April 2009 – Case of the Month #2
This is our second Case of the Month. These cases can also be accessed by clicking on the Case of the Month button on the left hand side of our Home Page. Please note that to view the images, you must click on the links in blue.
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We have extended the deadline of our contest / drawing, which is open to all dermatologists, dermatology residents or dermatology fellows. We will select 1 name at random from our Dermatologist Job / Practices email list as of 30 April 2009, and send them $50 by check or PayPal. To enter the drawing, send an email to NatPernick@Hotmail.com with “send me Derm jobs email” in the subject line.
This case was contributed by Dr. David S. Brenner, Assistant Medical Director, Department of Pathology and Director, Division of Microbiology, Bayhealth Medical Center in Dover, Delaware (USA). We invite you to contribute a Case of the Month by sending microscopic images in JPG or GIF format, with a clinical history and any other images (gross, dermoscopy, immunostains, etc.) that may be helpful, to NatPernick@Hotmail.com. We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US) after we send out the case. Please only send cases with a definitive diagnosis.
Dermatology Case of the Month #2
Clinical History
A 20
year old woman presented for excision of an apparent supernumerary nipple on
her left breast.
Micro images: low power;
medium
power; high power
#1; #2
What is your diagnosis?
(scroll down to continue)
Diagnosis
Juvenile
xanthogranuloma
Discussion
The dermis contains a fibrohistiocytic lesion with lymphocytes and numerous Touton giant cells. No epidermal involvement is noted. There are no mitotic figures, and only occasional mild nuclear atypia, which appears to be degenerative.
Juvenile xanthogranuloma is an uncommon (< 0.5% of pediatric tumors), benign fibrohistiocytic lesion of infants or young adults. It often appears in the skin of the face or trunk. Some lesions involute spontaneously, leaving a depressed and variably hyperpigmented area of skin.
Cutaneous lesions are usually small, from 0.1 to 2.0 cm, yellow-red and papulonodular. They contain a dense dermal infiltrate of lymphocytes, histiocytes, Touton giant cells (usually), eosinophils and neutrophils, which may extend into the subcutis. Over time, the epidermis thins out and the rete ridges become elongated. Extracutaneous lesions may lack the Touton giant cells.
Note: histiocytic giant cells may be Touton type - ring of nuclei surround foamy cytoplasm with cytoplasm usually also visible around the nuclei; Langhans type - nuclei form a horseshoe arrangement, not necessary a distinct category from Touton type, or foreign-body type - haphazard nuclear arrangement.
Immunostains are usually not necessary for diagnosis. The Touton giant cells and histiocytes are CD68+, HAM56+ and Factor XIIIa+. They are negative for S100 and CD1a (AJSP 2003;27:579).
The differential diagnosis includes Langerhans cell histiocytosis, a more common cutaneous disorder of childhood. These tumor cells have coffee bean nuclei (nuclear grooves) which are S100+ and CD1a+, and negative for CD68, HAM56 and Factor XIIIa. No Touton giant cells are present. Electron microscopy shows characteristic Birbeck granules. Xanthomas contain a uniform collection of foam cells and variable Touton giant cells, but lack the other inflammatory cells of juvenile xanthogranuloma. They are often associated with hyperlipidemia. Benign fibrous histiocytoma usually has a storiform pattern.
Treatment of juvenile xanthogranuloma is conservative excision, with a relapse rate of 7% (AJSP 2005;29:21). Newborns may develop systemic disease requiring chemotherapy or other systemic treatment (Pediatr Blood Cancer 2008;51:130, Pediatr Dermatol 2008;25:470).
Nat
Pernick, M.D., President
DermatologyOutlines.com
30100 Telegraph Road, Suite 408
Bingham Farms, Michigan (USA) 48025
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Email: NatPernick@hotmail.com