[A case of progressive hemifacial atrophy associated with immunological abnormalities]

We reported a case of progressive hemifacial atrophy associated with immunological abnormalities. This patient, a 35-year-old woman, had a history of high fever of unknown etiology associated with anemia and leukocytopenia at the age of 19. Eight months later, right hemifacial atrophy, alopecia and partial lipodystrophy of the right upper arm were developed. And the progression had stopped in the latter twenties. At the age of 35, she visited our hospital because of the hemifacial atrophy. Physical examination revealed right hemifacial atrophy, large alopecia at parietal and right temporal region, and partial lipodystrophy of the right upper arm. CT, MRI and ultrasound tomography of the head and the arm showed skin and scalp atrophy, temporal muscle atrophy and decrease of subcutaneous soft tissues. There was no autonomic dysfunction. Serum IgG was increased, and RA factor, anti-nuclear antibody, anti-DNA antibody and LE cell were positive. In this case, immunological abnormalities were considered to be related to the pathogenesis of hemifacial atrophy.