An Exceedingly Rare Case of Mechanobullous Epidermolysis Bullosa Acquisita in a Prepubertal Child: A Review of the Clinical and Laboratory Considerations

TL;DR

This paper reports a rare case of a blistering skin disease in a child and emphasizes the need for better guidelines for treating it in children.

Contribution

The paper presents an exceedingly rare case of mechanobullous EBA in a prepubertal child and suggests conservative management as a potential treatment approach.

Findings

An 11-year-old child was diagnosed with mechanobullous EBA using immunofluorescence and ELISA tests.

Treatment with a topical steroid and activity modifications improved the patient's condition.

The case underscores the importance of considering EBA in pediatric blistering disease differential diagnoses.

Abstract

Introduction: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune disease causing subepithelial blistering due to autoantibodies against type VII collagen. While mechanobullous EBA predominantly affects adults, our report presents an exceedingly rare case in an 11-year-old football player. Case Report: The patient reported a one-year history of blistering and scarring on the knees and scrotum. The diagnosis was established with direct immunofluorescence (DIF), mosaic indirect immunofluorescence (IIF) showing IgG antibodies reacting with the dermal side of salt-split primate skin, and multiplex ELISA revealing an elevated level of IgG antibodies against type VII collagen. Treatment with a superpotent topical glucocorticosteroid and activity modifications improved his condition. Review: This case highlights the importance of considering EBA in differential diagnoses of pediatric blistering diseases and suggests that conservative management may be effective in mild cases. We also review clinical and laboratory considerations on the topic of childhood EBA. Conclusions: Further studies are essential to develop evidence-based guidelines for pediatric EBA.

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