Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens

Abstract

Linear IgA bullous dermatosis (LABD) comprises a heterogeneous group of subepidermal blistering disorders characterized by in situ bound IgA antibodies in epidermal basement membrane. We report three children presenting clinical and immunopathological features characteristic of LABD. By immunoblotting, the three patients' sera contained IgA antibodies that reacted against the bullous pemphigoid (BP) antigen 180 and or BP230, molecular markers for BP. In addition, IgG antibodies directed against the ectodomain of BP180 were detected by an enzyme-linked immunosorbent assay using a eukaryotic recombinant form of BP180. Consistent with recent studies suggesting that the LABD antigen 1, the predominant autoantigen of LABD, is either a proteolytic product of BP180 or an isoform of the BP180 gene, our findings indicate that a subset of children with features of LABD have a distinct form of BP associated with an IgA response.

Childhood oral pemphigoid: a case report and review of the literature

“…Other nonhereditary mucocutaneous blistering diseases of child- Sex (10), blisters in BP do not form scars and the oral cavity is rarely the initial site of presentation (21,(23)(24)(25)(26)(27)(28)(29).…”

Bullous Pemphigoid of Childhood

“…In our 2 patients, blisters were indeed found mainly on the hands, feet, and face; however, mucous membranes were not affected. Moreover, of the 20 reported cases of childhood BP in which the target antigens were specified, 15 (75%) showed no mucosal involvement (reviewed by Trü eb, 22 Edwards, 31 and Arechalde 32 and their colleagues). Therefore, a larger number of well-characterized patients must be analyzed to resolve the issue of whether mucosal involvement is really a characteristic feature of childhood BP.…”

Childhood Bullous Pemphigoid

“…7 In children, BP is clinically similar to its adult counterpart except that mucous membrane involvement is more common in children and involvement of the hands and feet is much more common in infants younger than 1 year. Oral corticosteroids are the treatment of choice at a dosage of 1 to 2 mg/kg per day, 2 but dapsone, 8 sulfapyridine, 9 cloxacillin sodium, 2 erythromycin ethylsuccinate, 10 cyclosporine, 11 and immunoglobulins 11 have been suggested as alternative options. Little is known about the prognosis of the disease in children, but most reported cases had a disease duration of 1 year or less, 2 as in our cases.…”

“…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.…”