This report describes the case of a patient with multicentric reticulohistiocytosis. Immunohistochemical analysis revealed prominent markers of monocyte/ macrophage origin, as well as the presence of tumor necrosis factor ␣, interleukin-1 (IL-1), and IL-12; the occurrence of the latter in this disease has not previously been reported. Clinical, laboratory, radiographic, and histologic findings in multicentric reticulohistiocytosis are reviewed. In addition, all published cases of multicentric reticulohistiocytosis which included reports of cytokine and immunohistochemical analysis are reviewed, and evidence for a monocyte/macrophage origin and role in disease pathogenesis is provided.Multicentric reticulohistiocytosis is a rare systemic disorder of unknown etiology, characterized by severe destructive arthritis, development of cutaneous nodules, and a significant association with malignancies. Histologic analysis of cutaneous and synovial lesions reveals multinucleated foreign body-type giant cells and smaller histiocytes. The presence of lysosomes and the giant cell's ability to phagocytose have led many to suspect a monocyte/macrophage origin of the multicentric reticulohistiocytosis cells (1-3). More recently, immunohistochemical studies have yielded conflicting findings, with some supporting either a lymphocyte or dermal dendrocyte origin and others still reporting a monocyte/macrophage origin (2-7).We report herein a case of multicentric reticulohistiocytosis in which detailed immunohistochemical data were gathered. The data further support the notion of a monocyte/macrophage origin of the multicentric reticulohistiocytosis cells. In addition, we review the literature on multicentric reticulohistiocytosis, with particular emphasis on immunohistochemical findings.
CASE REPORTClinical, laboratory, and radiographic findings. The patient, a 44-year-old African American woman, presented with a 2 1 ⁄2-month history of relatively painless symmetric deformity and swelling of her distal interphalangeal (DIP) joints. She recently had noted the onset of a pruritic skin eruption on her right ear and a nodular lesion on the fourth finger of her right hand. Her earlier medical history was significant for cervical atypia with human papillomavirus. Other earlier medical history included menorrhagia and dysmenorrhea, microcytic anemia, and hypertension. Family history was significant only for type 2 diabetes mellitus. There was no history of travel, toxin exposure, or illicit substance use, and the patient had no pets.Physical examination revealed a single nodule on the right fourth finger as well as nodules in a beaded appearance over the cuticles on the same hand. Papules with slight scale were noted in the right external ear canal. Musculoskeletal examination revealed pain and moderate swelling in the second DIP joints bilaterally and in the left fifth DIP joint. Results of the clinical examination were otherwise unremarkable.Laboratory studies disclosed a microcytic anemia and thrombocytosis (hematocrit 35%, mean corp...