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The incidence of primary cutaneous B-cell lymphomas (PCBCL) in the pediatric population is unknown, and the information on pediatric PCBCL has mostly been gathered from individual case reports or series from single centers. To assess the incidence of PCBCL in the pediatric population (<20 years) compared with adult patients (≥20 years) and delineate its demographic and clinicopathologic characteristics. This was a population-based, retrospective cohort study of patients in 18 cancer registries in the United States diagnosed between 2000 to 2016 through the Surveillance, Epidemiology, and End Results (SEER) program. Age-adjusted incidence rates were calculated for PCBCL in pediatric and adult populations. Demographic, clinical, and pathological characteristics were compared between the two groups. 5,128 adult and 48 pediatric PCBCL cases were included. The median age at diagnosis in the pediatric group was 16.5 years. The major histologic subtypes of pediatric cases were marginal zone lymphoma (77.1%), diffuse large B-cell lymphoma (12.5%) and follicle center lymphoma (10.4%), which significantly differed from the distribution in the adult group (p<0.001) where subtypes were equally distributed. The age-adjusted pediatric PCBCL incidence rate (per 1,000,000 person‐years) was 0.12 (95% CI 0.09-0.16) and incidence in the adult population was approximately 40-fold higher (IRR 41.4, 95% CI 31.2-56.2, P<0.001). All 48 pediatric cases were alive during a median follow-up time of 48 months. Pediatric PCBCL is a rare and indolent disease, which affects mostly adolescents of both sexes. Clinical presentation is similar to adults, but marginal zone subtype is significantly over-represented. [ABSTRACT FROM AUTHOR]

Background: Inherited ichthyoses belong to a large, clinically and etiologically heterogeneous group of mendelian disorders of cornification, typically involving the entire integument. Over the recent years, much progress has been made defining their molecular causes. However, there is no internationally accepted classification and terminology., Objective: We sought to establish a consensus for the nomenclature and classification of inherited ichthyoses., Methods: The classification project started at the First World Conference on Ichthyosis in 2007. A large international network of expert clinicians, skin pathologists, and geneticists entertained an interactive dialogue over 2 years, eventually leading to the First Ichthyosis Consensus Conference held in Sorèze, France, on January 23 and 24, 2009, where subcommittees on different issues proposed terminology that was debated until consensus was reached., Results: It was agreed that currently the nosology should remain clinically based. "Syndromic" versus "nonsyndromic" forms provide a useful major subdivision. Several clinical terms and controversial disease names have been redefined: eg, the group caused by keratin mutations is referred to by the umbrella term, "keratinopathic ichthyosis"-under which are included epidermolytic ichthyosis, superficial epidermolytic ichthyosis, and ichthyosis Curth-Macklin. "Autosomal recessive congenital ichthyosis" is proposed as an umbrella term for the harlequin ichthyosis, lamellar ichthyosis, and the congenital ichthyosiform erythroderma group., Limitations: As more becomes known about these diseases in the future, modifications will be needed., Conclusion: We have achieved an international consensus for the classification of inherited ichthyosis that should be useful for all clinicians and can serve as reference point for future research., (Copyright © 2010 American Academy of Dermatology, Inc. All rights reserved.)