骨膜增生厚皮症并发肺囊肿和脊柱侧弯

Pachydermoperiostosis complicated by pulmonary cyst and scoliosis:a case report and review of the literature

报告1例并发肺囊肿和脊柱侧弯的骨膜增生厚皮症,并进行文献复习。患者男,20岁。头面部皮肤增厚,伴指、趾末端肥大进行性加重2年。经胸部、双手、双足、双侧尺桡骨、胫腓骨的X线检查,胸部CT检查,ECT(全身骨显像)及面部皮损组织病理检查,患者具有皮肤肥厚、杵状指等特征,并发肺囊肿及脊柱侧弯,诊断为骨膜增生厚皮症(亚临床型)。给予维胺酯口服3个月,患者皮损改善。骨膜增生厚皮症具有典型的临床特征、组织病理及影像学表现,不难诊断,维A酸类可以改善皮肤症状。

One case of pachydermoperiostosis complicated by pulmonary cyst and scoliosis is reported here and the literatures reviewed to enhance the understanding of the disease. The patient was a 20-year-old male, presented with a history of facial skin thickening and hypertrophy of the end of fingers and toes for 2 years. He was undergone medical history analysis, laboratory tests, X-ray of the chest, hands, feet, bilateral radius and ulna, tibia and fibula, chest CT scan, ECT （whole body bone scan）, and facial skin lesions pathological examination. The patient had the characteristics of pachydermoperiostosis, such as hypertrophy and clubbed fingers, coupling with pulmonary cysts and scoliosis. The diagnosis of pachydermoperiostosis （subclinical type） was confirmed. After giving oral viaminate capsules for 2 months, the skin lesions of patients improved. Pachydermoperiostosis has typical clinical features, the manifestations of pathology and iconography. It＇s not difficult to diagnose, and retinoic acid can improve skin symptoms.