Acute Graft-Versus-Host Disease Presenting as Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

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• In this multicenter retrospective study involving 31 patients with acute graft-versus-host disease (aGVHD), 15 patients had Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN)–like presentation. Patients with SJS/TEN–like aGVHD had a higher body surface area involvement and a higher incidence of pancytopenia, liver dysfunction, renal dysfunction, and bacteremia. The mortality rate among patients with SJS/TEN–like aGVHD was 80% during the follow-up period compared with 25% among patients with non–SJS/TEN–like aGVHD. Sepsis was the most common cause of death.
• It is critical to differentiate drug-induced SJS/TEN from SJS/TEN–like aGVHD because of their similar clinical symptoms. SJS/TEN–like aGVHD is associated with a poor prognosis.

–  Katherine M. Stiff, MD

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.

Journal of the American Academy of Dermatology

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

J Am Acad Dermatol 2022 Oct 21;[EPub Ahead of Print], YT Hung, YW Chen, YL Huang, YJ Lin, CB Chen, WH Chung 

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Benjamin Hidalgo-Matlock
Skin Care Physicians of Costa Rica

Clinica Victoria en San Pedro: 4000-1054
Momentum Escazu: 2101-9574

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