title: Erythrokeratodermia variabilis with erythema gyratum repens-like lesions.
abstract: 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.
authors.affiliation: Dermatology Unit, Edith Wolfson Medical Center, Holon, Israel, Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. landau@post.tau.ac.il
authors.forename: Marina
authors.initials: M
authors.lastname: Landau
authors.forename: Meirav
authors.initials: M
authors.lastname: Cohen-Bar-Dayan
authors.forename: Daniel
authors.initials: D
authors.lastname: Hohl
authors.forename: Joseph
authors.initials: J
authors.lastname: Ophir
authors.forename: Charles R
authors.initials: CR
authors.lastname: Wolf
authors.forename: Andrea
authors.initials: A
authors.lastname: Gat
authors.forename: Barukh
authors.initials: B
authors.lastname: Mevorah
mesh_terms: D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D002648:Child; D004890:Erythema / Q000235:genetics / Q000473:pathology; D005260:Female; D020022:Genetic Predisposition to Disease; D006579:Heterozygote; D006801:Humans; D017488:Hyperkeratosis, Epidermolytic / Q000235:genetics* / Q000473:pathology*; D016113:Ichthyosiform Erythroderma, Congenital / Q000235:genetics / Q000473:pathology; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D007557:Israel; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011379:Prognosis; D012494:Sampling Studies; D012720:Severity of Illness Index; D012873:Skin Diseases, Genetic / Q000175:diagnosis