responded well to topical steroids. In two, systemic treatment with prednisolone and dapsone or azathioprine was required. The diagnosis was made on the basis of histology and immunofluorescence (IF). All had positive direct IF with IgG and C3. Indirect IF demonstrated circulating IgG binding to the basement membrane zone in four, with dermal or epidermal binding on salt‐split skin substrate. Immunoblotting revealed antibodies to the BP230 and BP180 antigens. Immunoelectron microscopy in the child with dermal binding IgG and BP180 and BP230 on immunoblotting showed labelling at the lamina densa–lamina lucida interface adjacent to hemidesmosomes.