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INTRODUCTION

KEY POINTS

  • Cutaneous T-cell lymphoma (CTCL) refers to a group of non-Hodgkin lymphomas that primarily involve the skin.

  • Mycosis fungoides (MF) represents the most common form of CTCL.

  • The incidence and mortality rate of MF are higher in African Americans than in Caucasians, especially in African American females.

  • Hypopigmented patches and plaques are not infrequently the presenting signs of MF in patients with skin of color.

  • The age of onset is lower in patients with hypopigmented MF compared with nonhypopigmented disease.

  • Treatment of CTCL includes topical nitrogen mustard, phototherapy, oral and systemic chemotherapy, and radiation therapy.

Patients with skin of color comprise an important subset of those affected with cutaneous T-cell lymphoma (CTCL). CTCL refers to a group of non-Hodgkin lymphomas that primarily involve the skin and may later involve the lymph nodes, peripheral blood, and other organs. This group of lymphomas includes mycosis fungoides (MF) and Sézary syndrome. MF was first described in 1806 by the French dermatologist Alibert, who named the cutaneous nodules according to their mushroom-like appearance, even though fungi are not involved in the etiopathogenesis. MF usually presents with erythematous patches and scaly plaques, sometimes with an affinity for follicles.1 Less common presentations include hypopigmented macules, pustules, bullae, keratoderma, granulomatous papules and nodules, slacked skin, and subcutaneous plaques.2,3 Hypopigmented lesions are not infrequently the presenting sign of MF in patients with skin of color. Malignant cells have an affinity for the epidermis, allowing skin-directed therapy for most patients. More advanced stages of the disease are characterized by loss of this epidermal affinity. Sézary syndrome is the leukemic form of MF, characterized by generalized erythroderma, lymphadenopathy, and atypical T-cells located in the peripheral blood.

EPIDEMIOLOGY

According to Weinstock and colleagues,4,5,6,7 the incidence of MF is 6.4 persons per million annually. The median survival is 9.7 years, but depends largely on the tumor-node-metastasis (TNM) stage at diagnosis. There was an estimated 3.2-fold rise in incidence of MF between 1969 to 1971 and 1984 but no further increase from 1984 to 1992. The cause of this increased incidence is unknown but may be the result of advances in diagnosis and improved reporting of new cases. Unlike other lymphomas, MF is more common in African Americans than Caucasians, with an incidence 1.6 times higher in African Americans than Caucasians.5 In addition, the rate ratio for mortality is 2.4 times higher among African Americans than among Caucasians. On the other hand, the incidence in Asians and Hispanics is only 0.6 that of Caucasians. Early-onset MF, defined as patients with onset before age 40 years, has been seen more commonly in African American and Hispanic women. One study by Sun et al8 found African American and Hispanic women to be twice as likely to present with early-onset MF compared with Caucasian women. Furthermore, African American women with early-onset MF had the ...

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