ERYTHROKERATODERMIA VARIABILIS

Course overview

ERYTHROKERATODERMIA IS A GROUP OF INHERITED DISORDERS CHARACTERIZED BY WELL-DEMARCATED ERYTHEMATOUS LESIONS AND HYPERKERATOTIC PLAQUES. ERYTHROKERATODERMIA VARIABILIS (EKV), FORMERLY KNOWN AS MENDES DA COSTA SYNDROME, USUALLY INVOLVES MIGRATORY RED PATCHES ALONG WITH FIXED LOCALIZED OR GENERALIZED KERATOTIC PLAQUES. LATER, CLINICAL FEATURES OF PROGRESSIVE SYMMETRIC ERYTHROKERATODERMIA (PSEK) WERE OBSERVED WITHIN A SINGLE FAMILY THAT ALSO HAD EKV FEATURES. THE FORMER THINKING THAT THESE DISEASES WERE DISTINCT ENTITIES CHANGED, AND EKV AND PSEK SUBSEQUENTLY HAVE BEEN CONSIDERED MANIFESTATIONS OF A SINGLE INHERITED CLINICAL ENTITY. WITH THE IDENTIFICATION OF THE SAME GENE MUTATION IN PATIENTS WITH EKV AND PSEK, SOME AUTHORS PROPOSED THE DESIGNATION OF ERYTHROKERATODERMIA VARIABILIS PROGRESSIVA (EKVP) TO ENCOMPASS THE DIVERSITY OF THE CLINICAL PHENOTYPES OF BOTH EKV AND PSEK. THIS ACTIVITY EXPLAINS WHEN ERYTHROKERATODERMIA VARIABILIS SHOULD BE CONSIDERED, HOW TO PROPERLY EVALUATE FOR THIS CONDITION, AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN CARING FOR PATIENTS WITH THIS CONDITION.