Poikilodermatous mycosis fungoides

Abstract

A 60‐year‐old woman presented with a 10‐year history of pruritic patch lesions that had originated on her breast and progressed over the years to involve the lower part of the abdomen. Different cutaneous biopsies were obtained during this period revealing a superficial chronic dermatitis. She had been treated previously with intermittent cycles of medium‐strength topical steroids. This regimen was of only temporary benefit and progression of the skin lesions had occurred over the last year. The patient's medical history was otherwise unremarkable.Physical examination revealed atrophic red–brown skin with telangiectasia predominantly located on the breast (Fig. 1). Clinical examination also revealed various red‐to‐brown scaly plaques affecting the lower part of the abdomen.Representative clinical appearance with reticulate pigmentation, atrophy, scaling, and telangiectasiaimageTwo skin biopsies were obtained from the breast and the abdomen. Routine hematoxylin and eosin staining of the breast area revealed an atrophic epidermis covered by a scale of hyperkeratosis and parakeratosis. Scattered intraepidermal atypical lymphocytes were observed. In the dermis, there was a superficial band‐like infiltrate of atypical lymphocytes sparing the dermo‐epidermal interface. Pigmentary incontinence and dilated capillaries were present within the superficial dermis (Fig. 2). All of these findings were compatible with the poikilodermatous variant of cutaneous T‐cell lymphoma (CTCL). Plaques affecting the lower part of the abdomen showed clinical features of classic mycosis fungoides (MF), and histology revealed an epidermis with acanthosis, a dense band‐like infiltrate within the upper dermis, and a prominent epidermotropism with intraepidermal collections of lymphocytes. Immunohistochemical analysis of both biopsies identified the cells as CD2+, CD3+, CD4+, CD8–. Further detailed examination did not reveal any visceral or lymph node involvement.Biopsy specimen of the breast. (A) Atrophic epidermis and a superficial band‐like infiltrate (hematoxylin and eosin; original magnification, ×40). (B) Scattered intraepidermal atypical lymphocytes and melanophages within the superficial dermis (hematoxylin and eosin; original magnification, ×200)imageWhen topical mechlorethamine had no significant effect, psoralen plus UV‐A (PUVA) phototherapy was started at 0.5 J/cm2 twice weekly. After 20 treatments (cumulative dose, 143.5 J/cm2), the lesions had disappeared, leaving slight postinflammatory hyperpigmentation. No maintenance treatment was required and the patient remained in complete remission during a 14‐month follow‐up period.