Juvenile xanthogranulomas in Asian children

Abstract

Juvenile xanthogranuloma (JXG) is the most common non‐Langerhans cell histiocytic disorder. It can rarely be associated with systemic involvement. There is a paucity of literature on JXG in Asian children. We aim to describe the epidemiology, clinical features, systemic associations, histological features and outcome of a cohort of Asian children with JXG, and review the literature on the condition. We retrospectively reviewed the demographic, clinical and histological data of patients less than 16 years of age, diagnosed with JXG at our tertiary pediatric hospital between January 2002 and April 2019. A total of 147 children with JXG were identified, with a slight male preponderance of 53.1%. The median age of the onset was 15.5 months, with 69.4% presenting before 2 years of age. There was no racial predilection. The most frequently involved site was the head and neck region (44.2%). The majority of patients (76.2%) presented with a solitary lesion. Spontaneous resolution was documented in 57.7% of our patients with mean duration to resolution of 18.8 months. The proportion and speed of resolution did not differ in children with single or multiple lesions. No ophthalmologic complications were detected in our study cohort. JXG in children is generally limited to the skin and is rarely associated with systemic involvement, including the eye. Unless clinically indicated, the results from our study does not support routine screening for juvenile myelomonocytic leukemia, eye or systemic complications, even in the setting of multiple cutaneous JXGs.

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“… 5 Meanwhile, 2002–2019 Singaporean retrospective data reported 147 patients with JXG. 11 The incidence of JXG 15 and BCH 4 was higher in boys compared to girls (1.3:1 and 1.2:1, respectively). Existing literature stated that JXG is a disease of the young child, with a median age of onset at 2 years, but lesions may present at birth.…”

“…Statistically, initial lesions of JXG can be found on the head and neck (44.2%), lower extremities (21.8%), upper extremities (9.5%), trunk (6.8%), and more than one side of the body can be affected (12.9%). 11 In a report of 40 patients diagnosed with BCH, the most common affected sites are cheeks (22%), eyelids (13%), and ears (10%), and only one case (2.5%) at the scalp. 16 Some evidence mentions that BCH is a histopathological variation or part of the JXG spectrum, as both diseases show infiltration of histiocytes.…”

“…1 Location of the skin lesion Head and neck (44.2%), lower extremities (21.8%), upper extremities (9.5%), and trunk (6.8%). 11 Cheeks (22%), eyelids (13%), ears (10%), and scalp (2.5%). 16 Other organ involvement - Eye (10%) 1 - Oral mucous membrane (tongue, gingiva, and hard palate ) 6 - Intramuscular, lung, liver, testes, pericardium, spleen, central nervous system, renal.…”

“…The majority of patients have complete resolution (57.7%), while in some cases (6.6%) may leave a residual scar. 11 Certain treatment can be considered for rapidly growing skin-limited cases that raise aesthetic concerns. 12 However, this therapeutic approach may not always be consensual and should be evaluated on a case-by-case basis.…”