Suspected case of chronic bullous disease of childhood in a rural area of Southern Mozambique

Description

Chronic bullous disease of childhood (CBDC) is the most common, non-hereditary, autoimmune blistering disorder of childhood. This rare condition, characterised by linear IgA staining on direct immunofluorescence of the basement membrane of the squamous epithelium, has been considered the paediatric variant of adult linear IgA disease,1 although CBDC tends to occur in children less frequently, usually as a response to neoplasms or drug hypersensitivity.

The clinical hallmark of this condition is the abrupt appearance on normal or erythematous skin of many large, tense bullae, filled with clear or sometimes haemorrhagic fluid and acquiring the typical rosette-like pattern of 'string of pearls' (figure 1), with or without associated pruritus. Bullae predominantly appear in the genital and buttocks area, although they can also appear in the trunk, extremities and face (figure 2). Mucous membranes may also be affected. Secondary impetiginisation may be frequent,...

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