A 25 year old african american male with a history of asthma, hypertension and CD4 T lymphocyte deficiency presented to an outside facility with complaints of shortness of breath and musculoskeletal chest pain. He initially was found to have a pneumonia due to Pseudomonas aeruginosa and received a complete a course of meropenem. His dyspnea did not resolve, for which he returned to the outside facility. He underwent a VATS (video assisted thoracoscopic surgery) lung biopsy to get a definitive diagnosis. His lung biopsy showed a necrotizing granuloma and tissue cultures isolated yeast. He was treated with 2 weeks of fluconazole and was started on a steroid taper for presumed sarcoidosis. Unfortunately, he returned again to the outside facility shortly thereafter with worsening dyspnea, productive yellow cough, vision changes, night sweats, 30 pound weight loss, and fevers as high as 102 F. He developed presumptive candidemia (positive blood cultures for yeast with identification pending). The patient was started on vancomycin, aztreonam, levofloxacin, and micafungin prior to being transferred to our tertiary care hospital.
Upon admission to our hospital, further history revealed that he was employed as a customs official at an international airport and had a recent trip to the Caribbean for a cruise. He had a skin biopsy due to a papular rash on his face and chest (Fig. 1), and a high resolution CT chest showed cavitary consolidations in the right upper and lower lobe with diffuse bilateral lymphadenopathy (Fig. 2). Infectious and autoimmune workup was ordered (HIV testing by protocol was negative), and he also underwent bronchoscopy and bone marrow biopsy. Immunodeficiency differential included idiopathic CD4 lymphoma, common variable immune deficiency (CVID), systemic lupus erythematosus (SLE), HIV, and other vasculitides. His antibacterial therapy was discontinued, his steroid dose was decreased, and he was continued on micafungin. His clinical course worsened and he became tachycardic, tachypneic, and his lactic acid was increasing despite intravenous fluids, requiring transfer to the intensive care unit. Transesophageal echocardiogram (TEE) showed multiple 1 mm densities on the mitral valve. Fundoscopic examination showed 3 focal white lesions on left optic disc. Intravitreal tap was deferred and his micafungin was switched to amphotericin B.
While awaiting speciation of yeast in blood cultures, results of skin biopsy (Fig. 3), bone marrow biopsy (Fig. 4), and bronchoalveolar lavage on bronchoscopy (all preliminary results showing fungal elements), he was persistently febrile to over 102 F, for which voriconazole was added to amphotericin B. Tuberculosis, strongyloidiasis, cryptococcosis, histoplasmosis, blastomycosis, coccidiomycosis, and cytomegalovirus infection were negative. He had low IgG (456 mg/dL) and IgM (30 mg/dL), and elevated IgA (705 mg/dL). He developed diarrhea due to Clostridium difficile (C. difficile PCR positive), and was started on oral vancomycin and metronidazole. Given his progressive worsening clinical status as well as suspicion for a combined immunodeficiency with his T-cell lymphocytopenia and immunoglobulin deficiency, he was given a dose of intravenous immunoglobulin (IVIG), after which he developed respiratory decompensation requiring intubation. He developed worsening acidemia (pH 6.9), hypotension requiring multiple pressors, severe hypoglycemia despite dextrose IV infusion, worsening lactic acidosis ( > 20 mmol/L), and severe hypoxemia despite maximal ventilator support. His family opted to pursue comfort care and the patient passed away peacefully. The fungus/yeast was later identified as Sporothrix schenckii complex.