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LYMPHOMATOID PAPULOSIS

LYMPHOMATOID PAPULOSIS

A 45 year-old white male with a history of hand dermatitis presented with a 1-month history of an asymptomatic eruption on his trunk and chin. He denied fever, chills, upper respiratory symptoms or arthralgias. Upon examination, he was a well-developed, white male with purple-red indurated papules and nodules on his trunk and proximal upper extremities through his neck (figure 1). One of the papules was ulcerated (figure 2). His palms, soles and face were clear. DIAGNOSING THE CONDITION A diagnosis of lymphomatoid papulosis requires histologic and sometimes immunoflourescent studies. Three types of the disease have been categorized: - Type A: classified by the presence of large non-epidermotropic atypical cells with abundant cytoplasm, prominent nuclei and surrounding inflammatory cells (histiocytes). This type has a higher correlation to the development of lymphoma than Type B. - Type B: characterized by smaller epidermotropic atypical cells with cerebriform, hyperchromatic nuclei. Tends to be perivascular or bandlike in distribution. Types A and B may have an abundance of mitotic figures. - Type C: classified by presence of large clusters of large atypical CD30 and T cells, and few inflammatory cells. It’s estimated that lymphomatoid papulosis progresses to lymphoma in 5% to 10% of individuals. Mycosis fungoides accounts for 40% of the lymphoma cases and CD 30 T-cell lymphomas comprises 30%. Hodgkin’s lymphoma is seen in 25%. ETIOLOGY Lymphomatoid papulosis is characterized by chronic, recurrent and self-limited papulonodular skin eruptions.1 It may occur at any age but is most frequently seen in 20- to 40-year-old adults. Systemic symptoms are typically minimal. The primary lesions consist of red papules measuring as large as 1 cm in diameter and may progress to vesicular, pustular, hemorrhagic or necrotic lesions. Multiple papules arise in various distributions and may be in different stages of development when evaluated. The lesions will typically spontaneously regress within 3 to 8 weeks, and often heal with a varioliform, hyperpigmented or hypopigmented scar in a “burned out” appearance. TREATMENT Topical clobetasol propionate may be effective for treatment of individual symptomatic lesions. Therapy for lymphomatoid papulosis may not be necessary as many patients’ symptoms resolve within 8 weeks. Symptomatic therapy is indicated for the systemic symptoms. PUVA, topical chemotherapy or low doses of methotrexate may suppress the disease flare but patients should be alerted to the fact that their disease may persist for up to 40 years.
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