ment and excision surgery of skin lesions were then performed, but the lesions soon reappeared. He was then sequentially treated with thalidomide (50 mg/day) along with a topical corticosteroid for 1 year, but the lesions continued to increase in size and number. After obtaining written informed consent from the patient's parents, we administered baricitinib therapy (1 mg/day). Four weeks into treatment with baricitinib therapy, the patient observed a decrease in the number of nodules, and no new lesions appeared on his body. At last follow-up, after 10 months of treatment with baricitinib, substantial regression of the lesions was observed (Figure 1, C, D, and F).Discussion | Progressive nodular histiocytosis is clinically characterized by hundreds of lesions of 2 types: superficial yellow papules measuring smaller than 10 mm and large dermal fibrous nodules that are several centimeters in diameter. Histopathologically, xanthomatous papules show infiltration of foamy macrophages and Touton giant cells, whereas nodular lesions represent the proliferation of spindle-shaped histiocytes and multinucleate giant cells. 2 Progressive nodular histiocytosis mimics juvenile xanthogranuloma microscopically but presents with disseminated lesions and a progressive course. 3 There are usually no observable changes in lipoprotein metabolism. 1 This patient showed no improvement after treatment with various therapies, but responded well to treatment with baricitinib without adverse effects, infection, or abnormal laboratory test results. The JAK1/2 inhibitor ruxolitinib showed robust efficacy in the treating hemophagocytic lymphohistiocytosis, 4 and the JAK1 inhibitor upadacitinib improved a case of severe multicentric reticulohistiocytosis, 5 indicating that JAK inhibitors can potentially affect histiocytosis. This case indicates that baricitinib may be a novel therapeutic option for PNHC. The mechanism underlying the association of treatment with baricitinib with PNHC outcomes remains unclear, but may involve reductions in cytokine production and suppression of inflammation induced by macrophages and lymphocytes. 6 More clinical studies and additional data are needed to further confirm the efficacy of and mechanism behind baricitinib in relieving PNHC.