The Parry-Romberg syndrome is a rare and poorly understood disease characte
rized by slowly progressive, localized atrophy of the skin,subcutaneous tis
sue, muscles, and bones. The atrophy is typically localized in the face and
begins in youth. In some patients, imaging can show the lesions and atroph
y of the ipsilateral hemisphere of the brain. We report on a patient in who
m the disease has lasted 36 years and discuss the possibility that the Parr
y-Romberg syndrome is related to known autoimmune disorders of the soft tis
sue (e.g., linear scleroderma) and Rasmussen's syndrome. There are some rem
arkable clinical similarities between these two syndromes, including age of
onset, unilateral manifestation, and occurrence of focal seizures. It is m
ost probable that both diseases have an autoimmunological background.