Fasciitis in chronic graft-versus-host disease. A clinicopathologic study of 14 cases

Abstract

Objective: To describe the clinicopathologic features of fasciitis in patients with chronic graft-versus-host disease (GVHD) after allogeneic bone marrow transplantation from human leukocyte antigen (HLA)-identical donors.

Design: A retrospective cohort study.

Setting: Tertiary care center.

Patients: Patients who had allogeneic bone marrow transplantation and developed chronic GVHD with clinical and pathologic signs of fasciitis.

Main outcome measure: Analysis of clinical presentations and of deep cutaneo-muscular biopsy specimens.

Results: Between January 1974 and January 1991, 14 of 475 patients who had allogeneic bone marrow transplantation developed chronic GVHD that began with the sicca syndrome and liver or digestive tract involvement, or both, 60 to 170 days after the graft was received. Sudden and painful skin swelling was reported 350 to 3745 days after the graft was received. Follow-up over 2 to 7 years showed failure of the fasciitis to respond to steroid therapy or to any conventional treatment of chronic GVHD. Although 7 patients showed moderate improvement, the others remained functionally disabled because of skin tightness, joint stiffness, contractures, and sores. Patients with fasciitis in chronic GVHD had no specific immunogenetic profile and no history of L-tryptophan intake or phytonadione injections.

Conclusion: Among alloimmune syndromes, fasciitis is a distinct entity that leads to functional disability. This rare form of chronic GVHD may provide clues to understanding the mechanisms involved in fasciitis from other causes.