Multicentric reticulohistiocytosis in a 14-year-old girl

Multicentric reticulohistiocytosis is a rare multisystem disorder in which an infiltration of histiocytic cells causes papulonodular skin lesions and potentially a destructive polyarthritis. The active disease typically resolves spontaneously after 5-8 years, but the articular destruction can lead to permanent joint deformities. We present a case of multicentric reticulohistiocytosis in a 14-year-old girl. The number of papules on her hands decreased in number and her arthritic symptoms improved after 4 months of oral naproxen. Thirty months later her joint symptoms remained inactive and only six very small papules remained on her hands. Small flexor deformities were present in the distal interphalangeal joints of both index fingers. This case is an example of how multicentric reticulohistiocytosis can be a relatively stable and self-limited disease but still cause permanent joint deformities.