Chapter 21 - Neuroichthyosis

Abstract

This chapter describes neuroichthyosis, which refers to those conditions associating ichthyosis or ichthyosiform dermatoses to central or peripheral nervous system involvement. Ichthyosis is a group of genetically determined alterations of keratinization clinically characterized by persistent, clinically evident scales on the skin surface. Ichthyosiform dermatosis is a heterogeneous group of skin disorders characterized by different degrees of hyperkeratosis, scaling, and erythema. Lesions with capricious designs are often found in ichthyosiform dermatoses. Several well-recognized diseases and a few infrequently reported syndromes strongly support the concept that the association among ichthyosis, ichthyosiform dermatoses, and nervous system pathology is not coincidental. Ruds syndrome is inherited as an autosomal recessive trait. Tay syndrome is unusual ichthyotic syndrome of autosomal recessive inheritance is characterized by pili torti and a tricorrhexis nodosa-like alteration whose substrate is trichothiodystrophy. A syndrome of migratory ichthyosiform dermatosis with neurologic and ocular abnormalities, present since birth, has recently been described. The association of ichthyosis, thrombocytopenia, myasthenia, miosis, asplenia, migraine, and dyslexia has been recently reported.