Abstract
A 15 year old boy developed skin changes compatible with chronic graft versus host reaction (CGVHR) following bone marrow transplantation. His skin was thin, dry, inelastic, and telan-giectatic. Biopsy revealed basal vacuolization, loss of rete pegs, atrophy of sweat glands, perivascular lymphocyte cuffing, and dense dermal collagenization. Because of the dermatologic similarity to PSS, we compared this patient to 38 PSS patients for evidence of systemic involvement. He had significant restrictive lung disease (VC and TLC 50% of predicted), cardiac conduction abnormalities (first degree heart block, right bundle branch block), and a pericardial effusion. Each of these abnormalities was found in 30-40% of the PSS group. Arteriolar intimal proliferation was seen in the heart and kidneys of our patient at autopsy. This finding has been noted in approximately 25% of PSS patients. Our patient had Raynaud's phenomenon as did 95% of the PSS group. He had a striking polyclonal elevation in serum IgG (4 gms/dl), an uncommon finding in PSS. 84% of the PSS patients showed esophageal dysmotility, but our patient did not have this abnormality. The clinical and pathologic similarities between this patient and PSS patients suggest that CGVHR and PSS may have a common pathogenesis. The use of animal models of graft versus host reaction may lead to an understanding of mechanisms responsible for both disease processes.
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