毛发低硫营养不良(又名BIDS综合征)是一种罕见的综合征,其表现为低硫性脆发、智力障碍、低生育力及身材矮小.具有先天性鱼鳞病时又称IBIDS综合征.作者发现4例患者有高度光敏性,并结合文献上11例类似患者,提出PIBI(D)S是一种殊于IBIDS的独立的综合征,其可能与着色性干皮病(XP)的互补D组有联系.临床特点可能为常染色体隐性遗传.面容特殊,可表现为眼球内陷、小眼畸形、颏后缩、鼻瘦 Hair Low-sulfur malnutrition (aka BIDS syndrome) is a rare syndrome characterized by low-sulfur crisp hair, mental retardation, low fertility and short stature. Also known as IBDS syndrome with congenital ichthyosis The authors found that 4 patients were highly photosensitized and combined with 11 similar patients in the literature, suggesting that PIBI (D) S is an independent syndrome specific to IBIDS and may be associated with xeroderma pigmentosum Complementary group D. There are clinical features may be autosomal recessive. Face special, can be manifested as eye retraction, malformation, chin constriction, nasal lean