Netherton syndrome


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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 syndrome
Rille-Comèl disease - see under Rille

Netherton,

Earl Weldon, U.S. dermatologist, 1893–.
Comèl-Netherton syndrome - Synonym(s): Netherton syndrome
Netherton syndrome - brittle hair; atopic manifestations. Synonym(s): Comèl-Netherton syndrome; congenital ichthyosiform erythroderma
References in periodicals archive ?
Corneodesmosomal cadherins are preferential targets of stratum corneum trypsin- and chymotrypsin-like hyperactivity in Netherton syndrome.
Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome.
Serine protease activity and residual LEKTI expression determine phenotype in Netherton syndrome.
We treated 99% of the body surface area with pimecrolimus in some of our patients with Netherton syndrome, yet their blood levels after 3 weeks of therapy were still only one-tenth of what's needed for a systemic effect," the dermatologist said.
30pm) to raise money for Jack Oldacres, who suers from a rare and painful skin condition, Netherton Syndrome.
Their son Jack suffers from Netherton Syndrome and the money has been given to help research into the rare, painful skin condition - with the ultimate aim of finding a cure.
The staff there are great and invited me to give a talk at an assembly to explain all about Netherton Syndrome.