|Bożena Kordys-Darmolińska, Joanna Kasznia-Kocot, Małgorzata Kozowicz, Halina Woś |
A case of a 17-year old boy with Netherton syndrome is presented. The correct diagnosis was established when he was 11 years old. Netherton syndrome is a rare autosomal recessive disease. It is characterised by a triad of symptoms: congenital ichthyosiform erythroderma, hair-shaft defect (trichorrhexis invaginata) and atopic diseases. Diagnostic and therapeutic problems, and a variable clinical course of atopic diseases with a typical allergic march was described. The patient showed a food allergy to fish with anaphylactic reactions, seasonal and perennial allergic rhinitis and asthma bronchiale. Correct therapy for a child with Netherton syndrome depends on early diagnosis and requires collaboration of many specialists.
keywords: zespół Nethertona, schorzenia atopowe, Netherton's syndrome, atopic disease
pages: from 62 to 64
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