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

BACKGROUND: Lymphomatoid papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population are scarce. OBJECTIVES: To characterize the epidemiological, clinical, histopathological and prognostic features of paediatric LyP. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Lymphomatoid papulosis is a very rare skin condition caused by an abnormal increase in white blood cells (called 'lymphocytes') in the skin. The condition rarely affects children, so most of the scientific data published about this disease focuses on adults. This study involved 12 academic dermatology centres in Europe, the Middle East and North America, and gathered data from about 87 children who presented with symptoms of lymphomatoid papulosis before the age of 19 years. The aim of this study was to better describe this disease in the paediatric population and discuss its treatment options and evolution. We found that the presentation of the disease in children is roughly the same as in adults. Safe and effective treatment options exist. The disease is not life threatening, but it requires investigation by a dermatologist, both to make a careful diagnosis and to monitor it as sometimes associated cancers that originate from blood cells can occur, mostly on the skin.

Encefalitis equina venezolana: perfil clínico epidemiológico de la epidemia ocurrida en 1995 / Equine encephalitis in Venezuela: a clinical epidemiological profile of the 1995 epidemic

La encefalitis equina venezolana ha venido presentándose en brotes episódicos en la Guajira Venezolana desde aprox. 1936. En 1938 se aisló el agente causal por vez primera de un caballo enfermo en Venezuela. El síndrome predominante es el de una enfermedad autolimitada semejante a la gripe, y apenas 4 por ciento de las personas infectadas, principalmente niños menores de 15 años, sufre encefalitis. La mortlidad de niños menores de cinco años con encefalitis es de 35 por ciento. Se describe y analiza el perfil clínico epidemiológico de la epidemia de Encefalitis Equina Venezolana en cinco Municipios (Maracaibo, Mara, Insular, Padilla, Páez, Miranda) del estado Zulia (Venezuela) ocurrida entre el 15 de julio y el 17 de octubre de 1995. Se revisaron los datos obtenidos del departamento de vigilancia epidemiológica del Estado Zulia, teniendo un total de 11.072 casos con diagnóstico clínico-epidemiológico de Encefalitis Equina Venezolana, en una población de 574.769 habitantes comprendida por los cincos municipios ya citados, con tasa de ataque de 1,92 por ciento, no hay diferencia significativa en el sexo, el grupo etario más afectado son los menores de 5 años ocupando más de la mitad de la mortalidad con 8 casos, incluyéndose en este grupo dos muertes en recién nacidos cuyas madres se enfermaron en el último trimestre del embarazo