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[Question]

A 13-month-old boy presented with multiple pruritic tense blisters on the lower abdomen, lower extremities, groin, perineum and perioral areas for the past 1 week. There was no family history of skin disease, and no history of recent upper respiratory tract infections or drug intake prior to the onset of blistering. Physical examination revealed annular erythema with circumferential vesicles and blisters giving a €˜cluster of jewels €™ appearance. There was no mucosal involvement. What is the diagnosis?

 

 

 

[Answer]

Dermatophile Paediatric bullous dermatoses

 

Linear immunoglobulin A (IgA) dermatosis is an autoimmune subepidermal vesiculobullous disease of children (chronic bullous dermatosis of childhood) and adults. It is the most common acquired blistering disease in the paediatric population and can appear as early as 6 months of age.1 Vesicles and bullae are usually arranged in an annular manner described as the €˜cluster of jewels €™ appearance. Lesions in children occur mostly on the lower abdomen and anogenital areas with frequent oral and ocular involvement. Blood tests show antibodies directed against a 97-kd antigen in the lamina lucida of the dermoepidermal junction, and direct immunofluorescence shows linear deposition of IgA at the basement membrane zone. Remission occurs in 64% of children within 2 €“4 years. Most cases respond to dapsone or sulfapyridine. Additional therapy with systemic corticosteroids may be needed. Erythromycin may be a helpful adjunct with other systemic agents.

Acknowledgments

Images from the Color Atlas & Synopsis of Pediatric Dermatology, Second Edition, McGraw-Hill, 2009, used with permission.

References

  1. Mintz EM, Morel KD. Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin 2011;29:459 €“62. [PubMed ® abstract]

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