Acute graft-versus-host disease presenting as Stevens–Johnson syndrome and toxic epidermal necrolysis: A retrospective cohort study

• Published in: Journal of the American Academy of Dermatology 2023-04, Vol.88 (4), p.792-801
• Main Authors: Hung, Yi-Teng, Chen, Yen-Wen, Huang, Yenlin, Lin, Yu-Jr, Chen, Chun-Bing, Chung, Wen-Hung
• Format: Article
• Language: English
• Subjects: acute graft-versus-host disease; complication; Diagnosis, Differential; Graft vs Host Disease - complications; Graft vs Host Disease - diagnosis; Humans; morbidity; mortality; predictor; prognosis; Retrospective Studies; Sepsis; Stevens-Johnson Syndrome - etiology; Stevens–Johnson syndrome; survival; toxic epidermal necrolysis
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2022.10.035

Cutaneous manifestations resembling Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) have been reported in patients with acute graft-versus-host disease (aGVHD); however, the clinicopathological characteristics of SJS/TEN-like aGVHD remain unexplored. To investigate the clinicopathology, complications, and outcomes of patients with SJS/TEN-like aGVHD. We analyzed a multicenter cohort of patients with aGVHD between 2000 and 2021. We analyzed 31 patients with aGVHD, including SJS/TEN-like (n = 15) and non-SJS/TEN-like (n = 16). Patients with SJS/TEN-like aGVHD had significantly more extensive erythema and skin detachment/mucositis. SJS/TEN-like aGVHD was significantly associated with higher aGVHD grading and systemic complications, including pancytopenia, leukopenia, anemia, severe thrombocytopenia, coagulation abnormality, hepatitis, diarrhea, renal dysfunction, and bacteremia. A significantly lower hemoglobin/red cell distribution width ratio was identified in SJS/TEN-like aGVHD. Histopathology showed significant severe dyskeratosis and interface change. Patients with SJS/TEN-like aGVHD had lower 2-month survival rates and 5.35-fold higher 5-year mortality rates than those with non-SJS/TEN-like aGVHD. Total mortality rates of patients with SJS/TEN-like aGVHD reached 80% during follow-up; sepsis predominated the causes of death. Retrospective, nonrandomized study with a small sample size. SJS/TEN-like aGVHD is associated with multiple systemic complications and high mortality. Early recognition, differential diagnosis from drug-induced-SJS/TEN, and appropriate treatment are critical.