Linear IgA bullous dermatosis: report of an exuberant case

Abstract

Linear immunoglobulin A dermatosis is a rare autoimmune bullous disease, but the most common autoimmune bullous dermatosis in children. We report a typical exuberant case of linear IgA dermatosis in a ten-month old child, who showed good response to treatment with corticosteroids and dapsone.

Blistering rash in a young male child

“…[1] One such case report described a 10-month-old white child with LABD with a similar presentation to …”

“…[1] The aetiological factor triggering the autoimmune mechanism of the disease is unknown. [1] The typical clinical presentation of LABD is of small vesicles or bullae, which are annular and may develop into a target shape. There is a tendency for new vesicles to form in a ring around the old lesion, giving the 'string of beads/pearls' sign, which was present in our patient.…”

“…The most frequently associated drugs include vancomycin, diclofenac and co-trimoxazole, but the most consistent association described in the literature is with vancomycin. [1,3,5] The diagnosis is made on examination of a skin biopsy specimen, where an infiltration of neutrophils and eosinophils is seen under the epidermis. Direct immunofluorescence examination reveals IgA along the basement membrane in a linear pattern.…”

“…Only 80 years later was it recognised as a distinct clinical entity. [1] IgA autoantibodies react to components of the hemidesmosomes and basement membrane. [2] The blisters are classically tense, indicating that they are of subepidermal origin as opposed to the flaccid bullous lesions of epidermal origin.…”

Lesões Inguinais Vesicobolhosas Recorrentes numa Criança

“…The first-line treatment is dapsone or sulfapyridine. 1,2 In selected patients, oral prednisone or another corticosteroid-sparing agent such as colchicine may be used to achieve complete control of the disease. …”