Introduction
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with
massive lymphadenopathy, It is a benign disease of histiocytic
proliferation with no known etiology, it is thought to be due to immune
dysregulation or infection like HHV-6, HHV-8, parvovirus B19, EBV, CMV,
VZV, brucella, and klebsiella; however, there the evidence in
inconclusive (1). (2)
It presents with painless massive cervical lymphadenopathy [ nodal]
with fevers, But It can also involves other sites [ extra- nodal]
like brain, eyes, upper respiratory tract and skin(3). The peak incidence is in the second or third
decade, and predominant in males.
Common abnormalities in laboratory tests are increased erythrocyte
sedimentation rate (ESR), leukocytosis with neutrophilia, normocytic
anemia, and hypergammaglobulinemia. Histopathological examination
remains the mainstay of diagnosis – lymph nodes have massive sinusoidal
dilation, containing histiocytes positive for S-100 and CD68, and
negative for CD1a.
Rosai-Dorfman disease is seldom life-threatening disease
which commonly does not require therapy. There are no defined
therapeutic algorithms for its treatment. Due to the fact that in many
cases spontaneous regression is observed, usually the “watch and wait”
approach is used. Surgery and systemic (e.g. steroids or chemotherapy)
treatment is rarely required.(4)