BUDAPEST, HUNGARY — The death of a patient with lupus from disseminated cryptococcosis illustrates the difficulty in finding the balance between therapeutic immunosuppression and treatment of a resulting infectious disease, Dr. Gabor Szabad said at an international symposium sponsored by the European Academy of Dermatology and Venereology.
The patient was a 36-year-old woman who had been diagnosed with systemic lupus erythematosus in 1989 when she began experiencing muscle weakness, fever, and arthropathy and also developed the characteristic facial butterfly rash. She was antinuclear antibody positive, and double-stranded DNA antibodies were present.
During the subsequent decade, she developed lupus nephritis, cerebral lupus, lower extremity ulceration, and Raynaud's phenomenon-associated gangrene that necessitated the amputation of two fingers, Dr. Szabad said.
Throughout these years, she was receiving continuous immunosuppressive therapy with prednisolone, cyclophosphamide, and azathioprine in varying doses.
In 2002, she presented with reddish-brown infiltrates across the gluteal area and yellowish nodules and papules on the left hand. She had an elevated sedimentation rate, marked lymphopenia, and mild renal insufficiency; a thoracic CT scan showed evidence of pneumonia, said Dr.Szabad of the department of dermatology and allergology, University of Szeged (Hungary).
Histopathologic investigation of the skin lesions revealed the presence of yeastlike organisms in the dermis. Buff-colored mucoid colonies characteristic of the genus Cryptococcus grew on culture and were positive on a cryptococcal antigen latex agglutination test.
Subsequent efforts to manipulate antimycotic, antibiotic, and immunosuppressive therapies resulted in a seesaw course. She died of acute respiratory distress syndrome on day 51.
Autopsy findings included mesangial glomerulonephritis, pulmonary cryptococcosis, and cryptococcal epidermolysis, Dr. Szabad said.
Most cases of disseminated cryptococcosis occur in patients who are immunocompromised because they have AIDS or received an organ transplant.
“Lupus patients also are susceptible to opportunistic infections such as this because of their prolonged immunosuppressive therapy, but the intrinsic immunologic abnormalities of systemic lupus erythematosus can also contribute to the susceptibility,” he said.
Most cases of serious cryptococcal infection in patients with lupus manifest as meningeal disease. The first reported case of cutaneous cryptococcosis in a patient with underlying lupus involved a 24-year-old woman who presented with cellulitis of the thigh and gluteus and who went on to develop nephrotic syndrome, arterial hypertension, and ultimately fatal acute renal insufficiency (Mycoses 2001;44:419–21).