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Image Number 1514 (Linear IgA disease)


Diagnosis: Linear IgA disease

Site: Trunk

Sex: M

Age: 10


Description: Tense bullae.

Submitted by: Nameer Al-Sudany

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Differential Diagnosis

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.

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