Bone Involvement in Rosai-Dorfman Disease (RDD): a Case Report and Systematic Literature Review

Abstract

Purpose of review: Rosai-Dorfman disease (RDD) is a rare histiocytic disorder typically presenting as painless cervical lymphadenopathy. Extranodal involvement is common and may also affect bones. Here, we present a patient with typical nodal disease and multifocal bone manifestations. Further, a systematic literature review was performed to better understand the phenotype, clinical course and treatment options of such patients.

Recent findings: RDD is a nonmalignant, classically sporadic histiocytosis. Nevertheless, increasing evidence also suggests familial forms of the disease. According to our literature review, bone involvement is exceedingly rare and heterogeneous. Clinical outcome in terms of mortality seems to be favorable in most cases. Currently, therapy strategies include surgical and immunosuppressive treatments, but the optimal treatment of osseous RDD remains to be defined. Patients with osseous RDD may present to rheumatologists with arthralgia or arthritis. Due to the rarity of the disease, diagnosis and treatment remain challenging.

Keywords: Bone involvement; Rosai-Dorfman disease; Treatment strategies.

Fig. 1

Bone involvement in Rosai-Dorfman disease (RDD). X-ray of the hands shows a lytic lesion (arrow) of the left distal radius (a). MRI demonstrated an additional mass lesion (dotted arrow) involving the proximal part of the right second phalanx with soft tissue extension (b)

Fig. 2

Distribution of bone manifestations in reported RDD patients (n = 108)

Fig. 3

Distribution of extraosseus involvement (n = 80) in reported RDD patients