Lymphomatoid papulosis in children and adolescents: A clinical and histopathologic retrospective cohort.

BACKGROUND, AIMS AND OBJECTIVES: Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative disorder that is rare and not well described within the pediatric subpopulation. We sought to review the literature and characterize clinical and pathologic features among pediatric and adolescent patients diagnosed with LyP at a tertiary care center.

MATERIALS AND METHODS: A retrospective cohort of 27 pediatric and adolescent patients (defined as <20 years old) diagnosed with LyP at the Weill Cornell Medicine Dermatopathology division from 2006 to 2016 was identified. Subsequently, we reviewed the histopathologic characteristics and collected clinical follow-up data from patients and their providers. The parameters assessed included the pathological LyP subtype including the immunohistochemical staining pattern, the development of secondary lymphoma, disease duration and rate of remission.

RESULTS: While type A was the most prevalent subtype, B and C subtypes were also frequently observed. CD8 predominance was a common finding, especially among type B LyP patients and those with eccrinotropic granulomatous features. None of the patients with clinical follow-up have developed secondary lymphoma, and some patients experienced remission of their disease.

CONCLUSION: While type A appears to be the dominant variant described in children, types B, C, and even the newly described variants E and F may occur more often than previously reported. Pediatric LyP may be more indolent than the adult variant, but close clinical follow-up is still warranted.