erythrokeratodermia

erythrokeratodermia
A neurocutaneous syndrome characterized by papulosquamous erythematous plaques with onset shortly after birth; ataxia, nystagmus, dysarthria, and decreased tendon reflexes appear later in life; symmetrical progressive e. is inherited as an autosomal dominant disorder and does not involve the palms and soles. [erythro- + G. keras, horn, + derma, skin, + -ia, condition]
- e. variabilis [MIM*133200] a dermatosis characterized by hyperkeratotic plaques of bizarre, geographic configuration, associated with erythrodermic areas that may vary remarkably in size, shape, and position from day to day; hair, nares, and teeth are not affected; onset is usually in the first year of life; autosomal dominant or recessive inheritance, caused by mutationin the connexin gene encoding gap junction protein beta-3 (GJB3) on 1p.

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eryth·ro·ker·a·to·der·mia (ə-rith″ro-ker″ə-to-durґme-ə) erythema with hyperkeratosis.

Medical dictionary. 2011.

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Look at other dictionaries:

  • Erythrokeratodermia variabilis — Classification and external resources OMIM 133200 Erythrokeratodermia variabilis (also known as Erythrokeratodermia figurata variabilis, Keratosis extremitatum progrediens, Keratosis palmoplantaris transgrediens et progrediens, [1] …   Wikipedia

  • erythrokeratodermia progressiva symmetrica — progressive symmetric erythrokeratodermia a rare syndrome of aberrant keratinization, having variable inheritance; affected children develop verrucous plaques in symmetric distributions, especially over joints …   Medical dictionary

  • erythrokeratodermia variabilis — a rare autosomal dominant condition present at birth, characterized by both transient, migratory areas of erythroderma in various shapes and fixed plaques of hyperkeratosis. Called also keratoderma palmoplantare transgrediens and keratosis… …   Medical dictionary

  • эритрокератодермия — (erythrokeratodermia; эритро + кератоз + греч. derma кожа) сочетание эритродермии с участками кератоза …   Большой медицинский словарь

  • Эритрокератодерми́я — (erythrokeratodermia; Эритро + Кератоз + греч. derma кожа) сочетание эритродермии с участками кератоза. Эритрокератодермия вариабельная (е. variabilis) см. Эритрокератодермия изменчивая фигурная. Эритрокератодермия изменчивая фигурная (син.:… …   Медицинская энциклопедия

  • EKV — erythrokeratodermia variabilis …   Medical dictionary

  • keratoderma palmoplantare transgrediens — erythrokeratodermia variabilis …   Medical dictionary

  • keratosis palmoplantaris transgrediens — erythrokeratodermia variabilis …   Medical dictionary

  • Mendes da Costa syndrome — erythrokeratodermia variabilis …   Medical dictionary

  • EKV — • erythrokeratodermia variabilis …   Dictionary of medical acronyms & abbreviations

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