Leucoderma associated with flares of erythrodermic cutaneous T-cell lymphomas: four cases

Abstract

We describe four patients with erythrodermic cutaneous T-cell lymphomas (two with erythrodermic mycosis fungoides, and two with Sézary syndrome) who presented with extensive hypopigmented lesions that occurred during flares of their cutaneous disease. These cases must be distinguished from previously described hypopigmented mycosis fungoides where hypopigmented lesions were the sole manifestation of the lymphoma. In two cases a biopsy was performed on hypopigmented skin, showing an infiltrate of atypical lymphocytes with epidermotropism and absence of melanocytes, as in vitiligo. It is suggested that the hypopigmentation could be due to the cytotoxicity of tumour or reactional lymphocytes directed against melanocytes.

Decreased CD117 expression in hypopigmented mycosis fungoides correlates with hypomelanosis: lessons learned from vitiligo

“…1 In this context, hypopigmentation during flares of erythrodermic cutaneous T-cell lymphoma has been purported to result from reaction of cytotoxic T-cells. 12 On ultrastructural examination, damaged melanosomes 10 and a decreased number of melanosomes 11 have been observed in the lesional skin. The pathogenesis of hypopigmentation in vitiligo is also not completely understood; suggested mechanisms include autoimmune, neural, biochemical, oxidative stress, autocytotoxic, viral, and melanocyte detachment.…”

“…[6][7][8][9] The mechanism of hypomelanosis in hypopigmented mycosis fungoides is as yet unclear. Damaged melanosomes 10 and a decreased number of melanocytes 11,12 have been reported in hypopigmented mycosis fungoides lesions.…”

Concomitant Mycosis Fungoides and Vitiligo: How Mycosis Fungoides May Contribute to Melanocyte Destruction

“…This may be similar to melanoma-associated depigmentation that develops in melanoma, which is thought to result from a CD8+ cytotoxic reaction against melanoma tumor cells [25]. Other studies have associated therapeutic agents, including treatment with PUVA and IFNα, with vitiligo in CTCL patients [9,10,11,22,26,27]. …”

“…They suggested that a dense dermal lymphocytic infiltrate composed mainly of helper T cells contributed to melanocyte destruction by stimulating a cell-mediated cytotoxic process. More than a decade later, Bouloc et al [9] described four erythrodermic CTCL patients who developed vitiligo during disease flares. It was hypothesized that depigmentation resulted from the cytotoxic activity of reactive tumor cells directed against melanocyte antigens or from autoantibodies such as antityrosinase.…”

“…However, anecdotal evidence from experts at several CTCL centers across the United States supports that vitiligo occurs more frequently in those with MF than by chance alone, and it is likely that concomitant disease occurrence is underreported. Very few cases of vitiligo developing in patients with CTCL have been cited in the literature [8,9,10,11], and of these, vitiligo has been associated with either a particular CTCL disease state, such as an MF flare or Sézary syndrome, or specific CTCL treatments, including PUVA or interferon alpha (IFNα) therapy. Given that immune system deregulation has been implicated in both diseases, it is conceivable that dysfunction of the aberrant T cells in CTCL may predispose patients to T cell-mediated destruction of melanocytes.…”