Abstract

Graft-versus-host-disease (GVHD) is a multisystem disease that occurs when immune cells transplanted from a blood and marrow or solid organ graft recognize tissues from the transplant recipient as foreign. Diagnosis of solid-organ transplantation-associated (SOT) GVHD can be delayed due to its rarity and non-specific nature of skin manifestations. We performed a retrospective clinicopathologic study of all patients with SOT GVHD from our institution over a 20-year time period. Of 7 patients with SOT GVHD, 5 (71.4%) underwent liver transplantation, while 2 (28.6%) underwent bilateral, sequential lung transplantation. Six patients (85.7%) died, of whom 4 (57.1%) died during the follow-up period an average of 176 days post-transplant. The 7 patients presented an average of 62 days post-transplant with skin symptoms and received a diagnosis of GVHD an average of 77 days post-transplant. While diagnosis was made via FISH and chimerism by PCR, it was often delayed due to confusion with a drug reaction. All 7 patients presented initially with a skin rash and all progressed to develop multi-organ involvement. Frequent skin findings included pruritic, coalescing erythematous to violaceous macules and papules with desquamation. Fifteen skin biopsy specimens were available for review from 6 patients. In 5 patients (83.3%), skin biopsy showed vacuolar interface dermatitis. Hair follicle vacuolar interface changes were present in 11 of 14 cases containing a hair follicle (78.6%). Satellite cell necrosis was observed in 10 of 14 cases (71.4%) with epidermis present for review. Our study emphasizes skin involvement as the initial feature of SOT GVHD and the frequent presence of vacuolar interface changes and satellite cell necrosis on skin biopsy. Clinicians should maintain a high index of suspicion for this rare but potentially fatal condition in patients who have undergone solid organ transplantation, as early recognition is essential for prompt initiation of treatment.

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