Graft-versus-host reaction affecting lesional skin but not normal skin in a patient with piebaldism.
A case of graft-vs.-host disease (GVHD) arising solely within an area affected by piebaldism is described. The patient, a 35-year-old woman with a single hypopigmented patch on the right leg present since birth, had received an allogeneic bone marrow transplant (BMT) from an HLA-identical sibling donor, for treatment of a myelodysplastic syndrome (MDS). Beginning on day +38 post-BMT, the patch developed changes which were histologically consistent with GVHD. Syngeneic mixed epidermal cell-lymphocyte reaction (MECLR) testing of tissue from the patch, and from adjacent normal skin, showed differences which suggest that piebaldism-affected skin is immunologically different from normal skin. These findings may offer new insight into the pathophysiology of this disorder.
Chow
R K
RK
Division of Dermatology, BC Cancer Agency, Vancouver, British Columbia, Canada.
Stewart
W D
WD
Ho
V C
VC
D000328:Adult; D000754:Anemia, Refractory, with Excess of Blasts / Q000628:therapy; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D004817:Epidermis / Q000276:immunology; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008214:Lymphocytes / Q000276:immunology; D016116:Piebaldism / Q000150:complications* / Q000276:immunology / Q000473:pathology