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KMID : 0363220140520040260
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Korean Journal of Dermatology
2014 Volume.52 No. 4 p.260 ~ p.263
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Erythrokeratodermia Variabilis Showing Gradual Disappearance of Erythema
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Kim Hyo-Jin
Park In-Ho
Kang Jeong-Nan
Seol Jung-Eun
Sung Ho-Suk
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Abstract
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Erythrokeratodermia variabilis is an autosomal-dominant inherited disease associated with a mutation in gap junctionbeta (GJB) 3 and 4. It shows two characteristic features: migratory and irregularly shaped erythematous lesionsusually accompanied by a burning sensation and fixed, symmetrically located hyperkeratotic plaques. A 6-year-oldboy had developed erythematous scaly patches with a geographic pattern on the entire body at age 1, and thelesions had migrated with an irregular pattern. Accompanying hyperkeratotic plaque developed on the trunk and bothlegs when he was 4 years old. As he grew older, the erythematous patches progressively disappeared and thehyperkeratotic 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 andpathologic features, we diagnosed erythrokeratodermia variabilis in this patient. Herein, we report a case of erythrokeratodermiavariabilis showing gradual disappearance of erythema.
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KEYWORD
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Erythrokeratodermia variabilis, Gap junction beta, GJB 3, GJB 4
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