Multiple juvenile xanthogranulomas: an uncommon case

Dear Editor, Juvenile xanthogranuloma (JXG) is a common form of non-Langerhans cell histiocytosis, primarily affecting infants and young children. Its typical presentation is characterized by solitary or multiple yellow to red-brown papules or nodules on the skin and featuring a characteristic histopathological appearance [1]. While cutaneous involvement is most common, systemic manifestations are rare. The clinical course of JXG is generally benign, with lesions spontaneously regressing. Although systemic involvement can occur, it is infrequent and associated with a higher morbidity rate. Here, we present a case of an 8-year-old child with multiple JXGs exhibiting a characteristic clinical, dermoscopic, and histological appearance. An 8-year-old child, with no significant medical history, presented to our department with asymptomatic yellowish lesions progressively developing over the past 2 months. The lesions were noted to be diffuse, primarily affecting the trunk and occipital region. A dermatological examination revealed multiple xanthomatous papules (Figure 1). Polarized dermoscopy demonstrated a ‘sitting sun pattern’ (Figure 2). The remainder of the physical examination was unremarkable notably with no hepatosplenomegaly, lymphadenopathy, or ocular or pulmonary signs. Histological analysis of a skin biopsy showed slight epidermal atrophy overlaid with orthokeratotic keratosis. The dermis exhibited a diffuse proliferation of tumor cells, characterized by large to medium-sized cells with centrally rounded nuclei and xanthomatous, multivacuolated cytoplasm. Additionally, multinucleated giant cells with nuclei arranged in a central ring (Touton cells) were observed, consistent with a diagnosis of JXG (Figure 3). The patient is currently under clinical observation alone, awaiting spontaneous regression of the lesions.Figure 1: Clinical appearance showing multiple xanthomatous papules of the trunk.Figure 2: Polarized dermoscopy showing sunset appearance.Figure 3: Histological image showing dermal proliferation of epithelioid histiocytes with multiple giant cells and Touton cells (H&E *400).JXG is the most common form of non-Langerhans cell histiocytosis, primarily affecting infants and young children, although it can also occur in adults, with a slight male predominance [1]. Patients in both age groups typically present with yellow-brown dome-shaped papules and nodules that are commonly distributed across the upper body [2]. Two clinical variants of JXG are recognized: the small papular form and the large nodular form. Dermoscopy is a non-invasive technique widely utilized in describing the characteristics of xanthogranulomas. Various described aspects include the classic ‘setting sun’ pattern, ‘clouds’ of paler yellow areas, whitish areas, linear vessels, and a pigment network. Dermoscopy also plays a significant role in determining the stage of juvenile xanthogranuloma patients during follow-up: the ‘setting sun’ pattern may offer diagnostic value in the early and classic stages, whereas the ‘clouds’ of paler yellow areas are more frequently observed in the classic and transitional stages [3]. In juvenile xanthogranuloma, there is no associated hyperlipidemia. However, there is a more frequent association than expected between neurofibromatosis type 1 (NF1) and the presence of JXG (18% of patients with NF1 may have JXG). Cases of association with chronic juvenile myelomonocytic leukemia have also been reported in patients with NF1 presenting with multiple xanthogranulomas [4]. Extracutaneous manifestations of xanthogranuloma can occur, with the most common being ocular involvement (0.3–0.5% of cases), including hyphema (bleeding in the anterior chamber), acute glaucoma, uveitis, or iridal heterochromia. The indicative functional signs include a red, painful eye, with decreased visual acuity or photophobia, which warrants ophthalmologic examination. Ophthalmologic evaluation should be systematic in all children under the age of 2 with multiple xanthogranulomas, even in the absence of ocular symptoms. Other extracutaneous manifestations, in order of frequency, include pulmonary, hepatic, and much rarer splenic, gastrointestinal, renal, adrenal, cardiac, osseous, and central nervous system involvement [4]. JXG is generally benign with regression in children within 3–6 years according to the literature. Hyperpigmentation, atrophy, and anetoderma may persist. Recurrences after excision have been observed. Cases of fatal evolution are reported in patients with multiple localizations despite the use of systemic treatment such as high-dose corticosteroid therapy, radiotherapy, chemotherapy, and immunosuppressants [4,5]. Acknowledgments Declaration of patient consent: The authors certify that they have obtained all appropriate patient consent forms. In the form, the parent has given consent for images and other clinical information to be reported in the journal. The parent understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed. Patient consent: Taken. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.