CHILD综合征长期随访一例

Long-term follow-up of a case of CHILD syndrome

患儿女,87 d龄,出生后因右侧躯体片状红色斑块伴同侧肢体畸形于2012年1月就诊。皮肤科检查:右下腹、右臀部、右侧会阴部、右腿及右足部见明显红色斑块,表面附着黄色鳞屑,边界清楚,无明显渗出及异味;右下颌、右颈部、右腋下及右手第1~4指指背见淡黄色疣状增生;右足第2、3、4趾只有1个趾间关节且甲板缺如。右足X线片显示右足畸形及骨质缺损。诊断:CHILD综合征。患儿间断口服阿维A胶囊,外用凡士林、他卡西醇软膏、弱效糖皮质激素软膏6年余,用药时皮损可缓解,停药易反复,后改外用辛伐他汀软膏2年。皮损随年龄增长等比例增大,患肢较健侧肢体生长速度稍快。至2020年1月患儿右下颌、右颈部及右手第1~4指皮损消退,右手第5指出现新发带状疣状增生性斑块,右小腿皮损较前稍好转;无其他系统受累表现。

A 87-day-old female patient presented with patchy erythema on the right body and ipsilateral limb deformity after birth,and visited the hospital in January 2012.Skin examination showed obvious red plaques on the right lower abdomen,right buttock,right perineum,right leg and right foot,with yellow scales on the surface and clear boundaries,and there was no obvious exudation or odor;pale yellow verrucous hyperplasia was observed on the right lower jaw,right neck,right axilla,and on the back of the first to fourth fingers of the right hand;only 1 interphalangeal joint and no nail plate was observed on the second,third and fourth toes of the right foot.The X-ray of the right foot showed deformity and bone defects.The patient was diagnosed with CHILD syndrome,and treated with intermittent oral acitretin capsules,and topical vaseline ointment,tacalcitol ointment and weak-potency glucocorticoid ointment for more than 6 years.The skin lesions regressed during treatment,but occurred repeatedly after withdrawal.Thereafter,the patient was switched to simvastatin ointment for 2 years.The skin lesions increased proportionally with the increase of age,and the affected limb grew a little more rapidly than the healthy limb.By January 2020,the skin lesions on the right lower jaw,right neck,and the first to fourth fingers of the right hand had subsided,but new band-like hypertrophic verrucous plaques occurred on the fifth finger of the right hand;the skin lesions on the right leg were slightly improved,and no involvement of other systems was observed.