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

Abstract

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.

Vulvar Sikatrisyel Pemfigoid: Bir Çocuk Olgu

“…Erişkin büllöz pemfigoid olgularının %9'unda vulvar tutulum saptanırken bu oranın çocukluk çağı büllöz pemfigoidinde %50 gibi oldukça yüksek bir oranda olduğu belirtilmektedir. Literatürde bildirilen yaklaşık yirmi çocukluk çağı sikatrisyel pemfigoid olgusundan sadece üçünde olgumuzda saptandığı gibi izole vulvar tutulum olduğu belirtilmektedir 6,7 . Vulvar sikatrisyel pemfigoidde oküler tutulum, görme kaybı veya körlüğe yol açabilmesi nedeni ile mutlaka araştırılmalıdır.…”

Adolescent Oral Pemphigoid: Report of A Case and Review of Literature

“…There is no absolutely effective agent for MMP and the prognosis among childhood varies. 5,15,16 In addition to our case, literature has reported five childhood oral pemphigoid patients and it was striking to find out that all of them showed desquamative gingivitis as the only clinical manifestation. All six cases including ours have been treated successfully with corticosteroid therapy.…”

Childhood Bullous Pemphigoid

“…Sporadic reports of cases have also been published, but only a few articles report series of patients with characterization of the targeted antigens by immunoblot assay or ELISA. 3,9,15,16 Serum samples were available from 3 of our patients for further immunoblot assay and ELISA testing. The presence of autoantibodies against the NC16A region of BP180 were detected in all of them by immunoblot assay, and in 2 cases this was also confirmed by ELISA.…”