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A Sporadic Case of Ichthyosis Bullosa of Siemens
Annals of Dermatology ; : 211-214, 1997.
Artigo em Inglês | WPRIM | ID: wpr-70672
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.
Assuntos

Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Pele / Testamentos / Filamentos Intermediários / Vesícula / Dermatite Esfoliativa / Muda / Epiderme / Ictiose Bolhosa de Siemens / Ictiose Limite: Humanos Idioma: Inglês Revista: Annals of Dermatology Ano de publicação: 1997 Tipo de documento: Artigo

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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Pele / Testamentos / Filamentos Intermediários / Vesícula / Dermatite Esfoliativa / Muda / Epiderme / Ictiose Bolhosa de Siemens / Ictiose Limite: Humanos Idioma: Inglês Revista: Annals of Dermatology Ano de publicação: 1997 Tipo de documento: Artigo