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
History
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.
Aetiology
Disease associations:
Juvenile myelomonocytic leukaemia (JMML)
Neurofibromatosis type 1 (NF1)
Clinical features
Dermatological lesions:
Papulonodular lesion, tan – orange in color and several millimeters in diameter, which may be single/multiple
Plaque-like lesions (non-typical presentation)
Skin lesions on the right forearm, in the area of the right iliac ala and on the third toe of the left foot at the admission to the Department of Dermatology (A–C). The same skin lesions 1 month later (D–F) | Cichewicz, A., Białecka, A., Męcińska-Jundziłł, K., Adamska, U., Białecki, M., Neska-Długosz, I., Grzanka, D., & Czajkowski, R. (2019). Congenital multiple juvenile xanthogranuloma. Postepy dermatologii i alergologii, 36(3), 365–368. https://doi.org/10.5114/ada.2019.85643
Extracutaneous manifestations:
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
Clinical evaluation of the iris lesion. A. A Yellow-brown mass with irregular vascularization at the inferior part of the iris surface; became visible after ten days of treatment (dorzolamid/timolol and topical steroid). B. Ultrasound biomicroscopy of the anterior segment revealing multiple nodular hyperreflective heterogenous mass in the peripheral iris between 2 and 7 o’clock, growing into the anterior chamber angle and to the corneal endothelium (section at 5 o’clock). C. After 2 month of high-dose corticosteroid treatment iris lesion decreased completely without recurrence of hyphae or iritis. D. Ultrasound biomicroscopy confirming decrease of iris lesion (section at 5 o’clock). | Meyer, P., Graeff, E., Kohler, C., Munier, F., & Bruder, E. (2017). Juvenile xanthogranuloma involving concurrent iris and skin: Clinical, pathological and molecular pathological evaluations. American journal of ophthalmology case reports, 9, 10–13. https://doi.org/10.1016/j.ajoc.2017.09.004
Management
Self-limited disorder and skin lesions usually resolve spontaneously.
