Progressive facial hemiatrophy: report of five cases and biochemical analysis of connective tissue.

Five cases of progressive facial hemiatrophy (PFH) are reported. A nonindurated depression on normal-colored skin was observed in the cheeks of 3 subjects, and 2 patients showed indurated, pigmented atrophic lesions associated with linear scleroderma or generalized morphea. Lipoatrophy with mild subcutaneous fibrosis was observed histologically in the patients with nonindurated depressions. In contrast, the patients with indurated lesions exhibited a marked dermal fibrosis and the disappearance of appendices in the dermis. When compared with unaffected skin used as a control, collagen and glycosaminoglycan contents were not different in diseased areas. However, the dermatan sulfate/hyaluronic acid ratio was increased 1.5- to 3.2-fold in PFH patients regardless of their clinical and histological differences. These results suggest that both types of PFH may be based on a similar connective tissue disorder.