23 July 2009 – Case of the Week #153

 

This email is only sent to subscribers.  To subscribe or unsubscribe to this or our other email lists, email NatPernick@Hotmail.com, indicating subscribe or unsubscribe to [name of email].  Our email lists are: 

 

- Case of the Week - 3 weeks/month

- Pathologist/PhD jobs - biweekly

- Other laboratory jobs - biweekly

- Pathology fellowships - biweekly

- Pathology website news - monthly

- Pathology new books - monthly

- The Detroit College Promise - the scholarship for Detroiters that we sponsor (monthly)

 

For our DermatologyOutlines.com website, we have these email lists:

 

- Dermatologist jobs / Practice openings - monthly

- Dermatology fellowships - monthly

- Dermatology website news - monthly

- Dermatology new books - monthly

 

 

 

 

Covance Expands Cytokeratin Antibody Portfolio

 

Covance’s cytokeratin antibody portfolio now includes additional IVD labeled antibodies for IHC on formalin fixed paraffin embedded sections.  The addition of these antibodies adds to Covance’s growing portfolio of antibodies, detection chemistries and ancillaries for anatomic pathology.  

 

Visit our website at www.covance.com to view our cytokeratin portfolio or call 800.223.0796 for more information.

 

 

 

 Website News:

 

(1) We have updated our Mystery Case, on the right side of the Home Page below the banners.  We hope to update it every 1-2 months.

 

(2) We want to make it easy for you to fill all of your laboratory positions, so we have created a special low rate for advertising.  The cost is only $1000 (US dollars) for up to 5 ads posted within one year.  This means that you can post a new ad every 2-3 months for one low rate.  These ads include pathology assistants, blood bank technicians, medical technologists, managers and all positions associated with the lab, but not pathologists or PhD positions.  One ad can offer multiple positions.  Click here for more details.

 

 

To view the images or references in this Case of the Week, you must click on the links in blue.  Links in green are to journals with free full text-no registration.  You can also access these cases by visiting our Home Page, then click on the Case of the Week button on the left hand side.

 

Thanks to Dr. Mowafak Hamodat, Eastern Health of Newfoundland and Labrador, St. John's, Canada, for contributing this case.  To contribute a Case of the Week, email NatPernick@Hotmail.com with the clinical history, your diagnosis and high quality microscopic images (see www.webpathology.com for examples) in JPG, GIF or TIFF format (send as attachments, we will shrink if necessary).  Please include any other images (gross, immunostains, etc.) that may be helpful or interesting.  We will write the discussion (unless you want to), list you as the contributor, and send you $35 (US dollars) by check or PayPal for your time after we send out the case.  Please only send cases with high quality images (see www.webpathology.com for examples) and a diagnosis that is somewhat unusual (or a case with unusual features).

 

Case of the Week #153

 

Clinical History

 

A 34-year-old woman presented with a history of indurated papular lesions on her hands and face. The lesions were red-blue and located around the nail folds, on the dorsum of the fingers and around the mucosal surface on her face.

 

Micro images:

                                   

Low power                             Medium power                      High power

 

Immunohistochemistry:

                 

PAS low power                      PAS high power

 

What is your diagnosis?

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Diagnosis:

 

Multicentric reticulohistiocytosis

 

Discussion:

 

Multicentric reticulohistiocytosis (MRH) is a rare disorder of histiocytes (Am J Surg Pathol 1990;14:687) associated with destructive arthritis.  In 28% of cases, it presents with a neoplasm, and when malignancy is present, MRH precedes the development of cancer in 73% of cases (Med Pediatr Oncol 1985;13:273).  MRH may be a paraneoplastic process (J Am Acad Dermatol 1998;39:864), or the association may be simply due to reporting bias (eMedicine).

 

Multicentric reticulohistiocytosis typically affects women 40-50 years, but it has also been diagnosed in a 6 year old girl (J Rheumatol 1998;25:794).  It is characterized by skin lesions on the hands (clinical image), especially at the base of the nails.  Lesions may also appear on the face, ears, arms, scalp or mucosal surfaces.  The lesions vary from small papules to lesions several centimeters across, and are usually skin colored, yellow or red-brown.  

 

On histologic examination, there are prominent oncocytic histiocytes and multinucleated giant cells, which have eosinophilic, “ground-glass” cytoplasm (Dermatology 2007;214:268, Clin Exp Dermatol 2004;29:373).  The histiocytes and giant cells are positive for vimentin, CD68, CD45 and PAS, as well as CD163 and lysozyme.  They are negative for S100, desmin, muscle-specific actin, and CD34 (J Eur Acad Dermatol Venereol 2001;15:524, Skinmed 2004;4:71).  These findings are similar to those in solitary reticulohistiocytoma (Am J Surg Pathol 2006;30:521).

 

The differential diagnosis includes these other histiocytic disorders, which usually have a different clinical presentation:

 

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 emperipolesis, B cells and plasma cells

 

There is no consistently reliable treatment, although 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 in individual cases, but not consistently (Ryumachi 1993;33:68). 

 

Additional references:  Rheumatology 2008;47:1102, eMedicine

 

 

Nat Pernick, M.D., President and

Kara Hamilton, M.S., Assistant Medical Editor

PathologyOutlines.com, Inc.

30100 Telegraph Road, Suite 408

Bingham Farms, Michigan (USA) 48025

Telephone: 248/646-0325

Email: NatPernick@Hotmail.com

Alternate email: NatPernick@gmail.com