linear IgA bullous disease

linear IgA bullous disease

a condition characterized by linear deposits of immunoglobulin A binding to the area of the lamina lucida. Tense bullae are frequent, and the vesicles are likely to occur on the face, thighs, feet, and flexures. The disease tends to affect women more than men, and half of the patients are under the age of 60. A chronic bullous dermatosis disease of childhood begins in the first 10 years of life with bullae on the trunk, perioral, and pelvic areas but undergoes total remission at adolescence.
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Linear IgA bullous disease

linear IgA dermatosis

An autoimmune, subepidermal vesiculobullous disease that may be idiopathic, drug-induced (especially by vancomycin) or triggered by a preceding infection or cancer. It is similar to bullous pemphigoid dermatitis herpetiformis, associated with HLA-B8 and gluten sensitivity and linear deposition of IgA along the basement membrane, and often has circulating IgA antibodies against part of the 180-kD bullous pemphigoid antigen BPAg2. Remission occurs in 64% of children within 2 years of onset (average age of child onset, 4 years) and 48% of adults within 5–6 years of onset (average age of adult onset, 52).

Clinical findings
Prolonged prodromal itching, transient pruritus or burning before lesions appear; ocular manifestations are characterised by pain, grittiness or discharge. Bullae may be chronic or appear acutely, as seen in drug-induced disease. Lesions in children are typically located over the lower abdomen and anogenital region; in adults, the lesions overlie the trunk and limbs.

Diagnosis
Linear IgA deposition along the basement membrane by direct immunofluorescence.

Management
Leave bullae intact; cover ruptured lesions and erosions with sterile dressings.
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