Paediatric onset lymphomatoid papulosis: results of a multicentre retrospective cohort study, on behalf of the EORTC Cutaneous Lymphoma Tumours Group (CLTG)

Maël Blanchard; Marie-Anne Morren; Anne-Marie Busschots; Esther Hauben; Silvia Alberti-Violetti; Emilio Berti; Gianluca Avallone; Gianluca Tavoletti; Michele Panzone; Pietro Quaglino; Cristiana Colonna; Rutger C Melchers; Maarten H Vermeer; Robert Gniadecki; Christina Mitteldorf; Janika Gosmann; Rudolf Stadler; Constanze Jonak; Meital Oren-Shabtai; Emmilia Hodak; Rivka Friedland; Emily Gordon; Larisa J Geskin; Julia J Scarisbrick; Fatima Mayo Martínez; Lucero Noguera Morel; Kevin Pehr; Boyko Amarov; Mohamed Faouzi; Jan P Nicolay; Werner Kempf; Gabriela Blanchard; Emmanuella Guenova

doi:10.1093/bjd/ljae150

Paediatric onset lymphomatoid papulosis: results of a multicentre retrospective cohort study, on behalf of the EORTC Cutaneous Lymphoma Tumours Group (CLTG)

Br J Dermatol. 2024 Apr 10:ljae150. doi: 10.1093/bjd/ljae150. Online ahead of print.

Authors

Maël Blanchard^{1

2}, Marie-Anne Morren¹, Anne-Marie Busschots³, Esther Hauben³, Silvia Alberti-Violetti^{4

5}, Emilio Berti⁴, Gianluca Avallone^{4

5

6}, Gianluca Tavoletti^{4

5}, Michele Panzone⁶, Pietro Quaglino⁶, Cristiana Colonna⁷, Rutger C Melchers⁸, Maarten H Vermeer⁸, Robert Gniadecki⁹, Christina Mitteldorf¹⁰, Janika Gosmann¹¹, Rudolf Stadler¹¹, Constanze Jonak¹², Meital Oren-Shabtai¹³, Emmilia Hodak¹³, Rivka Friedland¹⁴, Emily Gordon¹⁵, Larisa J Geskin¹⁵, Julia J Scarisbrick¹⁶, Fatima Mayo Martínez¹⁷, Lucero Noguera Morel¹⁷, Kevin Pehr¹⁸, Boyko Amarov¹⁹, Mohamed Faouzi²⁰, Jan P Nicolay²¹, Werner Kempf^{1

2}, Gabriela Blanchard^{1

2}, Emmanuella Guenova¹

Affiliations

¹ Department of Dermatology, CHUV Lausanne, University of Lausanne, Switzerland.
² Division of Dermatology and Venereology, Geneva University Hospitals, Geneva, Switzerland.
³ University Hospitals Leuven, Leuven, Belgium.
⁴ Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
⁵ Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
⁶ Department of Medical Sciences, University of Turin, Dermatology Clinic, Turin, Italy.
⁷ Pediatric Dermatology Unit, dept of clinical sciences and community health, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
⁸ Department of Dermatology, Leiden University Medical Center, Leiden, Netherlands.
⁹ Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.
¹⁰ Department of Dermatology, Venereology and Allergology, University Medical Center, Göttingen, Germany.
¹¹ University Clinic for Dermatology, Johannes Wesling Medical Centre, UKRUB, University of Bochum, Minden, Germany.
¹² Medical University of Vienna, Division of General Dermatology, Vienna, Austria.
¹³ Division of Dermatology, Rabin Medical Center, Beilinson Hospital, and Sackler Faculty of Medicine, Tel Aviv University, Israel.
¹⁴ Pediatric Dermatology Unit, Schneider Children's Medical Center, and Sackler Faculty of medicine, Tel Aviv University Israel.
¹⁵ Department of Dermatology, Columbia University, New York, NY, USA.
¹⁶ Department of Dermatology, University Hospital Birmingham, Birmingham, United Kingdom.
¹⁷ Department of Dermatology, University Children's Hospital Niño Jesús, Madrid, Spain.
¹⁸ Division of Dermatology, Department of Medicine, Jewish General Hospital, McGill University, Montreal, Quebec, Canada.
¹⁹ Institute of Statistics and Econometrics, Sofia University "St. Kliment Ohridski", Faculty of Economics and Business Administration, 1113 Sofia, Bulgaria.
²⁰ Centre for Primary Care and Public Health (Unisanté), University of Lausanne, Switzerland.
²¹ Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, University of Heidelberg, Mannheim, Germany.

PMID: 38595050
DOI: 10.1093/bjd/ljae150

Abstract

Background: Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce.

Objectives: To characterize epidemiological, clinical, histopathological, and prognostic features of paediatric LyP.

Methods: This was a retrospective, multicentre international cohort study including 87 cases of children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years old at disease onset were included. Diagnosis was made in each centre based on clinical-pathological correlation.

Results: Eighty-seven patients from 12 centres were included. The mean age at onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2:1. The mean time between onset of first cutaneous lesions and diagnosis was 1.3 years (range 0-14 years). Initial misdiagnosis concerned 26.4% of patients. Initially, LyP was most often misdiagnosed as Pityriasis lichenoides et varioliformis acuta (PLEVA), insect bites, or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned for 20.7% of patients. The main histological subtype was type A in 55.1% of the cases. If analysed, monoclonal TCR rearrangement was found in 76.5% of the skin biopsies. The overall survival rate was 100% with follow up at 5 years available for 33 patients and at 15 years for 8 patients. A development of associated haematological malignancy (HM) occurred in 9.6% of the cases (7/73), including four mycosis fungoides (MF) cases, one primary cutaneous anaplastic large cell lymphoma (pc-ALCL), one systemic ALCL and one case of acute myeloid leukaemia. If we compare incidence rates of cancer with the world 0-19 years old population from 2001-2010, we estimate a significantly higher risk of associated malignancy in general, occurring before the age of 19 years old with incidence rate ratio of 87.49 (CI 86.01-88.99).

Conclusions: We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall the prognosis of the disease is good, with excellent survival rates for all patients. Due to increased risk of associated HM, a long-term follow-up should be recommended for LyP patients.