Hereditary disorder characterized by thickened fingernails and toenails. Several clinical variants have been described. Patients present at birth or during the first year of life with hypertrophy and yellowish-brown discolouration of the nails, in some cases associated with nail bed and hyponychial hyperkeratosis. Hyperkeratosis and hyperhidrosis of the palms and soles, acral bullae, follicular keratosis, and oral leukokeratosis may also occur. Autosomal dominant inheritance is the rule, although cases transmitted in an autosomal recessive form have also been reported.
Pachyonychia Congenita Hereditaria