Mutilating palmoplantar keratoderma with oral involvement

We report a 26 year-old man with bilateral progressive palmoplantar keratoderma, automutilation of the 3rd, 4th and 5th toes of the left foot, and recurrent episodes of gingivitis in childhood. The patient had signs common to both Papillon-Lefevre and Vohwinckel syndromes. Acitretin was given (30 mg/day) with good results.