Ichthyosis linearis circumflexa

Unlabeled Uses Dermographism, eczema, hypereosinophilic syndrome, hypopig-mented sarcoidosis, ichthyosis linearis circumflexa, lymphomatoid papulosis, mycosis fungoides, palmoplantar pustulosis, pruritus, scleromyxedema, systemic sclero-... 

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... 

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. 

Since the relationship between ichthyosis linearis circumflexa and lamellar or other forms of ichthyosis is not known, it is probably best to reserve the eponym, Nethertorfs disease, only for those patients with lamellar ichthyosis or to avoid using the eponym at all. 

Erythrodermia variabilis of Mendes da Costa, Netherton s disease, ichthyosis linearis circumflexa of Cornel and symmetrical progressive erythrokerato-... 

Ichthyosis linearis circumflexa of Cornel is an autosomal recessive disorder which, from birth or infancy, shows extensive migratory serpiginous and polycyclic lesions of erythema and scaling, with a distinctive double-edged scale at the borders. The course is chronic and the same histopathology is seen as in erythrokeratodermia variabilis. 

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