Giant Ulcer with Necrosis on Right Vulva, Groin, and Thigh-- A Case of Necrotizing Fasciitis Associated with Erythematous Pemphigus

Miao Wan,1,* Xiangrong Xu,2,* Xiaojiao Zhao,1 Xia You,1 Guiying Zhang,3 Hai Long,3 Ping He,1 Jian Long,1 Jianjian Zhu1 1Department of Dermatovenerology of the First People’s Hospital of Changde City, Changde Hospital Affiliated to Xiangya School of Medicine of Central South University, Changde, 415000, People’s Republic of China; 2Department of Plastic Surgery of the First People’s Hospital of Changde City, Changde Hospital Affiliated to Xiangya School of Medicine of Central South University, Changde, 415000, People’s Republic of China; 3Department of Dermatovenerology of the Second Xiangya Hospital of Central South University, Changsha, 410000, People’s Republic of China*These authors contributed equally to this workCorrespondence: Jianjian Zhu, No. 388, Renmin East Road, Changde, Hunan Province, People’s Republic of China, Tel +15873658886, Email lidocute@163.comAbstract: Necrotizing fasciitis is a rare, severe, rapidly progressing disease with a high mortality rate. We report a case of a 72-year female with erythematous pemphigus who developed erythema, swelling and ulceration on right vulva, groin, and thigh. The early clinical manifestations of the patient were nonspecific and easily misdiagnosed as cellulitis. However, upon the occurrence of ulceration and necrosis, deep fungal infection, pyoderma gangrenosum or lymphoproliferative disorders were considered. The pathology suggested IgG4-related diseases, plasmacytoma et al. But at last, surgical exploration and postoperative pathology confirmed the diagnosis of necrotizing fasciitis. The patient recovered after multiple aggressive surgical debridement procedures and antibiotic therapy and the patient has been followed up for 2 years without recurrence. Clinicians should be vigilant about the possibility of necrotizing fasciitis in patients with erythema, pain, rapid ulceration of skin and soft tissue, particularly in immunocompromised individuals with long-term use of immunosuppressive agents. It is crucial for saving life by early multi-disciplinary consultation, prompt diagnosis, and aggressive treatment.Keywords: debridement, infection, multidisciplinary consultation, necrotizing fasciitis, pemphigus