Abstract
A Japanese woman was diagnosed as having progressive facial hemiatrophy when she was 26 years old. After 30 years, Raynaud's phenomenon and sclerodactyly suddenly appeared; at the same time, positive rheumatoid factor and anti-ribonucleoprotein (anti-RNP) antibody were noted on serological examinations. When she was 60 years old, trigeminal nerve disturbance also appeared. The associations between progressive facial hemiatrophy, systemic scleroderma, and trigeminal nerve disturbance are interesting and should be discussed.
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