pachyonychia congenita

pachyonychia congenita
a rare genetically determined skin disorder (see genodermatosis) characterized by thickening of the nails together with other ectodermal abnormalities.

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a genetically heterogeneous syndrome characterized by increased thickness of the nails that progresses to onychogryphosis; hyperkeratosis of the palms, soles, knees, and elbows; widespread tiny cutaneous horns; leukoplakia of the mucous membranes; and usually hyperhidrosis of the hands and feet. Bullae may develop on the palms and soles following trauma. It is usually an autosomal dominant disorder caused by mutations affecting any of several genes encoding various keratins; inheritance may rarely be autosomal recessive. Called also Jadassohn-Lewandowsky syndrome.

Medical dictionary. 2011.

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  • Pachyonychia congenita — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 32826 ICD10 = ICD10|Q|84|5|q|80 ICD9 = ICD9|703.8 ICD9|757.5 ICDO = OMIM = MedlinePlus = eMedicineSubj = derm eMedicineTopic = 812 MeshID = D009264Pachyonychia congenita, also called simply… …   Wikipedia

  • Pachyonychia congenita of the Jadassohn-Lewandowski type — This is a type of pachyonychia congenita (elephant nails from birth). The characteristic features include: {{}}Abnormally thick curved nails (onychogryposis) Thickening of the skin (hyperkeratosis) of the palms, soles, knees and elbows White… …   Medical dictionary

  • Pachyonychia congenita with natal teeth — This is a type of pachyonychia congenita (elephant nails from birth) in which teeth are evident at birth. It is called the Jadassohn Lewandowski syndrome. The characteristic features include: {{}}Abnormally thick curved nails (onychogryposis)… …   Medical dictionary

  • Pachyonychia congenita, type 1 — This is a form of pachyonychia congenita (elephant nails from birth) in which teeth are evident at birth. It is called the Jadassohn Lewandowski syndrome. The characteristic features include: {{}}Abnormally thick curved nails (onychogryposis)… …   Medical dictionary

  • pachyonychia congenita — a rare genetically determined skin disorder (see genodermatosis) characterized by thickening of the nails together with other ectodermal abnormalities …   The new mediacal dictionary

  • Пахионихия Врожденная (Pachyonychia Congenita) — редкая наследственная патология, характеризующаяся утолщением ногтей. Источник: Медицинский словарь …   Медицинские термины

  • Pachyonychia — Elephant nail, a fingernail or toenail that is abnormally thick. From the Greek pachys, thick + onyx, nail = thick nail. * * * Abnormal thickness of the fingernails or toenails. [pachy + G. onyx, nail] p. congenita [MIM*167200] a syndrome of… …   Medical dictionary

  • Aplasia cutis congenita — Classification and external resources Aplasia cutis congenita ICD 10 Q …   Wikipedia

  • Jadassohn-Lewandowsky syndrome — pachyonychia congenita …   Medical dictionary

  • Пахиони́я врождённая — (pachyonychia congenita; греч. pachys толстый, плотный + onyx, onychos ноготь) наследственная болезнь, проявляющаяся поражением ногтей, очаговым кератозом кожи, лейкокератозом слизистых оболочек, см. Ногти …   Медицинская энциклопедия

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