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Resolution of Severe Pyoderma Gangrenosum in a Patient with Streaking Leukocyte Factor Disease after Treatment with Tacrolimus (FK 506)
- Kareem Abu-Elmagd, MD;
- David H. Van Thiel, MD;
- Brian V. Jegasothy, MD;
- Jerry C. Jacobs, MD;
- Patricia Carroll, MD;
- Horacio Rodriquez-Rilo, MD;
- Cheryl D. Ackerman, MD;
- John J. Fung, MD, PhD; and
- Thomas E. Starzl, MD, PhD
- From the Pittsburgh Transplant Institute and the University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania; Columbia University College of Physicians and Surgeons, New York, New York. Requests for Reprints: Kareem Abu-Elmagd, MD, Pittsburgh Transplant Institute, 3601 Fifth Avenue, Falk Clinic 5C, Pittsburgh, PA 15213. Acknowledgments: The authors thank Drs. Ramadan Sha'afi and Kozo Yasui for their help and technical assistance in measuring the serum factor. They also thank Nancy Johnson for patient care and Sharon Wesolowski for preparing the manuscript. Grant Support: In part by research grants from the Veterans Affairs and grant DK29961 from the National Institutes of Health, Bethesda, Maryland
Severe, lifelong, unresolving pyoderma gangrenosum occurs in association with recurrent episodes of sterile pyoarthrosis and the presence of a serum factor (called streaking leukocyte factor) [1] responsible for enhancing random migration of purified human neutrophils and mononuclear leukocytes in vitro. Pyoderma gangrenosum is only one feature of this unusual disease. Minor trauma of any sort leads to an excessive accumulation of both mononuclear and polymorphonuclear leukocytes in tissue. This causes subcutaneous induration, sterile abscesses, sterile pyoarthrosis, and extensive areas of skin necrosis similar to those occurring in classical pyoderma gangrenosum, except that the lesions are larger and more confluent. The arthritic lesions are characterized by synovial fluid leukocyte counts greater than 100 000/mm3 and a severe synovitis [1]. We describe a patient with the streaking leukocyte factor syndrome who has been treated successfully with tacrolimus (FK 506, Prograf, Fujisawa Pharmaceutical Co., Osaka, Japan).
Case Report
The patient was a 31-year-old white man who has had a lifelong history of recurrent and chronic sterile abscesses involving the soft tissues, skin, and joints, which began in infancy and was shown to be associated with streaking leukocyte factor in his serum [1]. Because of the hypertrophic and erosive nature of the arthritic process, the patient had surgical procedures on multiple large joints. His history is summarized in Table 1. The patient was referred to the autoimmune clinic at the University of Pittsburgh Medical Center in April 1991 with a well-established clinical, histopathologic, and serologic diagnosis of this syndrome. Before his referral, he had been treated at Columbia-Presbyterian Medical Center (New York, New York) and the National Institutes of Health with plasmapheresis, high doses of steroids, a wide range of immunosuppressive cytotoxic agents, and thalidomide, but none of these treatments yielded a response.
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Laboratory results included normal counts and types of leukocytes, …
