Netherton syndrome


Neth·er·ton syn·drome

(neth'ĕr-tŏn), [MIM*256500] congenital ichthyosiform erythroderma or ichthyosis linearis circumscripta associated with bamboo hair, atopy, urticaria, intermittent aminoaciduria, and mental retardation; probably an autosomal recessive trait that frequently resolves or improves in adolescence.

Netherton syndrome

Atopic diathesis, trichorrhexis invaginata, trichorrhexis nodosa Dermatology A rare skin disorder occurring almost exclusively in ♀, characterized by scaling in a circular pattern–ichthyosis linearis circumflexa Clinical Hair shafts held inside root–trichorrhexis invaginata; hair fragility "bamboo hair", allergies such as asthma, or food allergies that cause skin eruptions Pathogenesis Excess cornification of epidermis. See Bamboo hair.

Neth·er·ton syn·drome

(neth'ĕr-tŏn sin'drōm) Congenital ichthyosiform erythroderma or ichthyosis linearis circumscripta associated with bamboo hair, atopy, urticaria, intermittent aminoaciduria, and mental retardation; probably an autosomal recessive trait; frequently resolves or improves in adolescence.

Comèl,

M., Italian dermatologist and angiologist, 1902-1995. Comèl-Netherton syndrome - Synonym(s): Netherton syndromeRille-Comèl disease - see under Rille

Netherton,

Earl Weldon, U.S. dermatologist, 1893–. Comèl-Netherton syndrome - Synonym(s): Netherton syndromeNetherton syndrome - brittle hair; atopic manifestations. Synonym(s): Comèl-Netherton syndrome; congenital ichthyosiform erythroderma