We reviewed the clinical characteristics after the appearance of sclerodermatous cGVHD.
The development of sclerodermatous GVHD was observed in 22 (5.2%) out of 423 patients.
Immunosuppressive therapy was interrupted in 16 (72%) patients before sclerodermatous lesions had developed.
Partial response was achieved in five patients with extensive sclerodermatous GVHD.
Chronic cutaneous GVHD is categorized according to the type of lesions into lichenoid and sclerodermatous variants.
(3) found that lichenoid GVHD always preceded the sclerodermatous phase.
Sclerodermatous lesions begin with indurated plaques with loss of skin markings and appendages causing pain and chronic ulceration, predisposing to generalized wasting and pyogenic infections of the skin.
The presence of both lichen sclerosus-like lesions and the histological findings of septal panniculitis in the disease process suggest that the sclerosis in sclerodermatous GVHD can start and affect any level of the skin and can extend to involve the complete dermis, the subcutis, and even the fascia.
Sclerodermatous cGVHD can give rise to reduced range of motion and secondary effects including loss of strength, endurance and functional capabilities.
Spontaneous resolution of sclerodermatous GVHD may occur.
Numerous treatments, including prednisone, azathioprine, penicillamine, CsA, methotrexate, MMF, thalidomide, clofazimine, anti-CD20 monoclonal antibody, ECP, phototherapy with bath PUVA, UVA1 or UVB, etretinate or various combinations, have been tried with varying success in sclerodermatous GVHD (2), (3), (5), (15-22).
In conclusion, sclerodermatous GVHD has a late onset and clinically may be quite disabling.