Abstract

A 77-year-old male with myelodysplastic syndrome was referred to ENT with a several-month history of worsening left ear pain. A previously performed biopsy showed reactive changes. Physical examination revealed an auricular mass with significant soft tissue necrosis down to cartilage. A repeat biopsy was performed given high clinical suspicion for malignancy but also showed reactive changes with no evidence of malignancy. He was referred to rheumatology who raised concern for relapsing polychondritis and possibly VEXUS, an adult-onset inflammatory syndrome associated with MDS due to mutations in the UBA1 gene. The patient was prescribed a taper of oral steroids and referred to oncology for a bone marrow biopsy, however, was negative for UBA1 variants. At follow-up, the patient reported improved symptoms on high-dose oral steroids but worsening at low doses. While physical examination revealed progression, an autoimmune process was still favored given the reported initial improvement with therapy. The patient was referred to dermatology for additional diagnostic considerations. Additional clinical history, including exposure to farm animals and grains, prompted a review of the most recent biopsy. While there were non-specific degenerative changes to the cartilage, rare fungal hyphae were also found in the dermis. Subsequent cultures grew chaetomium, a dematiaceous mold found in damp environments that may lead to cutaneous and sometimes serious systemic infections in immunocompromised patients. The patient was referred to infectious disease and imaging was performed to evaluate for systemic disease. Brain MRI was negative. Chest CT revealed an ill-defined left upper lung lobe nodule, not amenable to CT-guided biopsy. The patient was started on oral voriconazole with visible improvement within two weeks of therapy. This case highlights the importance early evaluation for infectious causes of non-healing skin lesions in immunocompromised patients.

Financial Disclosure:
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