Erythrokeratodermia Variabilis Showing Gradual Disappearance of Erythema.
- Author:
Hyojin KIM
1
;
In Ho PARK
;
Jeong Nan KANG
;
Jung Eun SEOL
;
Ho Suk SUNG
Author Information
1. Department of Dermatology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea. derma09@hanmail.net
- Publication Type:Case Report
- Keywords:
Erythrokeratodermia variabilis;
Gap junction beta (GJB) 3;
GJB 4
- MeSH:
Burns;
Child;
Erythema*;
Erythrokeratodermia Variabilis*;
Gap Junctions;
Humans;
Inflammation;
Leg;
Male;
Parturition;
Sensation
- From:Korean Journal of Dermatology
2014;52(4):260-263
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Erythrokeratodermia variabilis is an autosomal-dominant inherited disease associated with a mutation in gap junction beta (GJB) 3 and 4. It shows two characteristic features: migratory and irregularly shaped erythematous lesions usually accompanied by a burning sensation and fixed, symmetrically located hyperkeratotic plaques. A 6-year-old boy had developed erythematous scaly patches with a geographic pattern on the entire body at age 1, and the lesions had migrated with an irregular pattern. Accompanying hyperkeratotic plaque developed on the trunk and both legs when he was 4 years old. As he grew older, the erythematous patches progressively disappeared and the hyperkeratotic plaque dominantly remained. His family history was unidentifiable because he was adopted at birth. Pathologic findings showed hyperkeratosis and superficial perivascular inflammation. Based on the clinical and pathologic features, we diagnosed erythrokeratodermia variabilis in this patient. Herein, we report a case of erythrokeratodermia variabilis showing gradual disappearance of erythema.
