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February 2010

Superficial Flaccid Bullae in a Female Neonate—Diagnosis

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Arch Dermatol. 2010;146(2):191-196. doi:10.1001/archdermatol.2009.366-b

The punch biopsy specimen from the buttock revealed hyperkeratosis and an intragranular vesicle. The granular and superficial spinous cells were vacuolized, and there were prominent, irregular, clumped, basophilic granules and eosinophilic intraspinous granules.

A rare superficial blistering disorder, IBS was first described by Siemens in 1937.1,2 It is inherited in an autosomal dominant manner and, to our knowledge, 29 affected families have been described in the literature. Patients develop widespread superficial bullae and vesicles at birth, but only a minority of patients are erythrodermic.24 Blistering is exacerbated by warm weather and friction, and bullae resolve without scarring or atrophy.5,6

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