Extensive Toxic Epidermal Necrolysis Versus Acute Graft Versus Host Disease After Allogenic Hematopoietic Stem-Cell Transplantation

Abstract

Toxic epidermal necrolysis (TEN) is a rare complication after allogeneic hematopoietic stem-cell transplantation and carries high mortality rates. Graft-vs-host disease (GVHD) is also a life-threatening complication, and potentially indistinguishable from TEN because of similar clinical symptoms. However, current therapeutic recommendations differ between these two conditions, thereby posing a diagnostic dilemma. The authors, herein, present a complicated postoperative course after bone marrow transplantation with concurrent gastrointestinal and hepatic GVHD, and extensive epidermolytic disease compatible with both severe cutaneous GVHD and TEN. An early consult to a specialized burn service, and prompt transfer to a burn intensive care unit with extensive supportive care and nursing are of paramount importance in the management of immunosuppressed patients with TEN. Better understanding of the pathogenesis of TEN and GVHD after hematopoietic stem-cell transplantation, further treatment strategies, and more advanced diagnostic techniques are still needed to achieve acceptable mortality rates.

Pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States

“…There were no cases of GVHD. Severe cutaneous GVHD can be difficult to distinguish from TEN 16,24 , and it is possible that some patients diagnosed with a malignancy and SJS-TEN/TEN actually had GVHD. The absence of TEN in 2011 could be attributed to sampling, as the NIS is a 20% sample of all US hospitalizations, and pediatric TEN is rare.…”

“…Calculation of incidences excluded patients who were transferred to another hospital. Costs were adjusted for inflation to the year 2014 according to the Consumer Price Index from the US Bureau of Labor Statistics 16 . Summary statistics were generated for LOS and inflation-adjusted cost-of-care, including sum, mean, standard deviation, minimum, maximum and median.…”

Graft-Versus-Host Disease After Simultaneous Pancreas–Kidney Transplantation: A Case Report and Review of the Literature

“…Diagnosis can be made clinically and by biopsy but is facilitated by demonstration of donor chimerism by FISH and/or STR which can be used for monitoring treatment response. Early diagnosis may allow successful treatment with (1) supportive care aimed at treating or preventing infections and cytopenias and (2) elimination of donor chimerism through high dose steroids, higher calcineurin inhibitors levels, and possibly the use of ECP. Whether ECP allows for a faster steroid taper resulting in fewer side effects remains to be elucidated.…”

“…This T cell-mediated disorder consists of donor immune cells (the graft) recognizing the transplant recipient (the host) as foreign, thereby initiating an immune reaction against the host. Common manifestations include fever, maculopapular rash, vomiting, diarrhea and abnormal liver enzymes (1).…”

Toxic Epidermal Necrolysis in Recessive Dystrophic Epidermolysis Bullosa following Bone Marrow Transplantation

“…In a recent case series of 36 children with TEN treated with IVIG, systemic corticosteroids, or a combination of both all patients survived (1). While adults with TEN in the setting of HCT have risk of mortality approaching 100% in some case series, it is unclear if this is also true in children (5). …”