A Case of Parry-Romberg Syndrome with Shortening of Ipsilateral Lower Extremity / 대한피부과학회지

ABSTRACT

Parry-Romberg syndrome is an extremely rare connective tissue disorder. It might be a form of linear scleroderma, and it manifests as progressive hemifacial atrophy, epilepsy, exophthalmos or alopecia. Herein we report a case of Parry-Romberg syndrome. A 68-year-old woman had left hemifacial atrophy, shortening and deformity of the left leg, and deformities to her side fingers that occurred from her 1st decade to 2nd decade. Sclerotic change had stopped spontaneously when she was 20 years old. Histopathologically, there were a few signs of skin appendages, but no sclerotic change. On autoimmune antibody test, no positivity was shown. X-ray showed shrinkage of the lung field, elevation of the left diaphragm, shortening of length and reduction of bone mass in the left femur, tibia, and fibula. And there were multiple melorheostasis. Neck CT showed left facial sclerotic change, bone mass loss, and multiple melorheostasis. Because the progression of sclerosis had stopped the patient did not receive any treatment.