Diagnostic and therapeutic dilemma in Stevens–Johnson syndrome-like acute graft-versus-host disease after liver transplantation: A case report

Abstract

BackgroundAcute graft-versus-host disease (aGVHD) is a severe and fatal complication after orthotopic liver transplantation (OLT). Clinical manifestations of severe aGVHD can resemble drug-induced Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), and there are also various medications, such as antibiotics and immunosuppressants, used after transplantation, causing a diagnostic dilemma. Furthermore, there have been no standardized diagnostic and therapeutic strategies for OLT-aGVHD due to its rarity.Case summaryA 52-year-old man presented with generalized maculopapular eruptions, fever, and pancytopenia 1 month after OLT and 4 days after taking sulfamethoxazole/trimethoprim. After assessment of the scoring criteria for drug causality of drug allergy, histopathological findings of skin biopsy, lymphocyte activation test of the potential offending drug, and microchimerism study, the diagnosis was in favor of aGVHD mimicking SJS/TEN. Considering severe sepsis, the anti-tumor necrosis factor alpha (TNF-α) agent, etanercept, was used to replace tacrolimus and corticosteroid. Skin lesions resolved gradually after anti-TNF-α biologics rescue; tacrolimus and corticosteroid therapy were re-administrated after controlling sepsis. Pancytopenia recovered and the patient was discharged in a stable condition.ConclusionWe demonstrated a diagnostic strategy for OLT-aGVHD. Targeting therapy with anti-TNF-α blockade and a temporary withdrawal of traditional immunosuppressants may be among effective and safe therapeutic options of OLT-aGVHD for those with severe sepsis.

Cutaneous and renal aspergillosis resulting from orthotopic liver transplantation

“…Initial evaluation of cutaneous lesions in solid organ transplant patients should investigate a wide variety of aetiologies. Non-infectious causes include drug-induced rashes such as drug reaction with eosinophilia and systemic symptoms11 and Stevens-Johnson syndrome 12. However, these differential diagnoses are unlikely in this patient considering she was afebrile on presentation and her rash was neither painful, pruritic nor rapidly progressing.…”

Stevens–Johnson syndrome/toxic epidermal necrolysis in an orthotopic liver transplant recipient: a case report

“…Further, the risk of acute GVHD after OLT increases with increasing recipient age (>65 years) [ 9 ], which made this diagnosis less likely in our patient. Though both diseases are partially mediated by cytotoxic T cells against epithelial tissue, recent immunohistologic studies suggest that TEN/SJS may have a greater relative depletion of CD4+ T lymphocytes compared to acute GVHS [ 10 ].…”