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: Marina Landau, Meirav Cohen-Bar-Dayan, Daniel Hohl, Joseph Ophir, Charles R Wolf, Andrea Gat, Barukh Mevorah
• Journal: Pediatric dermatology (Pediatr Dermatol) 2002 Jul-Aug Vol. 19 Issue 4 Pg. 285-92 ISSN: 0736-8046 [Print] United States
• PMID: 12220269 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Aged
• Biopsy, Needle
• Child
• Erythema (genetics, pathology)
• Female
• Genetic Predisposition to Disease
• Heterozygote
• Humans
• Hyperkeratosis, Epidermolytic (genetics, pathology)
• Ichthyosiform Erythroderma, Congenital (genetics, pathology)
• Immunohistochemistry
• Infant
• Infant, Newborn
• Israel
• Male
• Middle Aged
• Pedigree
• Prognosis
• Sampling Studies
• Severity of Illness Index
• Skin Diseases, Genetic (diagnosis)