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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...
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