Progressive nodular histiocytosis in a 9-year-old boy treated with cobimetinib

Pediatr Dermatol. 2022 Jan;39(1):115-118. doi: 10.1111/pde.14887. Epub 2021 Dec 21.

Abstract

Progressive nodular histiocytosis is a rare variant of non-Langerhans cell histiocytosis that affects the skin and mucous membranes and displays a progressive clinical course and poor response to treatment. We describe a case of severe progressive nodular histiocytosis harboring a KRAS p.G12S mutation in a 9-year-old boy, refractory to chemotherapy, who was successfully treated with the MEK inhibitor cobimetinib. This is the first report of the use of MEK inhibition for this histiocytosis subtype in a pediatric patient.

Keywords: cobimetinib; non-Langerhans histiocytosis; progressive nodular histiocytosis.

Publication types

  • Case Reports

MeSH terms

  • Azetidines*
  • Child
  • Histiocytosis* / diagnosis
  • Histiocytosis* / drug therapy
  • Histiocytosis, Langerhans-Cell*
  • Humans
  • Male
  • Piperidines / therapeutic use
  • Skin Diseases*

Substances

  • Azetidines
  • Piperidines
  • cobimetinib