A doctor may diagnose the JXG during a physical exam.
A JXG can look very similar to other skin conditions.
Therefore, a biopsy may be done.
This means one of the bumps (or a piece of one) is removed from your child's skin to be looked at under a microscope..
Introduction.
Juvenile xanthogranuloma (JXG) is a rare benign disorder.
It is the most common form of non-Langerhans cell histiocytosis (LCH)1, 2 and specifically belongs to the C group (cutaneous and mucocutaneous histiocytoses)..
Histologically, the lesions are comprised of macrophages, xanthomatous cells (macrophages containing lipid), multinucleated histiocytes with lipid at the periphery (Touton giant cells), and scattered lymphocytes and eosinophils..
It is characterized by xanthogranuloma which is composed of lipid laden foamy macrophages, lymphocytes, plasma cells with variable number of foreign body giant cells that leads to destruction of tissue of the affected organ [1]..
A juvenile xanthogranuloma (zan tho grahn you LOH mah), or JXG, is a benign skin lump or bump caused by a collection of cells called histiocytes.
These may be red, orange or tan at first, but over time may become more yellow in color.
These bumps usually appear on the head, neck and trunk..
Usually in children and infants, sometimes in adults.
Rosai-Dorfman disease - Emperipolesis.
Necrobiotic xanthogranuloma - also may have Touton giant cells.
Langerhans cell markers:
-- both should be negative
If Touton giant cells are absent -- this is essential.
Histiocyte markers:CD68, CD163 -- both should be positive.
Features:.
1) Dermal histiocytes: 1.1.
Abundant cytoplasm - may not be xanthomatous/foam cells. 2. +/-Touton giant cell - key feature. 2.1.
Large multi-nucleated cells where nuclei are distributed at the cell periphery.
Notes:.
1) Must prove they are non-Langerhans cell histiocytes, esp. if no Touton giant cells.
DDx:.
1) Langerhans cell histiocytosis.
The histologic findings are very similar to those of xanthogranulomatous cholecystitis; however, histiocytes in malakoplakia are typically positive for PAS, while those in xanthogranulomoatus cholecystitis are negative.
A 63 year old patient visited the hospital with upper quadrant abdominal pain.
The pathognomonic cells in xanthogranulomatous pyelonephritis are CD68 positive foam cells (xanthoma cells).
Answer B is incorrect because xanthoma cells are not epithelial cells.
Answer C is incorrect because inflammatory cells, such as:
Juvenile xanthogranulomas occur most commonly in the skin and show a characteristic morphology.
The lesions exhibit a well-demarcated, dense invasion of numerous histiocytes within the superficial dermis and blunting of the rete ridges along with scattered lymphocytes, plasma cells and eosinophils.
