Pediatric Dermatology Vol. 14 No. 2 113-116, 1997 Neurologic Abnormalities in Two Patients with Facial Hemiatrophy and Sclerosis Coexisting with Morphea Silvano Menni, M.D., Angelo Valerio Marzano, M.D., and Emanuela Passoni, M.D. Instiuae of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore, Milan, Italy Abstract: Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity characterized by unilateral atrophy ofthe skin, subcutane- ous tissue, and the underlying bony structures. This syndrome has many features of linear scleroderma en coup de sabre but is distinguished by more extensive involvement ofthe lower face and by only slight cutaneous sclerosis. We describe two unusual children wrth both atrophic and scle- rotic changes of haif of the face coexisting with multiple piaques of typical morphea. Both children developed neurologic disturbances with cranial magnetic resonance imaging (MRi) abnormalities 2 years and 15 years, respectively, after the onset of cutaneous lesions. Thus considering that it may not be possible to correlate impairment in neurologic function and cutaneous disease, as illustrated by our patients, we emphasize the importance of an accurate foilow-up. Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity of unknown origin characterized by unilateral atrophy of the skin, subcutaneous tissue, and underlying bony structures of the face and cranium (1,2). There have been several reports in which this der- matologic disorder was associated with central nervous system (CNS) abnormalities (3-7). CNS involvement has also been described in scleroderma en coup de sabre, a form of linear sclerodeiTna affecting the frontopadetal area of the face and scalp (8-13). We report two unusual children displaying both attophic and sclerotic hemifacial changes and, concur- rently, multiple plaques of classic morphea, the most common form of localized sclerodertna, on various sites of the body. Both patients showed neurologic manifesta- tions several years after the onset of cutaneous features. CASE REPORTS Case 1 A 17-year-old boy had a bandlike sclerotic lesion over his right forehead and parietal region extending down to and involving the ipsilateral nasal ala. The lesion was first noted when he was 7 years of age, and subsequently progressive atrophy of the entire right side of the face developed (Fig. 1), Some months later multiple circum- scribed sclerotic plaques with a violaceous halo appeared on various areas of his trunk, particularly on the back. These plaques became inactive widi atrophic and pig- mentary changes and stopped occurring over a 10-)'ear period (Fig. 2). He had initially been treated with petiicillin GIV for 10 days at a dose of 10,000,000 lU daily. He was then given Address correspondence to S. Menni, M.D., Institute of Derma- tological Sciences, Via Pace 9, 20122 Milan, Italy. 113