Pachyonychia congenita Jackson-Lawler type: a distinct malformation syndrome

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Abstract
A family with three members in two generations affected by pachyonychia congenita, hyperkeratosis and hyperhidrosis of the palms and soles, follicular keratosis, neonatal teeth and epidermoid cysts (Jackson‐Lawler syndrome) is described. The nosological autonomy of this condition is proposed and a further heterogeneity is suggested on the basis of histopathological changes in the subcutaneous cysts.