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Pediatr Dermatol. 2002 Jul-Aug;19(4):285-92.

Erythrokeratodermia variabilis with erythema gyratum repens-like lesions.

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  • 1Dermatology Unit, Edith Wolfson Medical Center, Holon, Israel, Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel.


A large pedigree with erythrokeratodermia variabilis (EKV) and erythema gyratum repens-like lesions is described. Clinical, laboratory, and histologic findings of this family are presented. The differential diagnoses of the following dermatoses with an erythematous and a hyperkeratotic component are discussed: erythrokeratodermia variabilis (Mendes da Costa), progressive symmetric erythrokeratoderma (Gottron), loricrin keratoderma, erythrokeratoderma en cocardes (Degos), Netherton syndrome, keratitis-ichthyosis-deafness (KID) syndrome, erythrokeratolysis hiemalis (Oudtshoorn disease), and nonbullous congenital ichthyosiform erythroderma.

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