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Pachyoinychia congenita

Abstract:

Pachyonychia congenita was first described in 1906 by Jadassohn and Lewandowsky in a patient presenting palmoplantar keratodermia, dystrophic nails, hyperhydrosis, bullae in feet during the summer and leucokeratosis. A 24-year-old man presented palmoplantar hyperkeratosis, dystrophy of the 20 nails, plantar hyperhydrosis with blister formation, oral leucokeratosis, follicular keratosis, hoarseness and lesions involving interarytenoid area. There was no history of consanguinity between the parents. A review of the syndrome and the attempts to classificate these disorders of keratinization is made, as well as it's possible treatment.