Sean Klepper M.D.
Artur Zembowicz M.D....
Also known as: sinus histiocytosis with massive lymphadenopathy, cutaneous sinus histiocytosis
- 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.
- 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.