JXG is a self-limited disorder that typically begins as one or more red cutaneous nodules that can become yellow, firm, and rubbery.
Conditions associated with JXG include neurofibromatosis type 1 (NF- 1), Niemann-Pick disease, urticaria pigmentosa, and epilepsy.
The etiology and pathophysiology of JXG are not fully understood.
The histologic features of JXG vary through the lifespan of the lesion.
Immunohistochemical studies of JXG specimens show positive staining for histiocyte markers, including CD68 and vimentin.
Different forms of JXG have been described based on clinical and histologic features.