Document Detail

Ichthyosis bullosa of Siemens: further delineation of the phenotype.
MedLine Citation:
PMID:  2138447     Owner:  NLM     Status:  MEDLINE    
We report a third family affected with ichthyosis bullosa of Siemens, and we further delineate the clinical spectrum of this mild type of epidermolytic hyperkeratosis. Erythroderma had never been present in any of the affected individuals. All of them exhibited a brownish, rimpled hyperkeratosis, the main characteristic sites being the joints, the shins and the periumbilical region. Blistering occurred after slight mechanical trauma and even after sweating, resulting in superficially denuded areas. Two affected family members also suffered from chronic, relapsing pustular eruptions surrounded by a transient erythematous flare. Light- and electron-microscopic examination revealed epidermolytic hyperkeratosis limited to the upper part of the epidermis. The pustular lesions were found to be subcorneal blisters filled with neutrophils. Ichthyosis bullosa of Siemens can be clearly distinguished from bullous ichthyosiform erythroderma. The observation of subcorneal pustular dermatosis occurring in this phenotype provides further evidence for the genetic heterogeneity of epidermolytic hyperkeratosis.
P M Steijlen; C M Perret; J H Schuurmans Stekhoven; D J Ruiter; R Happle
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Archives of dermatological research     Volume:  282     ISSN:  0340-3696     ISO Abbreviation:  Arch. Dermatol. Res.     Publication Date:  1990  
Date Detail:
Created Date:  1990-04-26     Completed Date:  1990-04-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8000462     Medline TA:  Arch Dermatol Res     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  1-5     Citation Subset:  IM    
Department of Dermatology, University of Nijmegen, The Netherlands.
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MeSH Terms
Epidermis / pathology,  ultrastructure
Etretinate / therapeutic use
Ichthyosis / drug therapy,  genetics*,  pathology
Microscopy, Electron
Middle Aged
Tretinoin / analogs & derivatives,  therapeutic use
Reg. No./Substance:
302-79-4/Tretinoin; 54350-48-0/Etretinate; 55079-83-9/Acitretin

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