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<p>Toxic epidermal necrolysis (TEN) and cutaneous graft-versus-host disease (GvHD) are rare, life-threatening skin eruptions that can occur after hematopoietic stem cell transplantation (HSCT). Because of their overlapping clinical and histologic features, severe cases can be difficult to distinguish. We report on the case of a&#xa0;65-year-old man with myelodysplastic syndrome who underwent allogeneic peripheral blood stem cell transplantation (9/10 HLA-mismatched donor, fludarabine/treosulfan conditioning). On day +35 posttransplant, he presented with a&#xa0;maculopapular eruption (suspected early acute cutaneous GvHD grade&#xa0;I) and a&#xa0;nondiagnostic skin biopsy. On day +49, a&#xa0;rapidly progressive maculopapular exanthema developed, involving approximately 60% of the body surface area. The eruption was partly bullous, showed a&#xa0;positive Nikolsky sign (1&#xa0;and&#xa0;2), and was accompanied by mucosal involvement with hemorrhagic lip lesions and genital erosions. Based on his recent exposure to amoxicillin/clavulanate and sulfamethoxazole/trimethoprim, TEN was initially suspected. Repeat dermatohistopathology and gastrointestinal biopsies after the development of severe diarrhea with blood confirmed severe acute cutaneous and intestinal GvHD (grade&#xa0;IV). Intensive immunosuppressive therapy with high-dose corticosteroids, cyclosporine&#xa0;A, and a&#xa0;JAK-1/2 inhibitor ruxolitinib, combined with extracorporeal photopheresis, resulted in complete cutaneous remission; however, chronic hepatic and intestinal GvHD subsequently developed. This case illustrates the critical diagnostic overlap between TEN and GvHD. Currently, histologic evaluation remains the most effective method to differentiate GvHD from TEN, although in some cases, punch biopsies may not yield a&#xa0;definitive diagnosis. Further research comparing biomarker profiles of GvHD and TEN is needed to improve diagnostic accuracy.</p>

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Wenn die Haut zuerst warnt: TEN-like Präsentation einer akuten kutanen GvHD

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月3日

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  • Maria Sanz Codina,
  • Damian Kostner,
  • Axel Schulenburg,
  • Christina Schellenbacher,
  • Johanna Strobl,
  • Mathias Drach

摘要

Toxic epidermal necrolysis (TEN) and cutaneous graft-versus-host disease (GvHD) are rare, life-threatening skin eruptions that can occur after hematopoietic stem cell transplantation (HSCT). Because of their overlapping clinical and histologic features, severe cases can be difficult to distinguish. We report on the case of a 65-year-old man with myelodysplastic syndrome who underwent allogeneic peripheral blood stem cell transplantation (9/10 HLA-mismatched donor, fludarabine/treosulfan conditioning). On day +35 posttransplant, he presented with a maculopapular eruption (suspected early acute cutaneous GvHD grade I) and a nondiagnostic skin biopsy. On day +49, a rapidly progressive maculopapular exanthema developed, involving approximately 60% of the body surface area. The eruption was partly bullous, showed a positive Nikolsky sign (1 and 2), and was accompanied by mucosal involvement with hemorrhagic lip lesions and genital erosions. Based on his recent exposure to amoxicillin/clavulanate and sulfamethoxazole/trimethoprim, TEN was initially suspected. Repeat dermatohistopathology and gastrointestinal biopsies after the development of severe diarrhea with blood confirmed severe acute cutaneous and intestinal GvHD (grade IV). Intensive immunosuppressive therapy with high-dose corticosteroids, cyclosporine A, and a JAK-1/2 inhibitor ruxolitinib, combined with extracorporeal photopheresis, resulted in complete cutaneous remission; however, chronic hepatic and intestinal GvHD subsequently developed. This case illustrates the critical diagnostic overlap between TEN and GvHD. Currently, histologic evaluation remains the most effective method to differentiate GvHD from TEN, although in some cases, punch biopsies may not yield a definitive diagnosis. Further research comparing biomarker profiles of GvHD and TEN is needed to improve diagnostic accuracy.