Hyperacute Graft Versus Host Disease Mimicking Stevens-Johnson Syndrome in a Patient with Allogeneic Hematopoietic Stem Cell Transplantation

Abstract

Grafts-versus-host disease (GvHD) is a severe systemic complication most commonly occurring after allogeneic hematopoietic stem cell transplantation (HSCT). We would like to share our clinical experience with a patient who developed a grade 4 hyperacute GvHD after haploidentical HSCT for acute myelogeneous leukemia (AML). Case PresentationA 18 year-old woman with a history of haploidentical HSCT was consulted for palmoplantar erythematous rash and severe mucositis. Twelve days prior to the consultation, she had allogeneic haploidentical HSCT from her younger sister for AML. Three days after HSCT, the patient developed neutropenic feverand intractable diarrhea; intravenous (IV) meropenem, teicoplanin and metronidazole were initiated. There was no bacterial growth in blood and urine cultures. However, abdominal computed tomography revealed findings compatible with typhlitis. Twelve days after HSCT, she was referred to our clinic due to severe mucocutaneous eruption.Dermatological examination showed diffuse hemorrhagic-crusted plaques on her lips and neck and dusky-edematous plaques involving volar areas (Figure 1). Our initial diagnoses were Stevens Johnson syndrome (SJS), hyperacute grade 4 GvHD and paraneoplastic pemphigus. A skin biopsy was taken from the neck which showed mild lymphocytic