Childhood bullous pemphigoid: Report of a case with characterization of the targeted antigens☆,☆☆,★,★★

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Abstract

The clinical and immunopathologic features of children with acquired subepidermal blistering disorders show considerable overlap, and their classification frequently requires characterization of the targeted antigens. A 8-month-old boy developed a generalized subepidermal blistering disorder with striking palmoplantar involvement. The patient’s serum contained antibodies reacting against the epidermal side of 1 M sodium chloride separated normal human skin. Immunoblotting analysis demonstrated circulating IgG autoantibodies that reacted against a eukaryotic recombinant form of human bullous pemphigoid antigen 180 (BP180). In addition, the patient had circulating IgG autoantibodies that bound a protein of 120 kDa in skin basement membrane zone extracts, that might correspond to the linear IgA bullous disease (LABD) antigen. This study illustrates that a child with clinical and immunopathologic features considered characteristic of childhood bullous pemphigoid (BP) had circulating IgG antibodies that bound to an eukaryotic recombinant form of human BP180, and hence, fulfilled the diagnostic criteria of BP. Review of the literature disclosed only 10 cases of childhood BP, that were characterized on the basis of the targeted antigens. The concomitant presence of circulating IgG autoantibodies against BP180 and a 120 kDa protein may signify either coexistence of autoantibodies with distinct specificities or reflect antigenic cross-reactivity between BP180 and the 120/97 LABD antigen. (J Am Acad Dermatol 1999;40:338-44.)

Section snippets

CASE REPORT

An 8-month-old, previously healthy white child was first seen by his pediatrician for evaluation of multiple tense bullae on the hands and feet (Fig 1).

. Cheiropompholyx-like, tense blisters of hands characteristic of childhood bullous pemphigoid.

Two weeks later, blisters developed on the trunk, and spread to the face and proximal extremities. Physical examination revealed that the child had widespread vesicles, blisters, and crusted erosions that were frequently distributed in annular and

Western immunoblotting (IB)

Equivalent dilutions of patient and control sera were tested against epidermal and skin BMZ extracts by Western immunoblotting (IB). Preparation of keratinocytes extracts with normal human cultured neonatal keratinocytes11, 12 and of dermal extracts has been described in details elsewhere.12, 13 Extracts were subjected to sodium dodecylsulfate-polyacrylamide gel (SDS-PGE), then electrophoretically transferred to nitrocellulose and stained by the peroxydase method.11, 12 Sera were tested at the

RESULTS

By IB that used epidermal extracts, the patient’s serum did not show any IgG or IgA reactivity, whereas control BP serum samples blotted a 180 kDa or a 230 kDa protein (Fig 5, panel A ).

. Panel A, Immunoblot of serum samples obtained from patient and control subjects by using extracts of cultured keratinocytes: lane 1, IgG reactivity of patient serum (dilution 1:20); lane 2, IgA reactivity of patient serum; lane 3, IgG reactivity of normal human serum; lane 4: IgG reactivity of BP control serum

DISCUSSION

The childhood form of BP was first proposed as a clinical and histopathologic entity distinct from pemphigus vulgaris by Lever in 1953.16 In 1970, Bean et al17 described the first case of a child with a putative diagnosis of BP based on IF. In 1991, Nemeth et al2 analyzed the features of 33 children with the diagnosis of BP. The patients described were considered to have childhood BP if they fulfilled the following criteria: (1) 18 years of age or younger, with the clinical appearance of tense

Acknowledgements

We are indebted to Dr Kim B. Yancey, Dermatology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Md, who kindly provided a cDNA clone used to generate full-length cDNA for BP180.

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    This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology.

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    Dr Fellas is a practicing Dermatologist in Nicosia, Cyprus.

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    Reprint requests to: Ralph M. Trüeb, MD, Department of Dermatology, University Hospital of Zurich, Gloriastr. 31, CH-8091 Zurich, Switzerland. E-mail: [email protected]

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    Copyright © 1999 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.