Liu Jing-Chao, Liu Xi-Gao, Xu Chao, Zhao Hai-Feng, Jiang Xian-Zhou
Department of Urology, Qilu Hospital of Shandong University, Jinan, China.
Medicine (Baltimore). 2019 Apr;98(16):e15263. doi: 10.1097/MD.0000000000015263.
Follicular occlusion triad (FOT) is an autosomal recessive inherited disease and no more than 3 variants of the triad have been reported. We give a report in which scrotal elephantiasis is a variant of FOT and further perform a literature review.
A 41-year-old man came to us because of a large scrotal cyst and generalized skin lesions that had occurred over the past 10 years. The generalized skin lesions consisted of hidradenitis suppurativa on the perineum and back, acne conglobata in the armpit, and dissecting cellulitis of the scalp. He took antibiotics for a long time but achieved poor effect. Furthermore, he told his father and elder brother also manifested such skin lesions.
Magnetic resonance showed a mass in the left scrotum with clear boundaries. A routine blood test showed a high leukocyte level of 12 × 10/L and a hemoglobin content of 78 g/L. C-reactive-protein increased. Series of autoimmune antibody tests were negative. The postoperative pathologic findings showed that the mass was an epidermoid cyst, and hematoxylin and eosin staining showed hyperkeratosis of the skin as well as inflammatory and edematous changes. A diagnosis of a variant of FOT was made.
We removed skin abscesses and lesioned the inner part with hydrogen peroxide. Then we performed an excision of the scrotal lesion.
The patient recovered well and had no evidence of recurrence at a 16-month follow-up.
We reported a case in which scrotal elephantiasis was a variant of FOT and surgical intervention played an important role in secondary urologic diseases.
毛囊闭锁三联征（FOT）是一种常染色体隐性遗传病，报道的三联征变异不超过3种。我们报告1例阴囊象皮肿为FOT变异的病例，并进行文献复习。
一名41岁男性因过去10年出现的巨大阴囊囊肿和全身性皮肤病变前来就诊。全身性皮肤病变包括会阴部和背部的化脓性汗腺炎、腋窝的聚合性痤疮以及头皮的穿掘性蜂窝织炎。他长期服用抗生素但效果不佳。此外，他表示其父亲和哥哥也有类似皮肤病变。
磁共振成像显示左侧阴囊有一界限清晰的肿块。血常规显示白细胞水平升高至12×10/L，血红蛋白含量为78g/L。C反应蛋白升高。一系列自身免疫抗体检测均为阴性。术后病理结果显示肿块为表皮样囊肿，苏木精-伊红染色显示皮肤角化过度以及炎症和水肿改变。诊断为FOT变异型。
我们切除了皮肤脓肿并用过氧化氢处理内部病变。然后对阴囊病变进行了切除。
患者恢复良好，16个月随访时无复发迹象。
我们报告了1例阴囊象皮肿为FOT变异的病例，手术干预在继发性泌尿系统疾病中起重要作用。
