pachyonychia


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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

(pak'ē-ō-nik'ē-ă),
Abnormal thickness of the fingernails or toenails.
[pachy- + G. onyx, nail]

pachyonychia

/pachy·onych·ia/ (pak″e-o-nik´e-ah) abnormal thickening of the nails.
pachyonychia conge´nita  a rare, congenital, dominantly inherited disorder marked by great thickening of the nails, hyperkeratosis of palms and soles, and leukoplakia.

pachyonychia

[pak′ē·ōnik′ē·ə]
an abnormal thickness of the fingernails or toenails.

pach·y·o·nych·i·a

(pak'ē-ō-nik'ē-ă)
Abnormal thickness of the fingernails or toenails.
[pachy- + G. onyx, nail]

pachyonychia

Abnormal thickness of the nails.

pachyonychia

abnormal thickening of the nails or claws.
References in periodicals archive ?
Notably, inherited keratin mutations causing type 2 pachyonychia congenita can produce EVHC along with a characteristic nail dystrophy, focal palmoplantar keratoderma, follicular keratosis, and multiple steatocysts.
Differential diagnosis includes Werner syndrome, focal dermal hypoplasia, alopecia areata, aplasia cutis congenita, incontinentia pigmenti, dyskeratosis congenital, familial simple anhidrosis, Naegeli-Franceschetti-Jadassohn syndrome, and pachyonychia congenita.
Hereditary causes include pachyonychia congenita, Mal de Meleda, Unna-Thost, Vohwinkel, Papillon-Lefevre syndrome, [3,4] mutilating keratoderma, lamellar ichthyosis.
Pachyonychia congenita tarda, another variant, characterized by a later onset ranging from late childhood to middle age has been described.
Differential Diagnosis of Onychomycosis (18-21) * Nail trauma * Psoriasis * Lichen planus * Paronychia * Bacterial infection * Pachyonychia congenita * Nail bed tumors (squamous cell carcinoma) and verrucae * Yellow nail syndrome * Alopecia areata * Contact/atopic dermatitis * Idiopathic onycholysis * Twenty-nail dystrophy (trachyonychia) * Nail changes associated with systemic disease or nail cosmetics
Mutations in these KRTs have been shown to cause pachyonychia congenita, a rare genodermatosis characterized by dystrophic, thickened nails and painful palmoplantar keratoderma.
Ellis Van Creveld syndrome (chondroectodermal dysplasia),9 cyclopia, pachyonychia congenita, cleft lip and palate, Down's syndrome, Pierre-Robin, Pallister-Hall, short rib-polydactyly type II, Wiedeman-Rautenstrauch (neonatal progeria).
Steatocystoma multiplex can be associated with pachyonychia congenita type 2 (which is characterized by hypertrophic nail dystrophy, focal keratoderma, multiple pilosebaceous cysts, and myriad conditions associated with ectodermal dysplasia) or eruptive vellus hair cysts [1,5].
Considering the symptoms of skin pigmentation, nail dystrophy and oral white patch, a differential diagnosis of pachyonychia congenita and dyskeratosis congenita were considered, and skin histopathological analysis was sent.
Moon SE, Lee YS, Youn II: Eruptive vellus hair cyst and steatocystoma multiplex in a patient with pachyonychia congenita.
Citation: "Development of SkinHumanized Mouse Models of Pachyonychia Congenita;" Marta Garcia, Marcela Del Rio, et al.
Citation: "Development of Skin-Humanized Mouse Models of Pachyonychia Congenita;" Marta Garcia, Marcela Del Rio, et al.