Skin nonmelanocytic tumor

Lymphoma and related disorders (see also Lymphoma chapter)


Multicentric reticulohistiocytosis

Last author update: 1 August 2012
Last staff update: 15 July 2021

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PubMed search: multicentric reticulohistiocytosis [title] skin

Nat Pernick, M.D.
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Cite this page: Pernick N. Multicentric reticulohistiocytosis. website. Accessed September 26th, 2023.
Definition / general
  • Rare disorder of women ages 40 - 50, with widespread cutaneous papules and nodules (eMedicine)
  • Often associated with a destructive arthritis and internal malignancy
  • Tumor cells are histiocytes and multinucleated giant cells containing abundant eosinophilic cytoplasm with a "ground glass" appearance
  • Formerly called lipoid dermatoarthritis
  • Usually women 40 - 50 years
Clinical features
  • Skin lesions on the hands, especially at the base of the nails
  • Lesions may also be on the face, ears, arms, scalp or mucosal surfaces
  • "Coral beads" and vermicular erythematous lesions bordering nostrils are pathognomonic (J Eur Acad Dermatol Venereol 2001;15:524)
  • Lesions vary from small papules to lesions several centimeters across, and are usually skin colored, yellow or reddish brown
  • Recommended to screen patients for malignancy (Rheumatology 2008;47:1102), since accompanied by neoplasm in 28% of cases
  • May be a paraneoplastic process (J Am Acad Dermatol 1998;39:864), or association with neoplasm may be due to reporting bias (eMedicine)
  • Associated with destructive arthritis
Case reports
Clinical images

Images hosted on other servers:




Various images

Destruction of knee articular surface

Erythematous patches on back

Microscopic (histologic) description
  • Prominent oncocytic histiocytes and multinucleated giant cells with eosinophilic, “ground glass” cytoplasm
Microscopic (histologic) images

Case #153

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Medium / high power


Images hosted on other servers:

Low power

Medium / high power

H&E, CD68 and vimentin

Positive stains
Differential diagnosis
  • Solitary reticulohistiocytoma: younger age, solitary lesions usually not on digits or face (Am J Surg Pathol 2006;30:521)
  • Epithelioid fibrous histiocytoma: usually < 1 cm on extremities, usually no giant cells, primarily myofibroblastic, not histiocytic
  • Epithelioid sarcoma: deep seated tumor with markedly atypical cells that form granuloma-like clusters with central necrosis; tumor cells are EMA+, keratin+, CD68-
  • Granulomatous inflammation: well formed granulomas and prominent lymphocytes, no large epithelioid histiocytes with eosinophilic glassy cytoplasm
  • Histiocytic sarcoma: typically forms a large mass of epithelioid histiocytes with significant nuclear atypia and mitotic activity
  • Juvenile xanthogranuloma: usually children, has scattered Touton type histiocytic giant cells and numerous eosinophils, but large epithelioid histiocytes are not prominent
  • Rosai-Dorfman disease: associated with adenopathy; histiocytes are pleomorphic and S100+
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