Clinical Characteristics and Disease Course in Black Patients With Lymphomatoid Papulosis: A Case Series

January 2020 | Volume 19 | Issue 1 | Case Reports | 89 | Copyright © January 2020

Shamir Geller MD,a,b Sarah J. Noor MD,a Patricia L. Myskowski MDa

aDermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medicine, New York, NY bDepartment of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel

Abstract
INTRODUCTION: Lymphomatoid papulosis (LyP) is a CD30+ T-cell lymphoproliferative disorder (LPD) presenting as a recurrent eruption of papules and nodules which resolve spontaneously. CD30+ LPD prevalence in African American (AA)/Black patients is lower compared to White patients. CD30+ LPD has been recently reported to have worse outcomes in AA patients compared to White patients.

METHODS: A retrospective chart review identified eight AA patients with LyP. We describe our experience with these eight patients and review the literature on similar cases.

RESULTS: In half of the eight included patients, lesions occurred 1-4 years before they were diagnosed. In six patients (75%), resolution of the lesions resulted in hyperpigmented macules and scars. Five patients (63%) had also mycosis fungoides. Most of the patients who were followed (4/7, 57%) did not have complete resolution at their last visit, despite different treatment approaches. Discussion: Our results highlight that although LyP has an indolent course in AA/Black patients, residual hyperpigmentation and scars frequently occur, highlighting the need for better treatments of this lymphoproliferative disorder in this specific population.

J Drugs Dermatol. 2020;19(1): doi:10.36849/JDD.2020.4602

INTRODUCTION

Lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (ALCL) comprise a disease spectrum known as primary cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ LPD).1 CD30+ LPD is the second most common group of cutaneous T-cell lymphomas (CTCL) after mycosis fungoides (MF) and is regarded as an indolent lymphoproliferative disorder1 LyP typically manifests clinically as a recurrent eruption of papules and nodules with variable central ulceration, scale or crust that resolve spontaneously within several weeks and may result in hyperpigmentation, hypopigmentation, and scarring. LyP histology is usually characterized by wedge-shaped cellular infiltrate that contains atypical large CD30+ lymphocytes.1 LyP is uncommon in Black patients and have been described in a few case reports.2 Analysis of two separate collaborative cancer databases in the united states showed that CD30+ LPD is mainly diagnosed in Caucasian patients and only rarely in African American (AA)/Black patients. AA/Blacks comprised only 7%-8%3,4 and 10%5 of all CD30+ LPD patients included in these two large databases. A recent study of prognostic factors in CD30+ LPD patients with an emphasis on outcomes among ethnic groups, showed that AA patients have a significantly poorer overall survival than Caucasians.5 These studies did not differentiate LyP from other CD30+ LPD cases and did not elaborate on the symptoms and clinical features. Therefore, these findings and recommendations are difficult to interpret in routine clinical practice. We herein report on the clinical characteristics and disease course of LyP in a series of eight AA/Black patients from our institution and three previously published case reports.

REPORT

Eight AA/Black patients with a confirmed diagnosis of LyP were identified in a retrospective search of the tumor registry and pathology database at Memorial Sloan Kettering Cancer (Table 1). Five males and three females presented to us between the years 2000-2017 with a median age of 42 (range of 28-75 years). Six patients were non-hispanic, one hispanic and ethnicity was unknown in one patient. Five patients (63%) were also diagnosed with MF either before, at the same time, or after their LyP diagnosis. In half of the patients, lesions had occurred 1-4 years before the patients were diagnosed and presented to us. In one case, a diagnosis of LyP was made three years earlier; and the rest of the patients had an onset of less than one year prior to diagnosis. On physical examination at presentation, all patients had multiple lesions; however, one patient presented with one active lesion and few scars from past lesions, and one patient had only hyperpigmented macules and scars. In six (75%) of the patients, resolution of