INCIDENCE

Although advances in conditioning regimens, cell delivery, and graft versus host disease (GVHD) prophylaxis have improved the prognosis of patients following allogeneic stem cell transplantation (SCT), chronic GVHD (cGVHD) of the skin continues to be a significant source of long-term patient morbidity in SCT recipients. The skin is the most common target of cGVHD and is remarkably variable in its presentations, resulting in potential cosmetic, functional, and even life-threatening sequelae.

PRESENTATION

Cutaneous cGVHD may overlap with acute disease, occur de novo, or develop after a disease-free interval following acute GVHD. Traditionally, the onset of cGVHD was classified as greater than 100 days after hematopoietic stem cell transplantation (HSCT) and heralded by the appearance of lichen planus-like lesions or sclerodermoid (sclerodermalike) changes. Newer HSCT regimens, as well as the delivery of donor lymphocyte infusions, have altered the timing of this “classic” presentation. Accurate estimation of the true incidence of cutaneous cGVHD is further hindered by the diversity of its clinical presentation (Table 16.1).

An effort to create a systematic classification of the cutaneous manifestations of cGVHD were undertaken as part of the 2005 National Institute of Health (NIH) Consensus Development Project. The proposed criteria for the diagnosis of cutaneous cGVHD include (1) distinction from acute GVHD; (2) at least one diagnostic clinical sign of cGVHD or one distinctive manifestation confirmed by biopsy or relevant testing; (3) exclusion of other possible diagnoses.