Risk of intraocular and other extracutaneous involvement in patients with cutaneous juvenile xanthogranuloma

Abstract

Cutaneous juvenile xanthogranulomas are generally limited to the skin. Because eye involvement is rare, a routine eye examination is of low yield and probably not warranted in children with no ocular or visual symptoms. New recommendations for systemic screening could not be drawn from this study.

Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience)

“…The incidence of extracutaneous/systemic involvement has not been well‐established. A study by Samulev et al 3 . reported no incidence of ocular involvement.…”

“…On histopathology, these papules and nodules are characterized by the accumulation of the histiocytes with a progressively increasing degree of lipidation within the dermis. The most common locations of these lesions are the head and neck, followed by the upper and lower extremities 1‐3 . Extracutaneous involvement is rare but may occur in the eyes, bones, lungs, and liver 3,4 .…”

“…The most common locations of these lesions are the head and neck, followed by the upper and lower extremities 1‐3 . Extracutaneous involvement is rare but may occur in the eyes, bones, lungs, and liver 3,4 . This skin disorder has also been associated with neurofibromatosis type 1 (NF1) 5‐8 .…”

The Rare Case of an Adult-Onset Xanthogranuloma of the Paranasal Sinuses: A Histological Dilemma

“…Compared to other non-LCH variants, JXG lesions are often solitary [9]. Although rare, extracutaneous organ involvement is possible, with the orbit and testes having been reported [10,11]. Xanthogranuloma was first described in 1905 by Adamson who observed cutaneous nodules in infancy, though the term JXG was proposed in 1954 by Helwig and Hackney [12,13].…”

Ein großer kongenitaler kutaner Tumor

“…Kongenitale JXG sind tendentiell größer und von atypischer Morphologie. Im Vergleich zum klassischen JXG, bei dem das Risiko einer systemischen Beteiligung nicht mehr als 1 % beträgt, weisen sie ein höheres Risiko einer systemischen Beteiligung auf, hauptsächlich mit okulärer und hepatischer Beteiligung [4]. In einer Serie von 51 angeborenen JXG-Fällen wurde in 35 % eine systemische Beteiligung gefunden; diese kann bei multiplen Läsionen auch größer sein.…”