Acitretin has been documented as an effective treatment for erythrokeratodermia variabilis (EKV) in adults; however, its use in children has not been previously documented. This case report discusses the use of acitretin in a 9-year old girl with EKV. EKV is a rare genodermatosis associated with a defect in the gene coding for gap junction protein beta-3 (GJB3), connexin 31. The resulting defect leads to a clinical presentation with erythematous patches that change in shape and position over time, while the concurrent hyperkeratotic plaques remain persistent. Acitretin at a dose of 15 mg/day (approximately 1 mg/kg) was initiated in this patient, and a dramatic improvement was noted after three weeks of treatment. The dose was subsequently reduced to 10 mg/day, which the patient continued without side effects for several months. However, when the patient discontinued the drug, the skin lesions recurred. Pulsed treatment during the summer months is planned for this patient, as EKV tends to worsen during warmer months.
Graham-Brown RAC and Chave TA. Acitretin for Erythrokeratodermia Variabilis in a 9-year-old girl. Pediatric Dermatology 2002; 19(6):510-512.
COPYRIGHT 2003 Journal of Drugs in Dermatology
COPYRIGHT 2003 Gale Group
Contact
Home
A
8-Hour Bayer