epidermolytic hyperkeratosis

epidermolytic hyperkeratosis
an autosomal dominant disorder of keratinization caused by mutation in the keratin genes KRT1 (locus: 12q13) and KRT10 (locus: 17q21-q22), resulting in structural defects in tonofibrils. It is present at birth and characterized by generalized erythroderma and severe hyperkeratosis. There are small, hard, verrucous scales over the entire body, especially in flexural areas, including the creases on the palms and soles. Recurrent bullae, primarily on the lower limbs, are characteristic in infancy and childhood. See also ichthyosis hystrix.

Epidermolytic hyperkeratosis. Compact orthokeratosis overlies an epidermis showing granular and vacuolar changes in its upper layers.


Medical dictionary. 2011.

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  • hyperkeratosis — Thickening of the horny layer of the epidermis or mucous membrane. SEE ALSO: keratoderma, keratosis. SYN: hyperkeratinization. h. congenita SYN: ichthyosis vulgaris. diffuse h. of palms and soles an …   Medical dictionary

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  • EHK — epidermolytic hyperkeratosis …   Medical dictionary

  • EHK — • epidermolytic hyperkeratosis …   Dictionary of medical acronyms & abbreviations

  • List of cutaneous conditions — This is an incomplete list, which may never be able to satisfy particular standards for completeness. You can help by expanding it with reliably sourced entries. See also: Cutaneous conditions, Category:Cutaneous conditions, and ICD 10… …   Wikipedia

  • Keratin 10 — Identifiers Symbols KRT10; BCIE; BIE; CK10; EHK; K10; KPP External IDs OM …   Wikipedia

  • Palmoplantar keratoderma — Classification and external resources A picture of a 40 y/o Caucasian female with only the soles of the feet affected. The amputation was prior to this admission ICD 10 L …   Wikipedia

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