Dermatologic Manifestations of Juvenile Xanthogranuloma Workup

Updated: Oct 17, 2019
  • Author: Sally H Monahan, MD; Chief Editor: William D James, MD  more...
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While the diagnosis is typically clinical in most cases, a skin biopsy may be performed, both for diagnosis and cosmesis. The specimen usually consists of a complete excision of the papule or nodule.


Histologic Findings

Histological examination of juvenile xanthogranuloma (JXG) demonstrates a variety of findings. A time-dependent progression exists in the development of the characteristic histological features of JXG, which correlates with the age of the lesion. Early biopsy specimens reveal a dense monomorphous histiocytic infiltrate in the dermis. Extension into subcutaneous tissue, fascia, and muscle occurs in approximately one third of cases. [26] Older lesions contain foam cells, Touton giant cells, and foreign body giant cells. A mixed cellular infiltrate of neutrophils, lymphocytes, eosinophils, and (rarely) mast cells may be noted. Old lesions demonstrate fibrosis. No histological difference is reported between cutaneous and systemic JXG. Because of the difficulty in diagnosing JXG and because of the transient presence of Touton giant cells in JXG lesions, these classic elements may not be present in every case.

The histiocytes contain pleomorphic nuclei, with few or absent mitotic figures, and irregular dense bodies. Clustered comma-shaped bodies occasionally are observed on electron microscopy but are not specific. Use of special stains is important to differentiate JXG from Langerhans and non–Langerhans cell histiocytoses. [27] In JXG, histiocytes are positive to antibodies against factor XIIIa, HAM56, HHF35, KP1 (CD68), Ki-M1P, and Vimentin, and are generally negative to CD1a and S-100. They always stain negatively to langerin (CD207). New reports have also demonstrated a CD4 positivity, which has been used as evidence that plasmacytic monocytes may be the normal cell type of the major constituent of JXG, instead of the dermal dendrocyte.