Erythroderma, Lethal Congenital

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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Other congenital malformations

OMIM Number

227090

Mode of Inheritance

Autosomal recessive

Description

Lethal congenital erythroderma is an extremely rare disorder in neonates characterized by peeling skin, failure to thrive, severe hypoalbuminemia, and small size. This disorder has been described in only four familes from Turkey, Oman, and Pakistan. All described patients have died before reaching one year of age.

Epidemiology in the Arab World

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Other Reports

Oman

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. The study included at least one Omani family.

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