COMEL-NETHERTON-SYNDROME

The Comel-Netherton syndrome is a rare autosomal recessive hereditary disease. A 23-year old female presented with the classical triad of ic hthyosis linea ris circumflexa, trichorrhexis invaginata with bamboo h airs of up to 12 cm length and atopic diathesis. Nevertheless, more th an 20 yea rs passed before the final diagnosis was established.ln addi tion, the patient was slightly mentally retarded and suffered from a g enital papillomatosis, minimal hypergammaglobulinaemia and a marked bi lateral eyelid ectropion, more severe than previously reported. Oral t herapy with Acitretin was quite successful.