Figure 2 - uploaded by Mohammad Adil
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Oral leukokeratosis on the dorsal aspect of the tongue Figure 1: (a and b) Dark brownish?colored dystrophic fingernails with hyperconvex nail plates. (c and d) Dystrophic toenails d c
Source publication
Pachyonychia congenita (PC) is a group of rare ectodermal
dysplasias inherited usually in autosomal dominant
fashion and characterized by early onset of nail dystrophy
and abnormal keratinization of mucosa and skin.PC
tarda (PCT) is a special type of PC characterized by later
onset, usually in the second and third decades. Only a
few cases of PCT h...
Citations
... Wrodzona pachyonychia congenita typu 3 charakteryzuje się leukokeratozą rogówki [9]. Wrodzona pachyonychia congenita typu 4 zwana jest również PC tarda, ponieważ pojawia się w drugiej lub trzeciej dekadzie życia [10]. ...
... Pachyonychia congenita type 2 presents with features of PC type 1 with lesser thickening of the soles, prenatal teeth, steatocystomas and various cysts, while PC type 3 has features of corneal leukokeratosis [9]. Pachyonychia congenita type 4 is also called PC tarda, as it presents in the second and third decades of life [10]. ...
Pachyonychia congenita (PC) is a very rare group of autosomal dominant genodermatoses caused by mutations in one of the genes that encode for nail keratin [1]. It has traditionally been classi ed into four types, with type 1 (Jadassohn-Lewandowsky type) and type 2 (Jackson-Lawler type) being the most common variants. We present here a case of PC type 1 with classical features.