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. 2019 Dec;8(4):258-278.
doi: 10.1007/s13671-019-00280-3. Epub 2019 Nov 11.

Inpatient Management of Mucocutaneous GVHD

Toral Vaidya  1 , Christian Menzer  1 , Doris M Ponce  2   3 , Alina Markova  1   4
Affiliations

Inpatient Management of Mucocutaneous GVHD

Toral Vaidya et al. Curr Dermatol Rep. 2019 Dec.

Abstract

Purpose of review: Graft-versus-host disease (GVHD) is an immune mediated disorder affecting 30 - 70% of patients after allogeneic hematopoietic stem cell transplantation (alloHSCT), and is a major cause of morbidity and non-relapse mortality (NRM) [1]. Dermatologists play a critical role in acute and chronic GVHD, as skin involvement is common and often the earliest involved site of disease [2].

Recent findings: GVHD shares clinical and histopathological features with a variety of other skin diseases, requiring thorough consideration of differential diagnoses in hematopoietic stem cell transplantation (HSCT) recipients with lesions suggestive of cutaneous GVHD. Treatment considerations for GVHD are influenced by factors such as disease classification, overall grading, organ involvement, associated symptoms, and immunological anti-tumor effect. Several treatments are available and may be indicated as monotherapy or adjuvant therapy to allow faster withdrawal or tapering of immunosuppression. While corticosteroids are often first line therapy, oral ruxolitinib has been recently approved for treatment of steroid-refractory aGHVD, and oral ibrutinib has been approved for steroid-refractory cGHVD.

Summary: This article provides current clinical, diagnostic, and therapeutic considerations relevant to the hospitalist for both acute and chronic mucocutaneous GVHD. Optimal inpatient management of these diseases requires an interdisciplinary team.

Keywords: GVHD; acute graft versus host disease; chronic graft versus host disease; dermatology hospitalist; graft versus host disease; inpatient dermatology; inpatient management.

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Conflict of interest statement

Conflict of Interest The authors declare no conflict of interest.

Figures

Figure 1.
Figure 1.
Clinical presentations of cutaneous aGVHD including early stage erythematous, blanchable macules (a,b) and late stage bullae (c) and skin sloughing (d)
Figure 2.
Figure 2.
Non-sclerotic cutaneous cGVHD. Lichen planus-like features with disseminated polygonal papules and plaques with fine white lines resembling Wickham striae on the dorsal hands and knees (a, d) and disseminated dyspigmented areas of palms, arms and legs (b, c, d).
Figure 3.
Figure 3.
Clinical presentation of sclerotic cutaneous cGVHD with progression within 7 months leading to restricted range of motion with decreased flexion of the cubital joint (a, b) and the wrist with clinically progressed “prayer sign” (c, d).
Figure 4.
Figure 4.
Oral cGVHD of the tongue with erythema and yellowish aphtous ulcerations of the lateral tongue (a), and reticular lichen planus-like hyperkeratotic plaques of the tongue dorsum (b).
Figure 5.
Figure 5.
Genital cGVHD with vulvovaginal sclerotic tissue changes resulting in introital stenosis.
Figure 6.
Figure 6.
Chronology of aGVHD and differential diagnoses.
See this image and copyright information in PMC

References

    1. Jagasia M, et al., Risk factors for acute GVHD and survival after hematopoietic cell transplantation. Blood, 2012. 119(1): p. 296–307. - PMC - PubMed
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    1. Zeiser R and Blazar BR, Acute Graft-versus-Host Disease-Biologic Process, Prevention, and Therapy. N Engl J Med, 2017. 377(22): p. 2167–2179.

      *This reference provides an overview of the biologic features of acute GVHD, and preventative and therapeutic strategies based on recent developments.

    1. Elsabbagh EM, et al., Acute GvHD Incidence and Outcome: Single Center Experience. Biology of Blood and Marrow Transplantation, 2017. 23(3): p. S231.

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