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 CASE REPORT
Year : 2006  |  Volume : 72  |  Issue : 1  |  Page : 43--46

Ichthyosis bullosa of Siemens: Response to topical tazarotene


Department of Dermatology and STD, Academy of Medical Sciences, Pariyaram, Kannur, Kerala, India

Correspondence Address:
S V Rakhesh
Department of Dermatology and STD, Academy of Medical Sciences, Pariyaram, Kannur 670503, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.19718

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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.






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Online since 15th March '04
Published by Wolters Kluwer - Medknow