[Netherton syndrome with recurrent herpes of facial skin]

In this paper the author presents the case of a girl with the typical Netherton's syndrome associated with herpes simplex recidivans. There was no relevans family history. In the first of life her skin appeared erythrodermic and diagnosis of atopic dermatitis was established. The bronchial asthma developed at age of four years. Total IgE and number of cosinofiles were elevated. The intradermal tests with pollen grass, house dust, eggs and milk were positive. Intradermal tests cellular immunity (PPD, Trichophytin) were negative. The number of T cells and B cells in the peripheral blood were normal, but NK cells were slightly decreased. Once or twice a year she suffers since 5 years of life exacerbation of herpes recidivas on her right site of the face. Microscopic examination of hairs revealed the typical appearance of trichorrhexis invaginata. Erythroderma persisted until the age of 12 years. Since then polycyclic and serpiginous lesions appeared.