Multicentric reticulohistiocytosis in children: two case reports and a literature review.
Chenxi Liu, Liping Wang, Hua Huang et al.
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Case in brief
The report describes two boys with multicentric reticulohistiocytosis (MRH), a disorder uncommon in children and liable to be mistaken for juvenile idiopathic arthritis. A 10.8-year-old presented with polyarthritis, coral-bead-like papules and subcutaneous nodules. A 14-year-old, previously diagnosed with systemic-onset JIA complicated by macrophage activation syndrome, had persistent atypical pruritic rashes despite conventional and biologic therapies. Skin biopsies confirmed MRH in both cases. Tofacitinib was reported to alleviate symptoms.
What made this case unusual
The diagnostic challenge was recognizing MRH in children, including in a boy whose prior JIA diagnosis and treatment had not accounted for persistent atypical skin lesions.
Diagnostic / clinical pearl
In a child with arthritis and atypical or coral-bead-like skin lesions, consider MRH and use skin biopsy to investigate; histopathology was key to confirmation in these cases.