10.5826/dpc.1202a73
text/xml
2160-9381
1893
en
Sibel Ersoy-Evans
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
dermatopathology
Hyperacute Graft Versus Host Disease Mimicking Stevens-Johnson Syndrome in a Patient with Allogeneic Hematopoietic Stem Cell Transplantation
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-05-02
2021-07-16
2022-05-02
2022-05-24
e2022073
Dermatol Pract Concept
Sibel Ersoy-Evans
2022/05/02
10.5826/dpc.1202a73
e2022073
Department of Dermatology and Venereology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
2160-9381
dermatopathology
en
e2022073
Hyperacute Graft Versus Host Disease Mimicking Stevens-Johnson Syndrome in a Patient with Allogeneic Hematopoietic Stem Cell Transplantation
https://dpcj.org/index.php/dpc/article/download/1893/1589
                    Dermatology Practical & Conceptual


   Hyperacute Graft-Versus-Host Disease Mimicking
     Stevens-Johnson Syndrome in a Patient With
           Allogeneic Hematopoietic Stem
                 Cell Transplantation
          Ecem Bostan1, Duygu Gulseren1, Deniz Ates Ozdemir2, Ibrahim Haznedaroglu3,
                                     Sibel Ersoy-Evans1


1 Department of Dermatology and Venereology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
2 Department of Pathology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
3 Department of Hematology, Hacettepe University, Faculty of Medicine, Ankara, Turkey



Key words: graft versus host disease, pathology, skin, Stevens-Johnson syndrome
Citation: Bostan E, Gulseren D,Ozdemir DA, Haznedaroglu I, Ersoy-Evans S. Hyperacute Graft-Versus-Host Disease Mimicking Stevens
Johnson Syndrome in a Patient with Allogeneic Hematopoietic Stem Cell Transplantation.Dermatol Pract Concept. 2022;12(2):e2022073.
DOI: https://doi.org/10.5826/dpc.1202a73
Accepted: September 7, 2021; Published: April 2022
Copyright: ©2022 Bostan et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None
Competing interests: None.
Authorship: All authors have contributed significantly to this publication
Corresponding author: Ecem Bostan, Hacettepe University, Faculty of Medicine, Department of Dermatology and Venereology, Ankara,
Turkey. E-mail: bostanecem@gmail.com




Introduction                                                            allogeneic haploidentical HSCT from her younger sister for
                                                                        AML. Three days after HSCT, the patient developed neutro-
Grafts-versus-host disease (GvHD) is a severe systemic com-             penic feverand intractable diarrhea; intravenous (IV) mero-
plication most commonly occurring after allogeneic hemato-              penem, teicoplanin and metronidazole were initiated. There
poietic stem cell transplantation (HSCT). We would like to              was no bacterial growth in blood and urine cultures. How-
share our clinical experience with a patient who developed              ever, abdominal computed tomography revealed findings
a grade 4 hyperacute GvHD after haploidentical HSCT for                 compatible with typhlitis. Twelve days after HSCT, she was
acute myelogeneous leukemia (AML).                                      referred to our clinic due to severe mucocutaneous eruption.
                                                                        Dermatological examination showed diffuse hemorrhag-
                                                                        ic-crusted plaques on her lips and neck and dusky-edem-
Case Presentation
                                                                        atous plaques involving volar areas (Figure 1). Our initial
A 18 year-old woman with a history of haploidentical HSCT               diagnoses were Stevens Johnson syndrome (SJS), hyperacute
was consulted for palmoplantar erythematous rash and se-                grade 4 GvHD and paraneoplastic pemphigus. A skin biopsy
vere mucositis. Twelve days prior to the consultation, she had          was taken from the neck which showed mild lymphocytic




Research Letter | Dermatol Pract Concept. 2022;12(2):e2022073                                                                       1
    Figure 1. (A) Initial dermatologic examination at the first consultation revealed diffuse hemorrhagic encrusting of the lips spread-
    ing beyond the vermillion border, diffuse ulcerative-hemorrhagic crusted plaques covering entire anterolateral neck. (B) Diffuse
    plantar erythema and edema. (C) Acral and palmar peeling along with erythema.




        Figure 2. (A) Biopsy shows hyperkeratinization and basal membrane irregularity (H&E, 40x). (B) Basal keratinocytes
        show vacuolar degeneration due to mild lymphocytic infiltrate (H&E, 100x). (C) Higher power reveals damaged basilar ke-
        ratinocytes which show hydropic degeneration with eosinophilic cytoplasm and single cell necrosis (arrows) (H&E, 200x).




inflammation and vacuolar change at the epidermal-dermal                  grounds, recently administered antibiotic drugs teicoplanin
junction, dyskeratosis in basal layer keratinocytes (Figure 2).           and meropenem were changed to cefepime. However, she
Direct immunofluorescence assay was negative. She contin-                 succumbed to death 5 weeks after HSCT.
ued to have intractable diarrhea and gradually increasing
levels of acute phase reactants, alkaline phosphatase (ALP),
                                                                          Discussion
gamma-glutamyl transferase (GGT) and of total/direct bil-
irubin levels. Anti-skin specific antibodies were negative.               GvHD is a severe systemic complication most commonly
With clinical, laboratory and histopathological findings, as              occurring after allogeneic HSCT even though GvHD cases
the diagnosis was hyperacute grade 4 GvHD (the beginning                  have also been reportedin association with solid organ trans-
of the rash was within the first week of HSCT) according                  plantation and non-irradiated blood product transfusion.
to consensus grading of hyperacute/acute GVHD [1]. IV                     Dermatological presentation may range from maculopapular
methylprednisolone was started as maintenance treatment.                  eruption to generalized erythroderma and epidermal slough-
Cyclosporine and mycophenolate mofetil were switched to                   ing mimicking toxic epidermal necrolysis [1]. This severe der-
tacrolimus. Since SJS was could not be excluded on clinical               matological presentation is accepted as stage 4 and grade 4



2                                                                              Research Letter | Dermatol Pract Concept. 2022;12(2):e2022073
GvHD [1]. Schultz et al reported a case of grade 4 GvHD           diagnosis when severe mucositis, palmoplantar involvement,
who developed extensive macular rash along with mucosal           and epidermal detachment similar to SJS are observed.
ulceration within weeks of liver transplantation [1]. Similar
to the letter by Klein et al, our patient developed severe mu-
                                                                  References
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second week of HSCT [2]. Distinctively, she had diarrhea          1. Schulz JT 3rd, Sheridan RL. Severe desquamating disorder after
and progressive elevation in bilirubin, ALP and GGT levels           liver transplant: toxic epidermal necrolysis or graft versus host
                                                                     disease? J Burns Wounds. 2006 Jan 28;5:e1.PMID: 16921414.
favoring the gastrointestinal involvement of acute GvHD.
                                                                     PMCID: PMC1687152.
                                                                  2. Klein B, Kolm I, Nair G, Nägeli MC. TEN-like acute cutane-
Conclusions                                                          ous GvHD in a stem cell recipient- A diagnostic dilemma. J Eur
                                                                     AcadDermatolVenereol. 2021;35(9):e585-e587. . DOI: 10.1111/
In all patients with a prior history of allogeneic HSCT, hyper-      jdv.17310. PMID: 33914967.

acute/acute GvHD should be considered in the differential




Research Letter | Dermatol Pract Concept. 2022;12(2):e2022073                                                                       3
