Rare benign fibrohistiocytic lesion and a type of granulomatous process most commonly on the skin.
M/C non-Langerhans cell histiocytic (non-LCH) disorder of childhood
It was first described by HG Adamson in 1905. Later, in 1912, JER McDonagh described 5 new cases of the disease. In 1950s, Helwig and Hackney described 140 cases of the condition and gave it its name: xanthogranuloma or juvenile xanthogranuloma, used ever since.
Juvenile myelomonocytic leukaemia (JMML)
Neurofibromatosis type 1 (NF1)
Papulonodular lesion, tan – orange in color and several millimeters in diameter, which may be single/multiple
Plaque-like lesions (non-typical presentation)
Eyeball (M/C non-dermal localisation of pathological lesions is the eyeball)
Other internal organs: Lungs, kidneys, heart, central nervous system, adrenal glands, pituitary gland, bones and bone marrow
Self-limited disorder and skin lesions usually resolve spontaneously.