Skin tumor - nonmelanocytic - Multicentric reticulohistiocytosis

Home Chapter Home Jobs Conferences Fellowships Books

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
=========================================================================

● 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
=========================================================================

● Formerly called lipoid dermatoarthritis

Epidemiology
=========================================================================

● Usually women 40-50 years

Etiology
=========================================================================

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

Clinical description
=========================================================================

● 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
=========================================================================

● 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
=========================================================================

● 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
=========================================================================

Fingers

Hands

Ear

Various images

Destruction of knee articular surface

Erythematous patches on back

Micro description
=========================================================================

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

Micro images
=========================================================================

Low power


Medium / high power


PAS

H&E, CD68 and vimentin

Positive stains
=========================================================================

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

Negative stains
=========================================================================

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

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+

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

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).