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Image Number #3518 (Linear IgA disease)

Diagnosis: Linear IgA disease

Description: Tense bullae.

Morphology: Blisters

Site: Trunk

Sex: M

Age: 10

Type: Clinical

Submitted By: Nameer Al-Sudany

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Differential Diagnosis
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History: Chronic Benign Bullous Dermatosis of Childhood (CBBDC) usually presents with tense bullae, often grouped, that arise on urticarial plaques on the trunk and extensor surfaces of the extremities. The so-called ‘string of pearls sign’ or 'cluster of jewels', seen in some cases, is the presence of blistering around the rim of polycyclic urticarial plaques. The disease associated with linear deposits of IgA and C3 at the basement membrane zone. The disorder responds well to oral dapsone.

DermNetNZ   eMedicine   PubMed   Dermatology Online   Archives   JAAD for "Linear IgA disease"

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