Sayama K, Hato N, Matsuda O, Shiraishi S, Miki Y
Department of Dermatology, Ehime University School of Medicine, Japan.
Dermatology. 1994;189(4):392-5. doi: 10.1159/000246886.
Proteus syndrome is a rare hamartomatous syndrome with a variety of abnormalities. A 6-year-old Japanese boy without apparent abnormalities at birth developed by 1 year of age cerebriform skin tumors on the right sole, soft masses on the left sole, palms and fingers, brownish verrucous lesions and whorled brownish patches on the right side of the neck, chest, external genitals and extremities, hemihypertrophy of the right lower extremity, lordoscoliosis, protuberance of the skull, epileptic seizures, hydrocephalus and mental retardation. This patient appears to be the second Japanese case of Proteus syndrome.
变形综合征是一种罕见的错构瘤综合征,伴有多种异常表现。一名6岁日本男孩出生时无明显异常,1岁时右侧足底出现脑回状皮肤肿瘤,左侧足底、手掌和手指出现柔软肿块,颈部右侧、胸部、外生殖器和四肢出现褐色疣状病变及涡状褐色斑片,右下肢半侧肥大,脊柱侧凸,颅骨突出,癫痫发作,脑积水及智力发育迟缓。该患者似乎是日本第二例变形综合征病例。