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

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State: Public
Version: Final published version
License: CC BY-NC 4.0
Serval ID
serval:BIB_4B285B4FA583
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Paediatric-onset lymphomatoid papulosis: results of a multicentre retrospective cohort study on behalf of the EORTC Cutaneous Lymphoma Tumours Group (CLTG).
Journal
The British journal of dermatology
Author(s)
Blanchard M., Morren M.A., Busschots A.M., Hauben E., Alberti-Violetti S., Berti E., Avallone G., Tavoletti G., Panzone M., Quaglino P., Colonna C., Melchers R.C., Vermeer M.H., Gniadecki R., Mitteldorf C., Gosmann J., Stadler R., Jonak C., Oren-Shabtai M., Hodak E., Friedland R., Gordon E., Geskin L.J., Scarisbrick J.J., Mayo Martínez F., Noguera Morel L., Pehr K., Amarov B., Faouzi M., Nicolay J.P., Kempf W., Blanchard G., Guenova E.
ISSN
1365-2133 (Electronic)
ISSN-L
0007-0963
Publication state
Published
Issued date
16/07/2024
Peer-reviewed
Oui
Volume
191
Number
2
Pages
233-242
Language
english
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
Abstract
Lymphomatoid papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population are scarce.
To characterize the epidemiological, clinical, histopathological and prognostic features of paediatric LyP.
This was a retrospective multicentre international cohort study that included 87 children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years at disease onset were included. LyP diagnosis was made in each centre, based on clinicopathological correlation.
Eighty-seven patients from 12 centres were included. Mean age at disease onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2 : 1. Mean time between the onset of the first cutaneous lesions and diagnosis was 1.3 years (range 0-14). Initial misdiagnosis concerned 26% of patients. LyP was most often misdiagnosed as pityriasis lichenoides et varioliformis acuta, insect bites or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned in 21% of patients. The main histological subtype was type A in 55% of cases. When analysed, monoclonal T-cell receptor rearrangement was found in 77% of 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. Associated haematological malignancy (HM) occurred in 10% of cases (n = 7/73), including four patients with mycosis fungoides, one with primary cutaneous anaplastic large cell lymphoma (ALCL), one with systemic ALCL and one with acute myeloid leukaemia. If we compared incidence rates of cancer with the world population aged 0-19 years from 2001 to 2010, we estimated a significantly higher risk of associated malignancy in general, occurring before the age of 19 years (incidence rate ratio 87.49, 95% confidence interval 86.01-88.99).
We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall, the disease prognosis is good, with excellent survival rates for all patients. Owing to an increased risk of associated HM, long-term follow-up should be recommended for patients with LyP.
Keywords
Humans, Lymphomatoid Papulosis/pathology, Lymphomatoid Papulosis/epidemiology, Male, Retrospective Studies, Child, Female, Adolescent, Child, Preschool, Infant, Skin Neoplasms/pathology, Skin Neoplasms/epidemiology, Skin Neoplasms/mortality, Age of Onset, Prognosis, Diagnostic Errors/statistics & numerical data, Pityriasis Lichenoides/epidemiology, Pityriasis Lichenoides/pathology, Pityriasis Lichenoides/diagnosis, Insect Bites and Stings/epidemiology, Insect Bites and Stings/complications, Molluscum Contagiosum/epidemiology, Molluscum Contagiosum/pathology, Molluscum Contagiosum/diagnosis
Pubmed
Web of science
Open Access
Yes
Create date
12/04/2024 10:56
Last modification date
20/07/2024 7:11
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