Graft-Versus-Host Disease (GVHD)

Figure 3.12 A: GVHD, acute* B: GVHD, acute* * Reprint from Morgan MB, Smoller BR, Somach SC, Deadly Dermatologic Diseases . New York, NY: Springer; 2007 C: GVHD, chronic (Reprint from Burgdorf WH, Plewig G, Landthaler M, Wolff HH, eds. Braun-Falco’s Dermatology. 3rd ed . Heidelberg: Springer; 2009)

• Clinical syndrome resulting from the transfer of immunologically competent cells to an immunosuppressed host
• Donor lymphocytes recognize the recipient as ‘foreign’ and mount an immunological attack primarily against the skin, mucosa, gastrointestinal tract and liver
• Histocompatibility (both major and minor complexes) between the donor and host is the most important factor in the development of GVHD
• One of the major complications of allogeneic hematopoietic stem cell transplantation (can also occur after transfusion of unirradiated blood products or donor lymphocytes in setting of solid organ transplantation)
• Two forms (acute and chronic GVHD) based on time of presentation since transplant date (part of same spectrum)
• Acute GVHD
  • Occurs within first 100 days following transplantation (typically within 1–3 weeks after transplantation)
  • Presents with a maculopapular eruption which may coalesce into confluent erythema (± evolve into erythroderma or bullae resembling toxic epidermal necrolysis); acral erythema with violaceous discoloration of pinna of ear suggestive
  • Triad of dermatitis, enteritis with diarrhea, and hepatitis with abnormal LFTs, ± high fever, conjunctival erythema
  • Histology: vacuolization of basal layer, necrotic keratinocytes, sparse perivascular or interface dermatitis, ± complete epidermal necrosis (severe cases)
  • Course: 30–50% of patients with moderate to severe acute GVHD die
  • Treatment: systemic corticosteroid
• Chronic GVHD
  • Occurs after mean of 4 months (as early as 40 days)
  • Evolves from acute GVHD in approximately 50% surviving patients, otherwise de novo
  • Divided into early lichenoid and late sclerodermoid
    • Lichenoid GVHD: violaceous to erythematous lichenoid papules over dorsal hands, forearms, trunk and may become widespread, ± mucous membrane involvement (lacy white plaques or erosions in mouth, salivary gland involvement with Sjögren-like syndrome)
    • Sclerodermoid GVHD: sclerotic plaques similar to morphea, ± hyperpigmentation, ± sicca symptoms, may also involve the gastrointestinal tract, lungs, liver and musculoskeletal system
  • Main cause of death of chronic GVHD: infection due to immunosuppression
  • Histology: chronic lichenoid GVHD similar to lichen planus; sclerodermoid GVHD with epidermal atrophy and dermal fibrosis
  • Treatment: topical calcineurin inhibitor, PUVA, UVB, prednisone, hydroxychloroquine, cyclosporine, mycophenolate mofetil, azathioprine, photopheresis