Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ DEFINITION ++ Mycosis fungoides and its variant Sézary syndrome, the two principal types of cutaneous T-cell lymphoma (CTCL), are malignant proliferations of mature memory T lymphocytes of the phenotype CD4+CD45RO+ (memory T cells), which invariably involve the skin. Other types of lymphoma may also have prominent skin involvement (see Table 65–1). In cases of CTCL in which erythrodermia is a prominent feature, the major considerations are shown in Table 65–2. ++Table Graphic Jump LocationTABLE 65–1WORLD HEALTH ORGANIZATION–EUROPEAN ORGANIZATION FOR RESEARCH AND TREATMENT OF CANCER CLASSIFICATION OF PRIMARY CUTANEOUS T-CELL AND NATURAL KILLER CELL LYMPHOMASView Table|Favorite Table|Download (.pdf) TABLE 65–1 WORLD HEALTH ORGANIZATION–EUROPEAN ORGANIZATION FOR RESEARCH AND TREATMENT OF CANCER CLASSIFICATION OF PRIMARY CUTANEOUS T-CELL AND NATURAL KILLER CELL LYMPHOMAS Mycosis Fungoides (MF) MF variants and subtypes Folliculotropic MF Pagetoid reticulosis Granulomatous slack skin Sézary Syndrome Adult T-Cell Leukemia/Lymphoma Primary Cutaneous CD30+ Lymphoproliferative Disorders Primary cutaneous anaplastic large cell lymphoma Lymphomatoid papulosis Subcutaneous Panniculitis-Like T-Cell Lymphoma Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Primary Cutaneous Peripheral T-Cell Lymphoma, Unspecified Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma (provisional) Cutaneous γδ T-cell lymphoma (provisional) Primary cutaneous CD4+ small-/medium-size pleomorphic T-cell lymphoma (provisional) Precursor Hematologic Neoplasm CD4+/CD56+hematodermic neoplasm (blastic NK-cell lymphoma) Source: Williams Hematology, 9th ed, Chap. 103, Table 103–1. ++Table Graphic Jump LocationTABLE 65–2CLASSIFICATION OF ERYTHRODERMIC CUTANEOUS T-CELL LYMPHOMAView Table|Favorite Table|Download (.pdf) TABLE 65–2 CLASSIFICATION OF ERYTHRODERMIC CUTANEOUS T-CELL LYMPHOMA Erythrodermic Subset (T4) Preexisting MF Blood Sézary syndrome Rarely Leukemia: B2 Erythrodermic mycosis Fungoides Always Normal or minimally abnormal: B0–B1 Erythrodermic cutaneous T-cell lymphoma, not otherwise specified Absent Normal or minimally abnormal: B0–B1 MF, mycosis fungoides. Source: Williams Hematology, 9th ed, Chap. 103, Table 103–4. ++ EPIDEMIOLOGY ++ CTCL is more common in males than females. Median age at diagnosis is 55 years. In the United States, there are approximately 3000 cases per year, representing about 3% of lymphomas. Americans of African descent have a higher incidence and more progressive disease than Americans of European descent. Asians and Hispanics are much less often affected. The etiology is unknown. ++ CLINICAL FINDINGS ++ Patients usually present with nonspecific skin lesions (chronic dermatitis) occurring years before diagnosis. Early in disease, patients are often diagnosed with eczema (spongiotic dermatitis), psoriatic-like dermatitis, or other nonspecific dermatoses associated with pruritus. Histologic diagnosis may be difficult in early stages. Neoplastic infiltrates may be minimal, masked by normal inflammatory cells, and the neoplastic mature CD4+ phenotype may be misinterpreted as normal inflammatory cells. Mycosis fungoides may be divided into patch stage (patch-only disease), plaque stage (both patches and plaques) and tumor stage (more than one tumor along with patches and plaques). A patch is defined as a flat lesion with varying degrees of erythema with fine scaling; a plaque is defined as a demarcated, erythematous, brownish lesion, with variable scaling of at least ... GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. What is MyAccess? Create a FREE MyAccess profile to: Use this site remotely Bookmark your favorite content Track your self-assessment progress and more!