A case of disproportionate macrodactyly or a mild form of Proteus syndrome? An interesting case.

[proteus syndrome]

We present a 20 -year -old Malay male whom we believe has Proteus syndrome , a rare congenital disorder of asymmetrical overgrowth of body tissues . There are fewer than 100 confirmed cases reported worldwide thus the clinical presentation and histopathological findings are of significance . Our patient presented with an overgrown right small finger and subcutaneous purplish pigmentation over his left upper arm and chest since birth . His small finger gradually increased in size . He had no abnormalities in sensation or power . Radiographs revealed a delta shaped middle phalanx of the small finger . His activities of daily living were uninterrupted but he requested debulking surgery for cosmetic reasons . Histopathological examination reported hypertrophic fatty tissue composed of well formed lobules of mature adipocytes interspersed with fibrous elements .