icthyosis bullosa of Siemens

icthyosis bullosa of Siemens

An autosomal dominant disorder with blistering and over-thinkening of the horny surface layer of the epidermis (hyperkeratosis). The condition features an unusually heavy shedding (moulting) of the surface layers of the skin and a grey, lichenoid appearance.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005