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Rosai-Dorfman disease.

Madhumita Nandi1, Rakesh Kumar Mondal, Supratim Datta

  • 1Department of Pediatric Medicine, Institute of Post-Graduate Medical Education and Research, (IPGMER), Kolkata, India. madhumitabanik@rediffmail.com

Indian Journal of Pediatrics
|April 1, 2008
PubMed
Summary

Rosai-Dorfman disease, a rare condition, can manifest as cervical adenopathy or rare orbital involvement. Steroid treatment is crucial for managing severe symptoms like pressure, disfigurement, and fever.

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Area of Science:

  • Oncology
  • Pathology
  • Ophthalmology

Background:

  • Rosai-Dorfman disease (RDD) is a rare, benign proliferative disorder of unknown etiology.
  • It is characterized by the proliferation of histiocytes and plasma cells, often presenting with lymphadenopathy.

Observation:

  • This report details two cases of RDD.
  • Case 1 presented with typical cervical adenopathy.
  • Case 2 exhibited rare bilateral orbital involvement.

Findings:

  • Both patients required systemic corticosteroid therapy.
  • Treatment was necessitated by significant pressure symptoms, cosmetic disfigurement, and, in one case, recurrent high fevers.

Implications:

  • This highlights the diverse clinical presentations of RDD, including rare orbital manifestations.
  • Early diagnosis and prompt steroid treatment are vital for managing RDD complications and improving patient outcomes.
  • Further research into RDD pathogenesis and optimal therapeutic strategies is warranted.