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Skin-Nonmelanocytic tumors
Multicentric reticulohistiocytosis
Authors: Kara Hamilton, M.S., and Nat Pernick, M.D., PathologyOutlines.com, Inc.
Revised: 19 December 2009, last major update December 2009
Copyright: (c) 2005-2009, PathologyOutlines.com, Inc.
Definition
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● Rare disorder of women ages 40-50, with widespread cutaneous papules and nodules, 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
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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)
● 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 Erythematous patches
articular surface 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 power High power
H&E, TRAP (fig B/D) PAS CD68 (fig A)
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+, and are associated with
Additional references
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End of Skin-Nonmelanocytic Tumors > Multicentric reticulohistiocytosis
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