[Netherton syndrome: weakened immunity, generalized verrucosis and carcinogenesis].
Two patients with Netherton's syndrome are presented who, in addition to the classical triad of trichorrhexis invaginata, ichthyosis linearis circumflexa and atopy, exhibit signs of reduced cellular immunity (negative skin tests to a battery of microbial antigens, impaired stimulation of lymphocytes with various mitogens and widespread viral warts resembling epidermodysplasia verruciformis). One patient had a squamous cell carcinoma possibly arising from a viral wart. It is suggested that Netherton's syndrome is part of the group of immunodeficiency syndromes with atopy-like symptoms.
D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000175:diagnosis / Q000276:immunology*; D002648:Child; D006801:Humans; D007057:Ichthyosis / Q000175:diagnosis / Q000276:immunology*; D007111:Immunity, Cellular; D008297:Male; D012878:Skin Neoplasms / Q000175:diagnosis / Q000276:immunology*; D014860:Warts / Q000276:immunology*
Netherton-Syndrom: Abwehrschwäche, generalisierte Verrukose und Karzinogenese.