Abstract
Pachyonychia congenita (PC, OMIM #615726) is a rare autosomal-dominant genodermatosis divided into two subtypes: PC-1 (Jadassohn-Lewandowski type, OMIM#167200) and PC-2 (Jackson-Lawler type, OMIM#167210). Both subtypes manifest with hypertrophic onychodystrophy and a variable degree of focal palmoplantar keratoderma (PPK), angular cheilitis and follicular keratoses of elbows, knees and hips. PC-1 usually shows most prominent leukokeratosis and is associated with K6a and K16 mutations. PC-2 shows [...]
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