Childhood vulval pemphigoid: a clinical and immunopathological study of five patients

We describe five girls with vulval pemphigoid: two had bullous pemphigoid c onfined 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 prese nted with vulval discomfort and erosions. Three had oral lesions, two peria nal and one eye and cutaneous involvement. Two girls with only vulval lesio ns and one with vulval and oral lesions responded well to topical steroids. In two, systemic treatment with prednislone and dapsone or azathioprine wa s required. The diagnosis was made on the basis of histology and immunofluo rescence (IF). All had positive direct IF with IgG and C3. Indirect IF demo nstrated circulating IgG binding to the basement membrane zone in four, wit h dermal or epidermal binding on salt-split skin substrate. Immunoblotting revealed antibodies to the BP230 and BP180 antigens. Immunoelectron microsc opy in the child with dermal binding IgG and BP180 and BP230 on immunoblott ing showed labelling at the lamina densa-lamina lucida interface adjacent t o hemidesmosomes.