Acute Skin Graft Versus Host Disease Post Liver Transplantation: A Case Report

Graft versus host disease (GVHD) is a rare complication after liver transplantation, occurring in 1-2% of patients. However, it is associated with a high mortality rate of 85-90%. It presents with skin rash and diarrhea, with or without pancytopenia. We report an unusual case of extensive skin only GVHD post liver transplantation, improved with steroids and etanercept.

Case: A 52 year old white male patient underwent deceased donor orthotopic liver transplantation for end stage liver disease secondary to non-alcoholic steatohepatitis. He also had history of psoriasis. He received immunesuppression with thymoglobulin, low dose prednisone, tacrolimus and mycophenolate mofetil and was discharged on postoperative day number 11. He presented on postoperative day number 15 with fever and chills and was suspected to have a wound infection; broad spectrum antibiotics were started. Given the suspicion of sepsis, tacrolimus was decreased and mycophenolate mofetil and prednisone were stopped. On postoperative day 21, he developed a maculopapular erythematous rash over his trunk and extremities, the rash slowly progressed into small bullae that eventually ruptured without sloughing of the skin, he also had ulcerations of the oral mucosa and lips. He did not have diarrhea or pancytopenia. Biopsy of the rash showed lymphocytic interface dermatitis and formation of multiple necrotic keratinocytes with resultant separation at the dermal epidermal junction; findings consistent with GVHD of the skin. When the rash started, the patient was concomitantly on Sulfamethoxazole/Trimethoprim for pneumocystis jirovecii prophylaxis and was on broad spectrum antibiotics for suspected sepsis, the rash however continued to progress despite stopping these antibiotics two days after the rash appeared. A repeat biopsy was performed to confirm that GVHD is more likely than a drug reaction and this again showed features of GVHD. Short tandem repeat testing to evaluate for chimerism failed to show that in the blood but showed 11% donor cells in the skin biopsy. A diagnosis of GVHD was made, IV steroids were started —five days after the rash developed —with a methylprednisolone bolus followed by 1mg/kg, this achieved mild improvement, etanercept was added after four days of steroids (25mg subcutaneously twice weekly for 8 doses) with further improvement.

Conclusions: GVHD post solid organ transplant as a diagnosis is often missed, because the presenting signs and symptoms are nonspecific and the condition is rare. Prognosis is in general poor and complications of pancytopenia usually lead to the high mortality associated with this disease. There are no guidelines as to how to treat this condition but careful intensification (or withdrawal) of immunesuppression can potentially lead to successful control of this disease process. We treated this patient with parenteral steroids and added etanercept with a favorable outcome.