Trichorrhexis invaginata

Ichthyosis linearis cirumflexa Cornel) will not be reported here. Frenk and Mevorah as well as Thorne and co-workers have shown that unknown substances are formed in the border of growing lesions where parakeratosis is present and keratohyalin granules are missing We found this process not only to be limited locally but to be restricted to short periods of abnormal differentiation (unpublished observations). In Netherton s syndrome, this type of ichthyosis is combined with trichorrhexis invaginata, the ultrastructure of which was described by Orfanos et aiy. ... 

Frenk, E. and Mevorah, B. (1972). Ichthyosis linearis circumflexa Cornel with trichorrhexis invaginata (Netherton s syndrome). An ultrastructural study of skin changes. Arch. Dermatol Forsch., 245,42... 

Orfanos, C. E., Mahrle, G. and Salomon, T. (T971). Netherton Syndrom. Ichthyosiforme Hautveranderungen und Trichorrhexis invaginata. Nachweis eines krankhaft veranderten Cortexkeratins im Haar. Hautarzt, 22,397... 

Netherton s disease is used to refer to an association of scaling disorders of the skin and hair abnormalities. Although one cannot be sure of the nature of the scaling disorder of Netherton s patients, lamellar Ichthyosis is a likely possibility. The hair defect was most likely trichorrhexis invaginata. An association between other hair disorders and other scaling conditions of the skin has also been reported Ichthyosis linearis circumflexa, along with pila torti, trichorrhexis nodosa, or trichorrhexis invaginata has been described in several patients. 

Netherton s syndrome is an inherited, autosomal recessive condition, but almost only females are affected . It starts as a transient erythroderma and subsequently develops migratory and fixed cutaneous lesions (similar to those of Erythrokeratodermia variabilis), a characteristic trichorrhexis invaginata on the scalp, eyebrows and eyelashes, and atopy. Palms, soles and nails are normal. The ingestion of nuts causes the exudation of skin lesions. Histologically, hyperkeratosis with parakeratosis, discontinuous stratum granu-losum, acanthosis and prominent rete ridges are present. Mononuclear exocytosis and infiammatory infiltrate in the upper dermis are also observed. 

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