[Netherton's syndrome. Current aspects. Apropos of 9 cases]

Data concerning 9 cases of Netherton's syndrome (NS) have been collected from 6 French dermatology units (table I). Analysis of these data has confirmed the information previously published, notably the prevalence of congenital ichthyosis erythroderma (CIE) as cutaneous manifestation in the neonatal period (77 p. 100), hair shaft dysplasia being rarely found at that stage (11 p. 100). In the majority of cases (5/9 in our series), CIE evolves in adults as ichthyosis linearis circumflexia (ILC), with trichorrhexis invaginata (TI) as the predominant hair shaft dysplasia. These data are in agreement with the diagnostic elements laid down by Dupré and Traupe. Some points may have been underestimated in the past. They include: hypernatraemic dehydration in the neonate; short stature and low weight (unrelated to endocrine disorders; mental and neurological retardation possibly associated with seizures. Various manifestations of hypersensitivity have been noted in 26 p. 100 of the published cases and in 6 of our 9 patients. The aggravating role played by hypersensitivity may be considered in some cases. NS must be regarded as a broad-spectrum disease the margins of which could be isolated skin manifestations in ILC and ichthyosis erythroderma with various associated disorders in cases with severe illness.