Hoffman M D, Fleming M G, Pearson R W
Department of Dermatology, Rush-Presbyterian-St Luke's Medical Center, Chicago, Ill, USA.
Arch Dermatol. 1995 May;131(5):586-9.
We describe a new variant of inherited epidermolysis bullosa and elucidate the clinical, histologic, and ultrastructural features of this condition.
This form of epidermolysis bullosa displays an autosomal dominant inheritance pattern, is characterized by acral bullae, and histologically demonstrates suprabasal clefting with acantholysis. Ultrastructural findings are nonspecific but reminiscent of those observed in benign familial pemphigus.
Acantholytic epidermolysis bullosa is a rare but distinct clinicopathologic entity that warrants inclusion in the nosologic classification of epidermolysis bullosa.
我们描述了一种遗传性大疱性表皮松解症的新变体,并阐明了该病症的临床、组织学和超微结构特征。
这种形式的大疱性表皮松解症表现为常染色体显性遗传模式,以肢端水疱为特征,组织学显示基底上裂隙伴棘层松解。超微结构发现无特异性,但让人联想到在良性家族性天疱疮中观察到的情况。
棘层松解性大疱性表皮松解症是一种罕见但独特的临床病理实体,值得纳入大疱性表皮松解症的疾病分类中。