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 176-1 and 176-2). She had been from evacuated New Orleans following Hurricane Katrina. She had waded through polluted waters for hours before being rescued by a 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 is currently receiving narrow-band UVB treatment twice weekly.
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. (Courtesy of Richard P. Usatine, MD.)
Hypopigmented patches on the arm of the woman in Figure 176-1 with mycosis fungoides. (Courtesy of Richard P. Usatine, MD.)
CTCL clinically and biologically represent a heterogeneous group of non-Hodgkin lymphomas, with MF and Sézary syndrome being the most common subtypes.1
- The annual incidence of CTCL in the United States has increased from 2.8 per 1 million (1973 to 1977) to 9.6 per 1 million (1998 to 2002) according to data from Criscione and Weinstock.2
- CTCL is a rare disease, with 1000 new cases per year in the United States, comprising approximately 0.5% of all non-Hodgkin lymphoma cases.3,4
- The two most common types of CTCL are MF (50% to 72%), which is generally indolent in behavior, and Sézary syndrome (1% to 3%), an aggressive leukemic form of the disease.2
- It is more common in African Americans than in whites, with an incidence ratio of 6:1.3
- It is more common in males, with a male-to-female ratio of 2:1.
- Median age at presentation is between 50 and 70 years,2 although pediatric and young adult cases do occur.1
- The exact etiology of CTCL is unknown, but environmental, infectious, and genetic causes have been suggested. CTCL is a malignant lymphoma of helper T cells that usually remain confined to skin and lymph nodes (LNs). MF is a specific type of CTCL named for the mushroom-like skin tumors ...