Resident Rounds Part III: Case Report: Fatal Cryptococcal Panniculitis in a Lung Transplant Recipient

May 2015 | Volume 14 | Issue 5 | Features | 519 | Copyright © May 2015

Bobby Y. Reddy MD,a Sheila Shaigany BS,a Lawrence Schulman MD,b and Marc E. Grossman MD FACPa

aDepartment of Der matology, Columbia University Medical Center, New York, NY
bDepartment of Medicine, Columbia University Medical Center, New York, NY

Abstract
Cryptococcal panniculitis is a rare entity previously reported in only 13 solid organ transplant (SOT) recipients. Cutaneous cryptococcosis in SOT recipients warrants extensive systemic workup and treatment as if central nervous system (CNS) disease is present. It should be included in the differential diagnosis of panniculitis in the immunocompromised host, as early diagnosis and treatment are critical. We report a fatal case of cryptococcal panniculitis in a 44-year-old lung transplant recipient.

CASE REPORT

A 44-year-old man with cystic fibrosis, status post bilateral lung transplant 14 years prior, was admitted to the hospital for progressively worsening shortness of breath, headaches, and painful skin nodules on the legs and buttocks of 4 months duration. He had been on a longstanding immunosuppression regimen of tacrolimus, mycophenolate mofetil, and prednisone. Five months prior, his pulmonologist noted decreased pulmonary function tests, and a chest X-ray revealed a new right mid-lung mass. A computed tomography (CT) of the chest was recommended for further evaluation but he did not follow up.
During this time he developed painful skin nodules on his thighs and buttocks. An initial biopsy performed by an outside dermatologist was read as erythema nodosum. His shortness of breath worsened and he developed new headaches, prompting hospital admission. He did not have fever, chills, or night sweats. He denied sick contacts, recent travel, and exposure to pets or birds. He had lived his entire life in the Northeast region of the United States and was employed as a bookkeeper at a library.
On admission, his vital signs were normal. Tender red 1 cm to 5 cm subcutaneous nodules were scattered on both medial thighs and buttocks (Figure 1). A CT scan of the chest revealed a 3.8 cm x 2.0 cm cavitary mass in the superior segment of the right lower lobe. A skin biopsy showed numerous non-encapsulated yeast cells in the subcutaneous fat with a gelatinous-type histologic reaction (Figure 2). A culture of skin biopsy showed mucoid colonies of cryptococcus neoformans on 5% sheep blood agar (Figure 3). The initial “erythema nodosum” skin biopsy, from several weeks prior, was positive for multiple yeasts with surrounding halos using a gomori methenamine silver stain.
A lumbar puncture was performed and cerebrospinal fluid (CSF) was positive for cryptococcal antigen at 1:1024. The India ink stain of the CSF is shown in Figure 4. The patient was started on intravenous amphotericin B (lipid complex) 300 mg Q12 hours along with oral flucytosine 1 g Q12 hours. He was discharged 10 days after admission with a peripherally inserted central catheter to continue treatment, but was readmitted 1 week later with recalcitrant disease. Despite continued aggressive anti-fungal therapy (including the addition of voriconazole 230 mg Q12 hours), he died 8 months later from complications of disseminated disease.

DISCUSSION

Cryptococcus neoformans is an opportunistic fungal infection with significant morbidity and mortality in the immunocompromised host. Solid organ transplant (SOT) recipients with disseminated disease account for 30% to 60% of non-HIV patients infected with cryptococcus, and carry a mortality rate of 33% to 42%.1,2 The central nervous system (CNS) serves as the most frequent site of dissemination from the primary pulmonary disease, followed by the skin and soft tissue (10%-20%). Cutaneous cryptococcosis in SOT recipients almost always results from disseminated disease, and primary cutaneous cryptococcosis (PCC) should be a diagnosis of exclusion.3 Cellulitis is the most common cutaneous mani-