Document Detail


Ichthyosis bullosa of Siemens: response to topical tazarotene.
MedLine Citation:
PMID:  16481710     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
S Rajiv; S V Rakhesh
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Indian journal of dermatology, venereology and leprology     Volume:  72     ISSN:  0378-6323     ISO Abbreviation:  Indian J Dermatol Venereol Leprol     Publication Date:    2006 Jan-Feb
Date Detail:
Created Date:  2006-02-16     Completed Date:  2006-06-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7701852     Medline TA:  Indian J Dermatol Venereol Leprol     Country:  India    
Other Details:
Languages:  eng     Pagination:  43-6     Citation Subset:  IM    
Affiliation:
Department of Dermatology and STD, Academy of Medical Sciences, Pariyaram, Kannur, Kerala, India.
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MeSH Terms
Descriptor/Qualifier:
Administration, Topical
Adolescent
Adult
Female
Gels
Humans
Hyperkeratosis, Epidermolytic / drug therapy*
Keratolytic Agents / administration & dosage,  therapeutic use*
Male
Nicotinic Acids / administration & dosage,  therapeutic use*
Skin Diseases, Vesiculobullous / drug therapy*
Treatment Outcome
Chemical
Reg. No./Substance:
0/Gels; 0/Keratolytic Agents; 0/Nicotinic Acids; 118292-40-3/tazarotene

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