多变根毛霉致原发性皮肤毛霉病1例及文献回顾

Primary cutaneous zygomycosis caused by Rhizomucor variabilis:a case report and literature review

报告1例多变根毛霉引发原发性皮肤毛霉病,并对国内报道的10例类似病例进行文献回顾。患者男,26岁。右上肢皮肤结节硬块18年,颈部肿块3年。患者曾接受伊曲康唑、氟康唑治疗,疗效不佳。X线片示右手指关节融合畸形,右上肢神经肌肉电生理检查提示神经源性损伤。皮损组织病理可见真皮内较多量多核巨细胞及少量浆细胞、嗜酸性粒细胞,多核巨细胞内见可疑菌丝,PAS及银染均可见阳性染色的菌丝。经真菌培养及分子生物学鉴定为多变根毛霉。经总用量为2 326 mg两性霉素B治疗后,患者皮损明显消退,随访2年无复发。文献回顾发现,多变根毛霉所致原发性皮肤毛霉病病程较长,致死率低,及时应用两性霉素B治疗一般都能取得较好疗效,且药物不良反应经过对症处理多数可以耐受。从该例患者来看,多变根毛霉感染亦可能造成关节神经的不可逆损伤,即使皮损最终得以清除仍因患肢畸残影响患者生活质量。因此及时足量地给予两性霉素B是多变根毛霉所致原发性皮肤毛霉病治疗的关键。

To report a case of primary cutaneous mucormycosis caused by Rhizomucor variabilis and review 10 similar cases from domestic literature. The patient was male and 26 years old, He had nodules and swelling on right upper extremity for 18 years, with neck involvement for 3 years. The irregular treatment of itraconazole and fluconazole was received, which leads no significant effect. X-ray film of the right hand showed abnormal fusion of knuckle. Neuromuscular electrical physical examinations of the right upper limb showed neurogenic injury. Histopathology of the cutaneous lesion showed lots of inflammatory cells and multinucleated giant cells infiltrating in the mid-dermis and sub-dermis. The silver and PAS staining revealed the presence of some gross and septate hyphae.The clinical isolate was identified as Rhizomucor variabilis by culture and molecular biology examination. The final diagnosis was as primary cutaneous mucormycosis. After receiving a total amount of 2326 mg amphotericin B treatment, the patient acquired significantly lesion subsided. No signs of recurrence were observed in two years follow-up. Literature review found that primary cutaneous mucormycosis caused by Rhizomucor variabilis shared longer course and lower death rate. Timely application of amphotericin B treatment can often achieve rather good efficacy, and the majority of side effects can be tolerated with symptomatic treatment. According to the patients we reported, Rhizomucor variabilis infection may result in irreversible damage of nerve and joints. Skin lesions could be eventually cleared. However, the limb deformities still affected the life quality of patients. Therefore, timely and decisive full dosage of amphotericin B therapy is the key to the treatment of primary cutaneous mucormycosis caused by Rhizomucor variabilis.