A Sporadic Case of Ichthyosis Bullosa of Siemens.
10.5021/ad.1997.9.3.211
- Author:
Gwang Seong CHOI
;
Ji Sub SONG
;
Eun So LEE
;
Won Hyoung KANG
;
Sungnack LEE
- Publication Type:Case Report
- Keywords:
Ichthyosis bullosa of Siemens(IBS) Bullous congenital ichthyosiform erythroderma(BCIE) sporadic
- MeSH:
Blister;
Dermatitis, Exfoliative;
Epidermis;
Humans;
Ichthyosis Bullosa of Siemens*;
Ichthyosis*;
Intermediate Filaments;
Molting;
Skin;
Wills
- From:Annals of Dermatology
1997;9(3):211-214
- CountryRepublic of Korea
- Language:English
-
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.
