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Skin - nonmelanocytic tumors

Other tumors of skin

Multicentric reticulohistiocytosis


Reviewers: Nat Pernick, M.D. (see Reviewers page)
Revised: 28 September 2012, last major update September 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● 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

Terminology
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● Formerly called lipoid dermatoarthritis

Epidemiology
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● Usually women 40-50 years

Etiology
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● Histiocytic origin (Am J Surg Pathol 1990;14:687)

Clinical description
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● 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 red-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
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● 6 year old girl (J Rheumatol 1998;25:794)
● 33 year old woman with generalized systemic involvement (Clin Exp Dermatol 2004;29:373)
● 34 year old woman with papular lesions on hands and face (Case of the Week #153)
● 61 year old man with associated liver carcinoma (Case Rep Dermatol 2012;4:163)
● Relapse of multicentric reticulohistiocytosis before relapse of associated neoplasm (Med Pediatr Oncol 1985;13:273)

Treatment and prognosis
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● No consistently reliable treatment (Ryumachi 1993;33:68)
● TNF inhibitors (Arch Dermatol 2008;144:1360), aminobisphosphonates (Arthritis Rheum 2003;48:3538) and immunosuppressive drugs (Dermatol Online J 2009;15:2) have been effective only in individual cases

Clinical images
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Fingers

   
Hands


Ear


Various images


Destruction of knee articular surface


Erythematous patches on back

Micro description
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● Prominent oncocytic histiocytes and multinucleated giant cells with eosinophilic, “ground-glass” cytoplasm

Micro images
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Low power

       
Medium / high power

   
PAS


H&E, CD68 and vimentin

Positive stains
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● Vimentin, CD68, CD45, PAS (Skinmed 2004;4:71)
● Also CD163, lysozyme

Negative stains
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● S100, CD1a, CD15, CD34, desmin, muscle-specific actin, Factor VIII

Differential diagnosis
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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+

End of Skin - nonmelanocytic tumors > Other tumors of skin > Multicentric reticulohistiocytosis


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