Pachydermoperiostosis. Touraine-Solente-Gole syndrome

Abstract

Two patients with idiopathic pachydermoperiostosis are described. The cutaneous changes consisted of large folds with deep furrows in the skin of the forehead, cheeks, and scalp. The skeletal changes were periostosis of the long bones, with enlargement of the hands and clubbing. Widespread papular mucinosis developed in one patient in whom a paraproteinemia of immunoglobulin-G-type was demonstrated. Large keloids were present in the other. Hyperkeratotic linear lesions of the skin of the palms and soles were present in both. The fold consisted of a dense collection of fibrocytes and dermal collagen without an increase in elastic fibers. Similar findings with large amounts of acid mucopolysaccharides were present in the hand skin. In the papular lesions of the trunk and extremities large accumulations of a similar substance were found. Hypertrophy of epidermal appendages was noted. Plastic surgery of the forehand and eyelids resulted in rehabilitation of one patient. Early recognition of acquired forms of an identical clinical syndrome is important in view of its association with carcinoma of the lung.