Sjogren's Syndrome Complicated by Naso-Orbito-Cerebral Mucormycosis: A Case Report.

Abstract

Background: Sjogren's syndrome (SS) is a chronic inflammatory autoimmune disease characterized by exocrine gland dysfunction. Mucormycosis is a rare yet life-threatening opportunistic fungal infection caused by Mucor species, with a high mortality rate. In patients undergoing long-term immunosuppressive therapy or corticosteroid use, especially when compounded by conditions such as diabetes or hyperlipidemia, Mucor can become pathogenic. Medical Record Description: We retrospectively analyzed the clinical data of a patient with Sjogren's syndrome complicated by rhinocerebral mucormycosis, focusing on its clinical presentation and progression. The patient was admitted to the hospital 20 days prior with symptoms of a "nasal infection" caused by a furuncle on the nose. The condition progressively worsened, resulting in nasofacial skin ulceration and necrosis, with black eschar formation around the affected area. Upon admission, comprehensive evaluations, including rapid histopathological and fungal tests, confirmed the diagnosis of mucormycosis. Despite surgical intervention under general anesthesia and antifungal therapy, the clinical outcome was ultimately fatal. Conclusion: Mucor infections commonly occur in patients with immunodeficiency and severe underlying diseases. The clinical manifestations are diverse, often presenting with skin tissue necrosis and the formation of black eschar. Fungal culture and histopathological examination remain the gold standards for diagnosing mucormycosis, while imaging studies are crucial for evaluating the extent of fungal dissemination and bone involvement. Given the rapid progression of the disease, early diagnosis and prompt treatment are critical for improving survival rates.