Graft-versus-host reaction affecting lesional skin but not normal skin in a patient with piebaldism

Abstract

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.

Differential diagnosis of skin lesions after allogeneic haematopoietic stem cell transplantation

“…In the late stage the patient develops sclerodermoid plaques on the trunk, buttocks and thighs and in severe cases also on the extremities (Figure 9). Skin involvement of cGVHD localized to an area of piebaldism has been reported 47 . Linear lesions have been reported both following Blaschko's lines 48 and in a dermatomal distribution 49,50 .…”

“…Skin involvement of cGVHD localized to an area of piebaldism has been reported. 47 Linear lesions have been reported both following Blaschko's lines 48 and in a dermatomal distribution. 49,50 Besides lichenoid and sclerodermoid lesions, patients with cGVHD can show clinical and histological lesions resembling ichthyosis vulgaris.…”

Piebaldism: an update

“…2 In one case, cutaneous graft-versus-host lesions were observed only in areas affected by piebaldism, suggesting that piebaldism-affected skin may be immunologically different from normal skin. 11 Pigmentary anomalies in piebaldism are typically restricted to the hair and skin. However, rare associations have been reported with piebaldism, in particular Hirschprung's disease or aganglionic megacolon, supporting evidence of a network of interacting genes and proteins for the regulation of melanocytes and enteric plexus neurons during their development at the time of embryogenesis.…”

“…Progression has been described in isolated cases, as in a mother and daughter with a novel Val620Ala (1859T→C) mutation in the kit gene upon genetic analysis 2 . In one case, cutaneous graft‐versus‐host lesions were observed only in areas affected by piebaldism, suggesting that piebaldism‐affected skin may be immunologically different from normal skin 11 …”

Cutaneous acute graft‐versus‐host disease that coincided with segmental type nevus spilus following an allogenic bone marrow transplantation

“…Dear Editor, Acute graft-versus-host disease (GVHD) is a complication of allogeneic hematopoietic stem cell transplantation and cutaneous manifestations can be a major initial symptom. 1 Here, we present a case of acute cutaneous GVHD, in which erythematous papules were exclusively observed in the area of nevus spilus that was distributed in a checkerboard pattern.…”