Lymphomatoid Papulosis

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Abstract
The concept of disorders with a benign clinical course but a malignant appearance on histopathologic examination is controversial. Lymphomatoid papulosis (LYP), characterized by spontaneously resolving papules and nodules with strikingly atypical lymphoid cells, belongs to these disorders and has been a mystery since its description by Macaulay in 1968.1 Previously believed to be an inflammatory process, it is now regarded as an indolent cutaneous lymphoma and is listed as such in the current European Organization for Research and Treatment of Cancer and World Health Organization classifications.2,3 Clinical lesions of LYP vary from papules and nodules to—less commonly—vesicles and pustules, and while individual lesions usually resolve within weeks or months, the disease may recur for decades. In a minority of cases, association with other lymphoproliferative disorders such as mycosis fungoides (MF), anaplastic large cell lymphoma (ALCL), and Hodgkin disease (HD) has been reported.4-6 Based on histopathologic findings, 3 types, A, B, and C, differing in cytological and architectural features, have been delineated.7 We present a retrospective study of 85 patients with LYP to redefine the clinicopathologic subtypes and variants of this disease.