Erythrokeratodermia variabilis with erythema gyratum repens-like lesions.
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.
Landau
Marina
M
Dermatology Unit, Edith Wolfson Medical Center, Holon, Israel, Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel. landau@post.tau.ac.il
Cohen-Bar-Dayan
Meirav
M
Hohl
Daniel
D
Ophir
Joseph
J
Wolf
Charles R
CR
Gat
Andrea
A
Mevorah
Barukh
B
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