[Netherton-Comel's syndrome]

Abstract

Two cases of the so-called Netherton's syndrome are described and the relevant literature is reviewed. On the author's opinion there exists no relationship between the disease and ichthyosiform dermatoses. It fits more adequately in the group of the "Génodermatoses érythémato-desquamatives circinées variables" of Bazex and Dupré. The syndrome is characterized by the association of two tipical clinical features: "Trichorrhexis invaginata", reported by Netherton and the "Ichthyosis linearis circumflexa", described by Comel. The authors propose therefore the eponymous designation of "Syndrome of Netherton-Comel". As there exists no link with the group of the ichthyosiform dermatoses, the authors suggest the name of "Dyskeratosis figurata variabilis" for the disease described described by Comel.