When I looked up the definition of this condition, I found "Juvenile xanthogranuloma is a form of histiocytosis, classified as "non-Langerhans cell histiocytosis",[1] or more specifically, "type 2".[2]".
When I then went to histocytosis, it lead me to 277.89 for "non-Langerthans cell".
However, when I checked the Coding Clinic I found this:
Year: 2000
Issue: First
Title: Juvenile xanthogranuloma
Body: Question:
A 9-year-old patient was recently admitted to our facility with complaints of numerous skin lesions. The attending physician diagnosed the patient as having juvenile xanthogranuloma. What is the correct ICD-9-CM code for juvenile xanthogranuloma?
Answer:
Assign code 216.X Benign neoplasm of skin, for juvenile xanthogranuloma, with the appropriate 4th digit to specify the site. This condition is not a systemic metabolic disorder and affected persons have normal lipid metabolism.
Juvenile xanthogranuloma is a benign cutaneous histiocytic proliferation. Clinically, the lesions are typically small. The histology of these lesions generally shows a dense dermal infiltrate consisting of lymphocytes, histiocytes, Touton giant cells, eosinophils, and neutrophils. The overlying epidermis is typically flattened. Ulceration is rare, but lesions may range from a few millimeters to 9 centimeters
Juvenile xanthogranuloma (JXG) is characteristically seen as a benign skin disorder in infants and young children and has a pronounced tendency to undergo spontaneous regression. Rarely, the skin lesions may be accompanied by intraocular or orbital involvement, but in contrast to Langerhans' cell histiocytosis, visceral, and bony involvement is rare.
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