Erythrokeratoderma Variabilis et Progressiva in Iraqi Population

摘要

Erythrokeratoderma variabilis et progressiva (EKVP) are heterogeneous inherited group of rare keratinising skin diseases characterized by well demarcated fixed hyperkeratotic plaques and transient erythematous patches. EKVP are a disorders caused by the mutation in the genes encoding connexin-31 (GJB3) and connexin-30.3 (GJB4), the components of gap junction, in an autosomal dominant and rarely in recessive manner. More than 200 cases are reported in patients with diverse genetic backgrounds. EKVP has been reported worldwide and most cases were whites of Northern and Middle European origin, but it also occurs in African Americans and Asians. Both sexes are affected equally. Herein, we report a total of seven cases of Iraqi patients with EKVP that appeared within the first year of life, aiming to shed light on this rare skin problem in order to help dermatologists to establish the right diagnosis and to keep EKVP included in differential diagnosis of other skin disorders that might have some similar clinical features.