A Case of Parry-Romberg Syndrome with Scleroderma <i>en Coup de Sabre</i>

MATSUMOTO Keiko, SHIRAISHI Ken, TOHYAMA Mikiko, SENZAKI Kensuke, SAYAMA Koji

doi:10.2336/nishinihonhifu.79.473

<p>A 42-year-old Japanese man presented with numbness of his left face and pain on his left side of the forehead 1 year prior to his initial visit to our clinic. At the first medical examination, the left cheek and nose wing were clearly depressed. The left side of the forehead presented pigmentation and an atrophic sclerosing plaque, while the contiguous region on the left frontal side of the head appeared atrophic and hairless. Computed tomography demonstrated a decreased amount of fat tissue at the left cheek. A skin biopsy from the left frontal side of the head showed a thickened dermis composed of abundant collagen bundles. We diagnosed Parry-Romberg syndrome together with scleroderma en coup de sabre. These two disorders frequently overlap ; thus, they are thought to be on the same spectrum of localized scleroderma. Both often accompany central nervous system imaging abnormalities without central nervous system symptoms. Since neurologic findings are not always revealed in correlation with skin lesions, neuroimaging studies should be considered in Parry-Romberg syndrome cases at the time of diagnosis.</p>

A Case of Parry-Romberg Syndrome with Scleroderma <i>en Coup de Sabre</i>

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