脂囊瘤样皮损表现的结节病一例

A case of sarcoidosis with cutaneous lesions resembling steatocystoma

47岁男性患者,咳嗽、咳痰10余年,伴颈部、上肢、躯干多发肤色结节1年。查体见颈部、双上肢、腰背部散在绿豆至黄豆大小脂囊瘤样肤色实性结节,表面光滑,界清、活动度好,无压痛。肺部计算机断层扫描示:双肺间质弥漫性病变,双肺门及纵膈淋巴结增大。皮损组织及肺部支气管镜活检显示真皮中深部和肺组织内见较多由上皮样细胞构成的"裸结节",周围无明显淋巴细胞浸润。诊断:系统性结节病。予以起始剂量30 mg/d醋酸泼尼松治疗1个月后,皮肤及肺部病变明显好转,逐渐减量至1年后停药,随访至今未复发。

A 47-year-old male patient presented to the hospital with chronic cough, expectoration for more than ten years, and scattered skin-colored nodules on his neck, upper limbs and torso. Physical examination showed soybean to peanut-sized, skin-colored nodules which resembling steatocystoma scattered on his neck, upper limbs, waist and back, with distinct border, firm texture and slick appearance, without tenderness. Chest CT scans showed bilateral pulmonary interstitial diffuse lesions with bilateral hilar and mediastinal lymphadenopathy. The histopathology of biopsies revealed naked sarcoidal granulomas in middle-deep dermis and lung tissue which were composed of epitheloid histiocytes and surrounded by scanty lymphocytes. Diagnosis： systemic sarcoidosis. The patient was treated with oral prednisone at an initial does of 30 mg/d, both skin and pulmonary lesions subsided significantly within one month. Prednisone therapy was progressively tapered and stopped after one year and no relapse was observed until now.