Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
Abstract
In 1937, Siemens described a Dutch family with superficial blistering,
flexural hyperkeratosis, and characteristic mauserung appearance. Since
then, less than 20 kindreds with this condition have been described in
the English dermatologic literature. A 14-year-old boy presented with
history of recurrent blistering and peeling of skin since the age of 1
month, predominantly seen over limbs and trunk, often associated with
secondary infection. His mother also had similar symptoms from
childhood. On examination, the child had typical mauserung peeling of
the skin and dirty gray hyperkeratosis in a rippled pattern over
flexures. Skin biopsy from the boy showed intracorneal blistering with
epidermolytic hyperkeratosis in the upper spinous layers. The typical
history and clinical features along with characteristic histological
findings confirmed our diagnosis of ichthyosis bullosa of Siemens. It
must be differentiated from other conditions with epidermolytic
hyperkeratosis and skin peeling, such as bullous ichthyosiform
erythroderma of Brocq and peeling skin syndrome. Our patient responded
well to 0.05% topical tazarotene gel over four weeks