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AT-A-GLANCE

AT-A-GLANCE

  • Acute graft-versus-host disease (GVHD) is a serious and potentially life-threatening sequelae of allogeneic hematopoietic stem cell transplantation. Skin manifestations range from a mild, asymptomatic morbilliform eruption to full-thickness skin loss resembling toxic epidermal necrolysis. Hepatic involvement is characterized by elevated total bilirubin. GI disease manifests as abdominal pain, nausea/vomiting, and secretory diarrhea.

  • The most important risk factor for chronic GVHD is a history of acute GVHD. Other important factors include human leukocyte antigen incompatibility, older age, female donor/male recipient, and peripheral blood stem cell source (vs bone marrow).

  • Chronic GVHD of the skin may resemble lichen planus, lichen sclerosus, morphea, systemic sclerosis, or eosinophilic fasciitis. The presentation can be remarkably variable, however, and may resemble folliculitis, keratosis pilaris, or psoriasis. Both epidermal and sclerotic skin manifestations may present at sites of trauma.

  • Patients with chronic GVHD may manifest other autoimmune skin diseases, such as vitiligo and alopecia areata.

  • The pathogenesis of chronic GVHD is poorly understood and nearly every organ system is at risk. The skin, oral mucosa, eyes, GI tract, and lungs are most frequently involved. In many cases, organ system disease resembles known autoimmune conditions.

  • Topical steroids and topical calcineurin inhibitors are used to treat mild, skin-limited chronic GVHD, and systemic steroids are first line in the treatment of moderate to severe chronic GVHD.

  • Optimal dermatologic management of chronic GVHD of the skin requires an understanding of other organ involvement, infection status, and cancer relapse risk. Close communication with the transplantation physician and a “team approach” to multispecialty management is needed.

EPIDEMIOLOGY

Approximately 50,000 hematopoietic stem cell transplantation (HCT) procedures are performed worldwide each year for an expanding array of hematologic malignancies and marrow failure syndromes, metabolic disorders, and immunodeficiencies. HCT may use autologous, syngeneic, or allogeneic donor hematopoietic stem cells (HCs). During autologous transplantation, the patient’s own HCs are returned to the patient following preparative chemotherapy with or without radiation. Allogeneic HCT (allo-HCT) is the transfer of HCs from a related (nonidentical) or unrelated donor to a recipient. Syngeneic transplantation is the transfer of HCs between identical twins. Graft-versus-host disease (GVHD) is the primary cause of non–relapse-related morbidity and mortality in allo-HCT, and also rarely occurs following transplantation of solid organs, transfusion of blood products, and autologous transplantation.

Transplantation regimens have advanced rapidly since the first successful allo-HCT was performed in 1968.1 Peripheral blood is now the primary source of donor HCs at many transplantation centers because of decreased risks of relapse and improved survival, although bone marrow transplantation is associated with reduced risk of GVHD.2 Reduced intensity and nonmyeloablative conditioning permitted older patients and others who would not tolerate myeloablative chemotherapy a chance for cure with HCT.3 Umbilical cord blood has gained prominence as a stem cell source in both pediatric and adult HCT given the low risks of GVHD and high engraftment rates.4 Donor leukocyte infusions, the administration of additional donor HCs ...

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