Erythroderma, Lethal Congenital

Shield et al. (1992) reported 4 patients and their extended families comprising 17 cases, all of whom had congenital exfoliative erythroderma resistant to treatment, associated with failure to thrive and hypoalbuminemia. All died in the first year of life. Three of the families had recognized consanguinity. In the fourth family, the parents were said to be nonconsanguineous but were a Turkish Cypriot couple coming from the same village. Other families were Turkish, Omani, and Pakistani. The erythroderma dated from birth, with no obvious nail or hair-shaft abnormalities.