Journal List > Ann Dermatol > v.9(3) > 1046920

TOOLS
Choi, Song, Lee, Kang, and Lee: A Sporadic Case of Ichthyosis Bullosa of Siemens
Case Report

Annals of Dermatology 1997; 9(3): 211-214.

Published online: 28 November 2016

DOI: https://doi.org/10.5021/ad.1997.9.3.211

A Sporadic Case of Ichthyosis Bullosa of Siemens

Gwang Seong Choi, Ji Sub Song, Eun-So Lee, Won Hyoung Kang, Sungnack Lee

Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology

Abstract

Few cases of ichthyosis bullosa of Siemens(IBS) have been reported since 1939, as a distinct entity from bullous congenital ichthyosiform erythroderma(BCIE). IBS can be differentiated from BCIE by the absence of congenital erythroderma and a different distribution of involved skin area. It's characteristic features include blistering, superficial erosion or moulting of the outer skin.
Histological features are tonofilaments aggregation confined to the granular and upper spinous layer of the epidermis. However, in BCIE these findings are present in the whole suprabasal compartment.
The original reports of Siemens and cases from other authors showed an autosomal dominant inheritance. Our patient developed IBS sporadically without a familial background.

Keywords: Ichthyosis bullosa of Siemens(IBS) Bullous congenital ichthyosiform erythroderma(BCIE) sporadic

TOOLS
Similar articles