Ichthyosis bullosa of Siemens: response to topical tazarotene.

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dc.contributor.authorRajiv, Sen_US
dc.contributor.authorRakhesh, S Ven_US
dc.date.accessioned2009-05-28T08:03:20Z
dc.date.available2009-05-28T08:03:20Z
dc.date.issued2006-01-17en_US
dc.description.abstractIn 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.en_US
dc.description.affiliationDepartment of Dermatology and STD, Academy of Medical Sciences, Pariyaram, Kannur, Kerala, India.en_US
dc.identifier.citationRajiv S, Rakhesh SV. Ichthyosis bullosa of Siemens: response to topical tazarotene. Indian Journal of Dermatology, Venereology and Leprology. 2006 Jan-Feb; 72(1): 43-6en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/52041
dc.language.isoengen_US
dc.source.urihttps://www.ijdvl.comen_US
dc.subject.meshAdministration, Topicalen_US
dc.subject.meshAdolescenten_US
dc.subject.meshAdulten_US
dc.subject.meshFemaleen_US
dc.subject.meshGelsen_US
dc.subject.meshHumansen_US
dc.subject.meshHyperkeratosis, Epidermolytic --drug therapyen_US
dc.subject.meshKeratolytic Agents --administration & dosageen_US
dc.subject.meshMaleen_US
dc.subject.meshNicotinic Acids --administration & dosageen_US
dc.subject.meshSkin Diseases, Vesiculobullous --drug therapyen_US
dc.subject.meshTreatment Outcomeen_US
dc.titleIchthyosis bullosa of Siemens: response to topical tazarotene.en_US
dc.typeCase Reportsen_US
dc.typeJournal Articleen_US
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