Rosai-Dorfman disease presenting as mediastinal lymphadenopathy: case report and review of the literature

Paediatr Int Child Health. 2013 May;33(2):120-3. doi: 10.1179/2046905512Y.0000000025.

Abstract

A 6-year-old boy presented with recurrent pneumonia and diarrhoea for 3 years. He had extensive mediastinal lymphadenopathy and atelectasis with low serum levels of IgA, IgG and IgG3. An inguinal lymph node biopsy demonstrated histological and histochemical features of Rosai-Dorfman disease. There was emperipolesis and histiocytes were immune-positive for S100 proteins. He responded to corticosteroids and regular infusions of immunoglobulins.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adrenal Cortex Hormones / administration & dosage
  • Biopsy
  • Child
  • Histiocytosis, Sinus / diagnosis*
  • Histiocytosis, Sinus / drug therapy
  • Histiocytosis, Sinus / pathology*
  • Histocytochemistry
  • Humans
  • Immunoglobulins / administration & dosage
  • Immunohistochemistry
  • Immunologic Factors / administration & dosage
  • Lymphatic Diseases / drug therapy
  • Lymphatic Diseases / etiology*
  • Lymphatic Diseases / pathology*
  • Male
  • S100 Proteins / analysis

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulins
  • Immunologic Factors
  • S100 Proteins