Home   Chapter Home   Jobs   Conferences   Fellowships   Books

 

 

 

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

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

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

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

● Formerly called lipoid dermatoarthritis

 

Epidemiology

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

● Usually women 40-50 years

 

Etiology

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

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

 

Clinical

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

● 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)

● 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

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

 

Figure 1                       

Fingers               Hands                                                              Ear                               Various images

 

 

                               

Destruction of knee                           Erythematous patches

articular surface                                on back

 

Micro description

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

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

 

Micro images

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

 

                     

Low power                                                     Medium power         High power

 

 

                               

H&E, TRAP (fig B/D)            PAS                                                                        CD68 (fig A)

 

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+, and are associated with

 

Additional references

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

eMedicine

 

End of Skin-Nonmelanocytic Tumors > Multicentric reticulohistiocytosis

 

 

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must also 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, (c) 2001-2009, PathologyOutlines.com, Inc.  Information from third parties may also be protected by copyright.  Please contact us at copyrightPathOut@gmail.com with any questions.