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  • Cutaneous lymphomas are clonal proliferations of neoplastic T or B cells, rarely natural killer cells or plasmacytoid dendritic cells. Cutaneous lymphomas are the second most common group of extranodal lymphomas. The annual incidence is estimated to be 1 per 100,000.
  • The WHO-EORTC classification is shown in Table 20-1.
  • For rare conditions not dealt with in this Atlas the reader is referred to C Assaf, W Sterry, in K Wolff et al (eds): Fitzpatrick's Dermatology in General Medicine, 7th ed. New York, McGraw-Hill, 2008, pp 1386–1402.

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Table 20-1 WHO-EORTC Classification of Primary Cutaneous Lymphomas
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ICD-9 : 204.0/208.9 • ICD-10 : C83/E88   Image not available. Image not available.

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  • Adult T cell leukemia/lymphoma (ATLL) is a neoplasm of CD4+/CD25+ T cells, caused by human T cell lymphotrophic virus I (HTLV-I).
  • Manifested by skin infiltrates, hypercalcemia, visceral involvement, lytic bone lesions, and abnormal lymphocytes on peripheral smears.
  • HTLV-I is a human retrovirus. Infection by the virus does not usually cause disease, which suggests that other environmental factors are involved. Immortalization of some infected CD4+ T cells, increased mitotic activity, genetic instability, and impairment of cellular immunity can all occur after infection with HTLV-I. These events may increase the probability of additional genetic changes, which, by chance, may lead to the development of leukemia 20–40 years after infection in some people (≤5%). Most of these effects have been attributed to the HTLV-I-encoded protein tax.
  • ATLL occurs in southwestern Japan (Kyushu), Africa, the Caribbean Islands, southeastern United States. Transmission is by sexual intercourse, perinatally, or by exposure to blood or blood products (same as HIV).
  • There are four main categories. In the relatively indolent smoldering and chronic forms, the median survival is ≥2 years. In the acute and lymphomatous forms, it ranges from only 4–6 months.
  • Symptoms include fever, weight loss, abdominal pain, diarrhea, pleural effusion, ascites, cough, sputum. Skin lesions occur in 50% of patients with ATLL. Single to multiple small, confluent erythematous, violaceous papules (Fig. 20-1), ±purpura; firm violaceous to brownish nodules (Fig. 20-2); papulosquamous lesions, ...

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