Multicentric Reticulohistiocytosis: A Case Report of an Atypical Presentation in a 2‐Year‐Old

Abstract

Multicentric reticulohistiocytosis (MRH) is a rare systemic inflammatory granulomatous disease marked by severe and often rapidly progressive polyarticular arthritis and cutaneous papulonodules. Initial clinical diagnosis may be difficult. We describe a 2-year-old girl presenting with pink dermal papules on the forehead, thighs, elbows, knees, and palms of the hands. Based on clinical findings and skin biopsy results, she was initially diagnosed with granuloma annulare. At 5 years of age, she developed arthritis, fatigue, and more widespread skin papules leading to the diagnosis of MRH. To our knowledge, this is the youngest individual with MRH yet described. We outline the timeline and unique features of her case and review the literature pertaining to MRH in children. Although rare, MRH can be permanently debilitating, making prompt diagnosis critical. A standardized approach to investigation and management needs to be developed.

Efficacy of Vinblastine and Prednisone in Multicentric Reticulohistiocytosis With Onset in Infancy

“…[1][2][3] It usually affects individuals in the fourth decade of life and is very rare in children. [2][3][4] To the best of our knowledge, ∼13 pediatric cases have been reported (Table 1), our case being the youngest of all with the earliest presentation at the age of 3 months.…”

“…There is no definite treatment protocol for MRH, and various drugs have been attempted with inconsistent results. 1,3 We tried vinblastine and prednisone in this case with good response, which has not been attempted before.…”

“…Clinically, patients present with cutaneous papulo-nodular lesions and progressive symmetric and erosive arthritis. 1 Review of literature showed ∼13 cases of childhood MRH, including the present study, defining its rarity in childhood. 1,2 To the best of our knowledge, the present case is the youngest in the literature with onset of disease in infancy.…”

“…1 Review of literature showed ∼13 cases of childhood MRH, including the present study, defining its rarity in childhood. 1,2 To the best of our knowledge, the present case is the youngest in the literature with onset of disease in infancy. MRH is often misdiagnosed due to its rarity and overlapping clinical and histologic features.…”

“…Characteristic histologic findings include infiltration of histiocytes and multinucleated giant cells with a homogeneous eosinophilic ground glass cytoplasm. [1][2][3][4] Early diagnosis and immediate treatment are important to prevent irreversible joint deformity. There is no definite treatment protocol for MRH, and various drugs have been attempted with inconsistent results.…”