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Leukemic and Lymphomatous Infiltrates of the Skin

»	Define lymphoma.
Mycosis Fungoides
»	Is there a lymphoma that begins in the skin?
»	What type of lymphoma is mycosis fungoides?
»	How common is mycosis fungoides?
»	How does mycosis fungoides begin?
»	What is parapsoriasis?
»	What type of skin lesions are seen in patients with mycosis fungoides?
»	Describe the three subtypes of mycosis fungoides.
»	What is the TNM classification of mycosis fungoides?
»	How is mycosis fungoides treated?
»	Describe topical nitrogen mustard (HN2) therapy.
»	If a patient develops allergic contact dermatitis to topical nitrogen mustard, does the treatment have to be permanently discontinued?
»	Is photochemotherapy an effective treatment of mycosis fungoides?
»	What are the major side effects of bexarotene in the treatment of patients with cutaneous T-cell lymphoma?
»	How does one manage the side effects of bexarotene?
»	Are interferons effective in treating mycosis fungoides?
»	Is chemotherapy an effective treatment of mycosis fungoides?
»	What is extracorporeal photophoresis?
»	Are there any other FDA-approved treatments for cutaneous T-cell lymphoma?
Other Lymphomas and Leukemias
»	Outline the Ann Arbor clinical staging system for Hodgkin’s disease.
»	What is a Reed-Sternberg cell?
»	What are the histologic classes of Hodgkin’s disease?
»	Does Hodgkin’s disease occur in the skin?
»	How are cells immunophenotyped? What does the CD nomenclature mean?
»	What is lymphomatoid papulosis?
»	Are CD30-positive cells specific for lymphomatoid papulosis?
»	What is HTLV-1 virus? What is its significance?
»	Can multiple myeloma present with skin lesions only?
»	What is pseudolymphoma of the skin?
»	Can B-cell lymphomas present with skin lesions?
»	What is the most common type of leukemia in adults?
»	Can leukemia present with specific skin lesions?
»	What are some nonspecific skin lesions seen in patients with leukemia?

Describe the three subtypes of mycosis fungoides.

Fig. 46.4 A patient with Sézary syndrome.
Sézary syndrome (Fig. 46-4) presents with the classic triad of erythroderma, lymphadenopathy, and atypical circulating mononuclear cells (Sézary cells). These cells are moderately large mononuclear cells with hyperconvoluted nuclei. They resemble activated T cells, and when >15% of circulating lymphocytes are atypical, it is considered significant, with 10% to 15% being considered borderline. However, the finding of circulating Sézary cells must be evaluated in context with the clinical picture and skin biopsy. Severe pruritus, ectropion, nail dystrophy, peripheral edema, alopecia, and keratoderma of the palms and soles are common associated features. The disease tends to wax and wane and generally progresses faster and is more resistant to treatment than typical mycosis fungoides.
Pagetoid reticulosis (Woringer-Kolopp disease) is characterized by single or grouped hyperkeratotic skin lesion(s). Skin biopsy shows striking epidermotropism, with numerous atypical mononuclear cells, both singly and in clusters, scattered through all levels of the epidermis. The disease tends to be slowly progressive and responds well to local radiation.
The tumor d’emblee form was initially thought to be a type of mycosis fungoides that began with skin tumors without the usual progression through a patch-and-plaque stage. Recent reports suggest that some of these cases are B-cell primary cutaneous lymphomas and some represent Ki-1–positive primary cutaneous T-cell lymphomas.