Sclerodermatous graft-vs-host disease: clinical and pathological study of 17 patients.

OBJECTIVE: To collect and review all cases of sclerodermatous chronic graft-vs-host disease from January 1, 1982, through December 31, 2000.
SETTING: University hospital in Madrid, Spain. PATIENTS: During the study period, 493 allogenic bone marrow transplantations were performed. Sclerotic lesions developed in 17 patients.
RESULTS: Sclerotic lesions appeared after a mean of 529 days. Previously, 10 (59%) of 17 patients showed a leopard-skin eruption. Sclerosis was generalized in 12 patients and localized in 5. Nine patients presented with rippling of the skin and 8 with lichen sclerosus lesions. We found no anti-Scl-70 or anti-centromere antibodies. Results of histological analysis showed pandermal or deep-dermal sclerosis, slight vacuolar degeneration of the basal cell layer, and follicular damage with follicular plugs. In 6 (50%) of the 12 patients with evaluable biopsy specimens, septal panniculitis was found. Squamous syringometaplasia and mucin deposits were also detected. Treatment with high doses of prednisone and azathioprine helped in 8 of 9 patients. In 12 patients, sclerosis disappeared after 487 days.
CONCLUSIONS: Leopard-skin eruption, follicular involvement, ripply skin, and lichen sclerosus lesions have been described poorly or not at all in sclerodermatous graft-vs-host disease. The presence of lichen sclerosus, morphea, septal fibrosis, and fasciitis suggests that the sclerosis can start at and affect any level of the skin. Treatment with prednisone and azathioprine seems to halt the process. Most patients have a good prognosis with treatment. Although most lesions disappear, small areas of fibrosis may remain that do not produce any physical or functional impairment.