A 35-year-old Chinese male with diabetes mellitus presented to Peking University Third Hospital (Beijing, China) on November 14, 2011, with progressive swelling, ulceration, and destruction of the face. The lesions had begun 2 months earlier as infiltrated rhino facial and maxillofacial erythema, with nasal obstruction, discharge, rhinorrhea, and epistaxis, which rapidly increased in size and ulcerated. The patient was initially diagnosed, in the local hospital, with periodontitis and the treatment with tooth extraction was ineffective. Next, the lesion was diagnosed with skin and subcutaneous infection in the rhino face and treatment with antibiotics was administered, but the necrotic ulcer progressively enlarged. Subsequent clinical diagnosis was made of rhino facial mycosis and the patient was treated with itraconazole, but the ulcer still progressed with the disease eroding through his hard palate, sinuses, nose, and face, resulting in destructive changes in the maxilla and mandible, with systemic manifestations of fever, weight loss, and hepatosplenomegaly. As the ulceration progressed, he was suspected as having LMG and was therefore transferred 2 weeks after to the university clinic for further diagnosis and treatment.
On examination, his left half central face was swollen, ulcerated, and destructed, and the nose, lower eyelid, chin, and upper lip were all necrotic. Black eschar covered the left side of the face with an underlying grayish pus (Figure 1A). The anterior wall of the left maxilla was perforated with a green-yellowish pus seen at the bottom of the maxilla (Figure 1B). The hard palate had patches of black, pale discoloration showed perforation (Figure 1C), and soon proceeded with complete destruction (Figure 1D). Pale gray, wool-like mycelium was observed on the necrotic tissue of the nose (Figure 1E). The patient had a normal temperature, pulse, respiration, and blood pressure. His body weight was 42 kg (20 kg less than before onset). Blood tests showed levels of hemoglobin: 90 g/L, white cells: 7.5 x 109 /L with 81.8% band cells, platelets: 433 x 109 /L, blood glucose level: 17.7 mmol/L, total iron binding capacity: 40.2 mol/L, transferrin: 147 mg/dL, prothrombin time: 14.2 s, prothrombin activity: 61.0 (80-150), alanine aminotransferase: 43 U/L, alkaline phosphatase: 394 U/L, and r-GT: 354 U/L. The tests for HIV Ab, anti-TB, EBV IgM, and syphilis were all negative. X-ray computed tomography (CT) showed left facial soft tissue deficiency, absence of left anterior bone wall of the maxillary sinus, mucosal thickening with increased density of bilateral maxillary sinus, ethmoid, sphenoid sinus, bilateral hydrothorax, and high-density fuzzy shadow in the lower part of the lungs. Meningeal enhancement was seen on MRI.
A skin biopsy was performed on the lesions of the nasal dorsum, nasal mucosa, and granuloma. The biopsied tissue was sent for lymphoma diagnosis to the authored pathologists for microbiological evaluation in our laboratory. Pathology showed inflammation, necrosis, and pleomorphic cell infiltration, with atypical hyperplasia. Angioinvasive, angiocentric, and angiodestructive characteristics were typical with "Onion-skin" lesions (Figure 2A). In immunohistochemistry, the atypical lymphoid cells were positive for NK/T cell markers (CD2+, CD3+, CD8+, and individual CD56+), cytotoxic granule associated proteins (GZMB and TIA1), and hyperplasia (Ki67+):typical of ENKTL (Figure 2). Patient was diagnosed as LMG/T cell non-Hodgkin's lymphoma at the Department of Pathology, Peking University, according to the WHO classification of mature T- and NK-cell neoplasms with negative EBER and EBV testing.