Rosai-Dorfman disease

    Article Contributors: 
    Sean Klepper M.D.
    Artur Zembowicz M.D....

    Also known as: sinus histiocytosis with massive lymphadenopathy, cutaneous sinus histiocytosis

    Clinical Features:

    • Disease of unknown etiology which causes massive cervical lymphadenopathy with systemic symptoms
    • The skin is the most common extranodal site to be involved.
    • Skin-limited disease has been described.
    • Cutaneous lesions present as single or multiple erythematous papules, nodules or plaques.

    Histologic Features:

    • Diffuse or nodular dermal proliferation of histiocytes with large nucleoli, vesicular chromatin and prominent nucleoli.
    • Emperipolesis is frequently seen (histiocytes phagocytose other leukocytes).
    • Admixed with the histiocytic infiltrate are variable numbers of lymphocytes, plasma cells, neutrophils and sometimes small numbers of eosinophils.
    • In the center of the nodules of histiocytes there are dilated lymphatics filled with histiocytes.
    • Surrounding the nodules of histiocytes are lymphoid aggregates with germinal centers and thick-walled blood vessels surrounded by plasma cells.
    • The histiocytes are positive for S-100 and negative for CD1a, in contrast to Langerhan's cell histiocytosis.

    Cases associated with this book:

  • Rosai-Dorfman disease
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: Dermpedia Teaching Collection