Juvenile Xanthogranuloma – An Uncommon Histiocytic Disorder of Children

A 4-year-old boy presented with an asymptomatic nodule on his forehead for 3 years. On examination, a solitary yellowish raised nodule of 3 cm × 2 cm × 2 cm was present over the center of the forehead [Figure 1a]. Dermoscopy showed a homogeneous yellowish hue with linear branching vessels and dotted vessels at the periphery displaying a “setting sun” pattern [Figure 1b]. A punch biopsy from the nodule showed an atrophic epidermis with a dermis showing a dense lymphohistiocytic infiltrate [Figure 1c]. Multiple lipid-laden macrophages and touton giant cells were present in the dermis [Figure 1d]. Ocular and systemic examination was within normal limits. On clinical, dermoscopy, and histopathological correlation, a diagnosis of juvenile xanthogranuloma was made.Figure 1: (a) Well-defined solitary yellowish nodule of size 3 cm × 2 cm × 2cm present over forehead. (b) Dermoscopy displaying a “setting sun” pattern. (c) Histopathology (H and E stain, ×100) showing atrophic epidermis with a dense collection of lymphocytes and histiocytes in the dermis. (d) Histopathology (H and E stain, ×400) showing multiple Touton type of giant cells with a central eosinophilic core and peripherally arranged nucleiJuvenile xanthogranuloma is a type of non-Langerhans cell histiocytosis that manifests as yellowish-red papule/nodule that can mimic mastocytoma, necrobiotic xanthogranuloma, dermatofibroma, etc.[1] Initially, it presents as a typically pink-reddish color, but it progressively acquires a characteristic yellow, orange, or brownish color. JXG can be associated with systemic lesions that commonly affect the eyes, spleen, and lungs.[2] Diagnosis of JXG is essential as it can have systemic involvement, especially in multiple lesions. Furthermore, solitary cutaneous lesions are usually self-limiting. The “setting sun” pattern on dermoscopy and histopathology is crucial in diagnosing juvenile xanthogranuloma. Declaration of consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.