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Clinical and Immunological Findings in a Series of 4 Cases

Maria Isabel Martinez-De Pablo, MD; Maria Antonia González-Enseñat, MD; Asunción Vicente, MD; Montserrat Gilaberte, MD; José Manuel Mascaró Jr, MD

Arch Dermatol. 2007;143(2):215-220.

Background  Bullous pemphigoid (BP) is an autoimmune blistering disease that is rare in childhood. As in adult BP, antibodies against the 180-kDa antigen (BP180) seem to be involved in the pathogenesis of the disease, but, to date, only a small number of children with the disease have been examined immunologically.

Observations  We report the cases of 4 infants with BP aged 5 to 12 months. All of them had involvement of the hands and feet, and they all achieved a complete remission in less than 6 months when treated with oral prednisolone stearoyl glycolate. Three patients could be examined using antigen characterization techniques. Autoantibodies against the NC16A domain of BP180 were found by immunoblot assay in all 3 and by enzyme-linked immunosorbent assay in 2 of them. Interestingly, although IgA autoantibodies were detected in only 1 of them by indirect immunofluorescence, all of them had IgA autoantibodies, and 2 of the 3 had IgG autoantibodies against NC16A as detected by immunoblot assay. One patient also had IgG autoantibodies against the carboxyterminal domain of BP180.

Conclusions  IgA-specific antibodies against BP180 were detected in all our patients. These findings further raise the question about the relationship between BP and linear IgA bullous dermatosis, the most common autoimmune blistering disorder in children.

Author Affiliations: Department of Dermatology, Hospital Clinic, and University School of Medicine (Drs Martinez-De Pablo and Mascaró), Departments of Dermatology, Hospital de Sant Joan de Deu (Drs González-Enseñat and Vicente) and Hospital del Mar (Dr Gilaberte), Barcelona, Spain.

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