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PATIENT STORY

A 52-year-old black woman presented with a 7-month history of a hypopigmented rash in a symmetric distribution on her upper thighs and arms (Figures 180-1 and 180-2). She had been evacuated from New Orleans following Hurricane Katrina. She had waded through polluted waters for hours before being rescued by boat. Four days passed before she had access to a shower, at which time she noticed a single erythematous spot the size of a silver dollar on her left thigh. Over the next several weeks, it faded to hypopigmented macules and plaques and eventually spread to both thighs and arms. The physical examination revealed no lymphadenopathy. A hematoxylin and eosin (H&E) stain of a full-thickness punch biopsy revealed "cerebriform" lymphocytes at the dermal–epidermal junction characteristic of mycosis fungoides (MF), a type of cutaneous T-cell lymphoma (CTCL). Her blood tests were essentially normal, and she was HIV-negative. The patient reported no improvement with topical high-potency generic steroid to affected areas and was started on narrow-band UVB treatment twice weekly.

FIGURE 180-1

The hypopigmented patches of mycosis fungoides on the thighs of a 52-year-old black woman. This is the patch stage of the disease. Although this mimicked vitiligo, the distribution and appearance warranted a biopsy that provided a definitive diagnosis of mycosis fungoides. (Reproduced with permission from Richard P. Usatine, MD.)

FIGURE 180-2

Hypopigmented patches on the arm of the woman in Figure 180-1 with mycosis fungoides. (Reproduced with permission from Richard P. Usatine, MD.)

INTRODUCTION

CTCL clinically and biologically represent a heterogeneous group of non-Hodgkin lymphomas.1

EPIDEMIOLOGY

  • The annual incidence of CTCL in the United States increased from 2.8 per 1 million (1973–1977) to 10.2 per 1 million (2005–2009).2 The incidence appears to have stabilized over the past 20 years, with an annual percentage change of only 0.1%.2

  • CTCL is a rare disease, with about 3000 new cases per year in the United States.

  • The most common types of CTCL are MF (50% to 72%), which is generally indolent in behavior (Figures 180-1, 180-2, 180-3, 180-4, 180-5); primary cutaneous CD30+ lymphoproliferative disorders (LPD; about 30%); and Sézary syndrome (SS; 1% to 3%), an aggressive leukemic form of the disease.3 LPDs include lymphomatoid papulosis (12%) and cutaneous anaplastic large-cell lymphoma (C-ALCL, 9%) (Figure 180-6).3,4

  • CTCL is more common in African Americans than in whites, with an incidence rate ratio of 1.3:1 (2005–2009).2

    • African-American and Hispanic individuals have an earlier onset of disease; in one study database, the mean age at diagnosis was 60.0 ± 14.8 years for Caucasian, 49.3 ± 16.9 years for African-American, and 47.4 ± 17.8 ...

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