pachyonychia congenita

pachyonychia

[pak″e-o-nik´e-ah] abnormal thickening of the nails.pachyonychia conge´nita a congenital autosomal dominant syndrome primarily affecting males, characterized by increased thickness of the nails, hyperkeratosis involving the palms, soles, knees, and elbows, widespread tiny cutaneous horns, leukoplakia of the mucous membranes, and usually excessive sweating of the hands and feet; sometimes associated with development of bullae on palms and soles after trauma.

pach·y·o·nych·i·a con·gen·'i·ta

[MIM*167200] a syndrome of ectodermal dysplasia of abnormal thickness and elevation of nail plates with palmar and plantar hyperkeratosis; the tongue is whitish and glazed owing to papillary atrophy; autosomal dominant inheritance caused by mutation in the keratin 16 gene (KRT16) on chromosome 17q or the keratin 6A gene (KRT6A) on 12q. Synonym(s): Jadassohn-Lewandowski syndrome

pach·y·o·nych·i·a con·gen·i·ta

(pakē-ō-nikē-ă kŏn-jeni-tă) Syndrome of ectodermal dysplasia of abnormal thickness and elevation of nail plates with palmar and plantar hyperkeratosis; the tongue is whitish and glazed owing to papillary atrophy.
Synonym(s): Jadassohn-Lewandowski syndrome.

Jadassohn,

Josef, German dermatologist in Switzerland, 1863-1936. Borst-Jadassohn type intraepidermal epithelioma - see under BorstFranceschetti-Jadassohn syndrome - Synonym(s): Naegeli syndromeJadassohn nervus - congenital papillary acanthosis of the epidermis, with hyperplasia of sebaceous glands developing at puberty and presence of apocrine glands in nonapocrine areas of the skin. Synonym(s): nevus sebaceusJadassohn-Lewandowski syndrome - ectodermal dysplasia of abnormal thickness and elevation of nail plates with palmar and plantar hyperkeratosis. Synonym(s): pachyonychia congenitaJadassohn-Pellizzari anetoderma - cutaneous atrophy preceded by erythematous or urticarial lesions of the trunk and upper portions of the extremities.Jadassohn-Tièche nevus - a dark blue or blue-black nevus covered by smooth skin and formed by heavily pigmented spindle-shaped or dendritic melanocytes in the reticular dermis. Synonym(s): blue nevus

单词	pachyonychia congenita
释义	pachyonychia congenita pachyonychia [pak″e-o-nik´e-ah] abnormal thickening of the nails.pachyonychia conge´nita a congenital autosomal dominant syndrome primarily affecting males, characterized by increased thickness of the nails, hyperkeratosis involving the palms, soles, knees, and elbows, widespread tiny cutaneous horns, leukoplakia of the mucous membranes, and usually excessive sweating of the hands and feet; sometimes associated with development of bullae on palms and soles after trauma. pach·y·o·nych·i·a con·gen·'i·ta [MIM167200] a syndrome of ectodermal dysplasia of abnormal thickness and elevation of nail plates with palmar and plantar hyperkeratosis; the tongue is whitish and glazed owing to papillary atrophy; autosomal dominant inheritance caused by mutation in the keratin 16 gene (KRT16) on chromosome 17q or the keratin 6A gene (KRT6A) on 12q. Synonym(s): Jadassohn-Lewandowski syndrome pach·y·o·nych·i·a con·gen·i·ta (pakē-ō-nikē-ă kŏn-jeni-tă) Syndrome of ectodermal dysplasia of abnormal thickness and elevation of nail plates with palmar and plantar hyperkeratosis; the tongue is whitish and glazed owing to papillary atrophy. Synonym(s): Jadassohn-Lewandowski syndrome. Jadassohn, Josef, German dermatologist in Switzerland, 1863-1936. Borst-Jadassohn type intraepidermal epithelioma* - see under BorstFranceschetti-Jadassohn syndrome - Synonym(s): Naegeli syndromeJadassohn nervus - congenital papillary acanthosis of the epidermis, with hyperplasia of sebaceous glands developing at puberty and presence of apocrine glands in nonapocrine areas of the skin. Synonym(s): nevus sebaceusJadassohn-Lewandowski syndrome - ectodermal dysplasia of abnormal thickness and elevation of nail plates with palmar and plantar hyperkeratosis. Synonym(s): pachyonychia congenitaJadassohn-Pellizzari anetoderma - cutaneous atrophy preceded by erythematous or urticarial lesions of the trunk and upper portions of the extremities.Jadassohn-Tièche nevus - a dark blue or blue-black nevus covered by smooth skin and formed by heavily pigmented spindle-shaped or dendritic melanocytes in the reticular dermis. Synonym(s): blue nevus FinancialSeep/c
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