Respiratory function in progressive systemic sclerosis.

In progressive systemic sclerosis, sometimes referred to as generalized scleroderma, atrophic and sclerotic changes in the skin and internal organs are usually preceded by Raynaud's phenomenon. The disorder, which affects women more frequently than men, forms one of the socalled collagen vascular diseases and, although the aetiology is uncertain, there is evidence to suggest that auto-immune mechanisms are involved (Fennell, Rodnan and Vazquez, 1962; Rowell, 1962; Rowell and Beck, 1963). The cutaneous manifestations, which are called acrosclerosis, involve mainly the face, hands, and feet. Atrophy of the tissues of the face gives a shrunken, tight, and shiny appearance with a beaked nose and small mouth opening, around which there is radial furrowing (Fig. 1). The hands (Fig. 2) and, to a lesser extent, the feet show characteristic changes. Atrophy occurs first in the terminal phalanges, and spreads proximally to give tapering digits. The nails curve over the ends of the fingers and painful ulcers and whitlows are frequent. The skin of the fingers and hands is smooth and bound down to the deeper structures. The hands become fixed in a position of semiflexion and eventually may become very small.