Pediatric Rosai-Dorfman-Destombes Disease: From Isolated Cervical Lymphadenopathy to Multisystem Disease

Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis with a broad clinical spectrum, ranging from localized lymphadenopathy to severe multisystem involvement.Due to its rarity, clinical data on pediatric RDD remain limited, particularly in North Africa.We report two contrasting pediatric cases of RDD managed at a tertiary center in Morocco to illustrate this clinical heterogeneity.The first was that of a five-year-old girl who presented with chronic, isolated, painless cervical lymphadenopathy.Laboratory findings were normal.Biopsy confirmed RDD with classic emperipolesis and an S100+, CD163+, CD68+, CD1a-immunophenotype. Targeted molecular analysis showed no mutations in KRAS, NRAS, or BRAF exon 15.Active surveillance led to progressive spontaneous regression.The second case was of a 20-month-old boy initially misdiagnosed with osteomyelitis due to painful bone lesions.He rapidly developed severe multisystem disease with axillary lymphadenopathy, respiratory distress secondary to pulmonary nodules, hepatomegaly, renal lesions, and widespread osteolytic destruction.Laboratory testing revealed a marked inflammatory syndrome, anemia, and polyclonal hypergammaglobulinemia.Subsequent lymph node biopsy established the diagnosis of RDD.Treatment with vinblastine and prednisone achieved long-term complete remission, with persistent bone deformity as the sole sequela.Pediatric RDD poses a significant diagnostic challenge due to its marked clinical variability.Histopathological confirmation showing emperipolesis and characteristic immunophenotyping is essential.Isolated nodal forms carry a favorable prognosis and can be managed conservatively, whereas severe multisystem disease requires prompt systemic therapy.