Background: Lymphomatoid papulosis (LyP) is a CD30(+) lymphoproliferative skin disease that has been described in association with Hodgkin lymphoma. It has also been reported to progress to mycosis fungoides or cutaneous anaplastic large-cell lymphoma. Objective: To study the clinical and histologic features of LyP and response to treatment in a patient series. Materials and methods: For this retrospective, descriptive, observational study of patients with histologically confirmed LyP and sufficient follow-up data on record, we extracted histologic findings on skin biopsy, clinical presentation, clinical course, and response to treatments. Results: Eighteen patients (10 male, 8 female) were identified. Most biopsies (14/18, 78%) showed a wedge-shaped lymphocytic infiltrate with CD30(+), CD3(+), and CD56(-) cells. A type A histologic pattern was present in the biopsies of 83% of the patients. The most common presentation (83%) consisted of papules on the trunk; for 62% LyP resolved after a single episode. Twelve percent of the patients developed mycosis fungoides (mean follow-up, 7 years); no other associations were noted. Discussion: Although few series of patients with LyP have been published in recent years, the findings reported generally coincide with our observations. Conclusion: LyP is typically a CD30(+) lymphoproliferative disorder that usually runs a benign course and responds well to treatment. (C) 2011 Elsevier Espana, S.L. and AEDV. All rights reserved.