نتایج جستجو برای: pachyonychia congenital

تعداد نتایج: 120621  

Journal: :Journal of Investigative Dermatology 2001

Journal: :Journal of Investigative Dermatology Symposium Proceedings 2005

2016
Anup Kumar Tiwary Dharmendra Kumar Mishra

PC is commonly described as a rare genodermatosis characteristically manifesting as massive subungual hyperkeratosis with nail thickening, focal palmoplantar keratoderma alongwith deep f issur ing and blistering, oral leukokeratosis and discrete follicular hyperkeratosis [1,2]. Other features including abnormalities of teeth, hairs and larynx can also be seen depending on the clinical types. On...

Journal: :The Journal of investigative dermatology 2011
Neil J Wilson Sancy A Leachman C David Hansen Alexandra C McMullan Leonard M Milstone Mary E Schwartz W H Irwin McLean Peter R Hull Frances J D Smith

Pachyonychia congenita (PC) is a rare autosomal dominant skin disorder characterized predominantly by nail dystrophy and painful palmoplantar keratoderma. Additional clinical features include oral leukokeratosis, follicular keratosis, and cysts (steatocysts and pilosebaceous cysts). PC is due to heterozygous mutations in one of four keratin genes, namely, KRT6A, KRT6B, KRT16, or KRT17. Here, we...

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