AI Article Synopsis
- A study explored the clinicopathological characteristics of acute graft-versus-host disease (aGVHD) that resembles Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), revealing significant differences in symptoms and outcomes.
- Among 31 patients studied, those with SJS/TEN-like aGVHD showed more severe skin and mucosal reactions, higher overall disease grading, and increased complications like anemia and renal dysfunction.
- The findings indicated a concerning 80% mortality rate for SJS/TEN-like aGVHD during follow-up, emphasizing the need for early diagnosis and distinct treatment from drug-induced SJS/TEN.
Article Abstract
Background: 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.
Objective: To investigate the clinicopathology, complications, and outcomes of patients with SJS/TEN-like aGVHD.
Methods: We analyzed a multicenter cohort of patients with aGVHD between 2000 and 2021.
Results: 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.
Limitations: Retrospective, nonrandomized study with a small sample size.
Conclusion: 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.
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| http://dx.doi.org/10.1016/j.jaad.2022.10.035 | DOI Listing |
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Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taipei, Keelung, Taoyuan, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan; Whole-Genome Research Core Laboratory of Human Diseases, Chang Gung Memorial Hospital, Keelung, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taiwan; Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China; School of Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan; Genomic Medicine Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taiwan.
Article Synopsis
- A study explored the clinicopathological characteristics of acute graft-versus-host disease (aGVHD) that resembles Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), revealing significant differences in symptoms and outcomes.
- Among 31 patients studied, those with SJS/TEN-like aGVHD showed more severe skin and mucosal reactions, higher overall disease grading, and increased complications like anemia and renal dysfunction.
- The findings indicated a concerning 80% mortality rate for SJS/TEN-like aGVHD during follow-up, emphasizing the need for early diagnosis and distinct treatment from drug-induced SJS/TEN.
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