Granulomatous reaction is an inflammatory reaction, which is defined by the presence of granulomas in the tissue. A granuloma is characterized by the accumulation of epithelioid cells together with a variety of multinucleated giant cells. The causes of granulomas are very diverse and many infectious and non-infectious disorders could cause granuloma formation [1]. Fungal infections are mostly associated with suppurative reactions however, they may be seen with granulomatous response in the tissue. Although fungal infections are relatively common in humans, the formation of a granulomatous mass is a less frequent event. On the other hand, among the fungi, Aspergillus is the most common cause of granulomatous reaction [2]. Mucormycosis is a disease caused by an invasive fungus from order Mucorales and it is primarily transmitted through inhalation of spores, orally or inoculation in a trauma [3]. It usually occurs in patients who have compromised immune system such as uncontrolled or poorly controlled diabetic patients or malignancies. Neutrophils play a pivotal role in protecting the body against this infection; therefore neutropenic patients are at risk of Mucor mycosis [4]. It has been shown that malignancy is the most prevalent associated comorbidity in the developed societies whereas, diabetes is the most important comorbidity in the developing societies in Mucor cases [5,6]. The classification of Mucormycosis is based on its anatomical location and includes rhino cerebral, pulmonary, cutaneous, gastrointestinal, disseminated, or uncommon presentations [7].  In fact, the clinical manifestation is mainly relevant to the underlying disease, as the diabetic patients are more likely to have the rhino-orbital-cerebral Mucormycosis, and the patients with hematological malignancies and soft tissue trauma are more likely to suffer from the sino-pulmonary form and soft tissue Mucormycosis respectively [8]. One of the hallmarks for diagnosis of Mucormycosis in any anatomical region is the invasion into the vessels and the subsequent tissue infarction [9]. Although the prognosis of Mucor mycosis is generally poor, here we report a case of Mucor mycosis that was treated successfully.

Case Report

A 64-year-old man presented to a local clinic with a history of right cheek swelling and numbness for one month (day 1). His medication included Aspirin, Clopidogrel, Metoprolol and Losartan for cardiovascular disease, Insulin and Repaglinide for diabetes and Sulfasalazine for inflammatory bowel disease. After 15 days his condition deteriorated, and he developed fever and chills. He was admitted to a rural hospital because of these symptoms in addition to high uncontrolled blood sugar and fever. After the primary work-up, controlling his blood sugar and empirical antibiotic administration, he was discharged without diagnosing the cause of his cheek swelling. Although the fever was subsided, the patient still suffered from swelling of his right cheek and after 102 days was transferred to the central hospital in Yazd city, Iran for more investigations (day 117).

He had been well otherwise and denied any history of hemoptysis, weight loss, fever, pain in his face, nasal congestion, epistaxis or postnasal discharge. In the general physical examination, the patient was an alert and conscious male. There was no evidence of cyanosis, clubbing and peripheral lymphadenopathies. Nasal examination revealed a normal colored mucosa without evidence of necrosis, hemorrhage and septal deviation. Mucosa of the mouth and pharynx were normal. Eyes movements in all directions were unremarkable. Ears examination was normal. The laboratory results were normal except for an Erythrocyte Sedimentation Rate (ESR) of 90 mm/hr and a hyperglycemic profile. A paranasal CT scan revealed mucosal thickening in the right maxillary sinus with destruction of the anterior wall of right maxillary sinus and alveolar ridge that was in favor of neoplastic lesion. In addition, mucosal thickening in the left maxillary sinus, ethmoidal air cells and left sphenoidal sinus could be observed. No abnormality in bilateral orbits was detected. A chest X-ray showed no abnormalities. The patient underwent Caldwell-luc surgical resection of the right maxillary sinus and the specimen was sent to the pathology ward. Histological examination of the H&E stained slides demonstrated that the sinus mucosa was infiltrated by a necrotizing granulomatous inflammation. The granulomas were composed of epithelioid cells and there were scattered multinucleated giant cells. Among some of the granulomas, there were a few ribbons like and broad non-septate hyphae that showed positive reaction in PAS stain (Figures 1-3) which helped establishing the diagnosis of granulomatous Mucor mycosis. Finally, after the treatment with Liposomal Amphotericin B (150 mg/day) and Caspofungin (70 mg/day) for six weeks, the patient was recovered. The creatinine level was monitored and ranged between 0.9-1.2 mg/dL during the treatment period.