Cutaneous rosai-dorfman disease and morphea: coincidence or association?

Am J Dermatopathol. 2009 Jul;31(5):487-9. doi: 10.1097/DAD.0b013e318196f883.

Abstract

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease. A third of patients with this disease have extranodal involvement affecting the skin. Of these individuals, only around 3% will have purely cutaneous Rosai-Dorfman disease, which is limited to skin manifestations without systemic involvement. Cutaneous (localized) scleroderma or morphea, on the other hand, is a more common disease that most often affects women of all ages. Both conditions have unknown etiologies. Presented here is a case of a 60-year-old white woman with cutaneous Rosai-Dorfman disease and coexisting morphea. Representative biopsies from both areas were performed: one showing a dermal S-100+ histiocytic infiltrate with emperipolesis and the other showing a deep perivascular and interstitial plasma cell infiltrate with dermal sclerosis and loss of perieccrine fat. A laboratory and radiologic workup revealed no evidence of systemic involvement by either entity. The diagnosis of coexisting cutaneous Rosai-Dorfman disease and morphea was established. To our knowledge, this is the first report of these 2 entities found simultaneously in 1 patient.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Anti-Inflammatory Agents / therapeutic use
  • Coronary Artery Disease / complications
  • Diabetes Mellitus, Type 2
  • Female
  • Histiocytosis, Sinus / complications*
  • Histiocytosis, Sinus / drug therapy
  • Histiocytosis, Sinus / pathology*
  • Humans
  • Hypertension / complications
  • Immunohistochemistry
  • Middle Aged
  • Scleroderma, Localized / complications*
  • Scleroderma, Localized / drug therapy
  • Scleroderma, Localized / metabolism
  • Scleroderma, Localized / pathology*
  • Triamcinolone / therapeutic use

Substances

  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents
  • Triamcinolone