Sir,

Juvenile xanthogranuloma (JXG) is an uncommon dermatological condition rarely linked to systemic manifestations, with predilection for the eyes. We would like to present a case of an unusual eyelid presentation of JXG, which had not been reported previously.

Case report

A 2-year-old baby girl of African extraction presented to the eye clinic with a 6-month history of a slowly enlarging pedunculated spherical left upper eye lid painless tumour that was 6 mm in diameter with a central umbilication (Figure 1). She was otherwise fit and healthy. Ocular examination was otherwise unremarkable. Her mother was concerned that the lesion would get bigger and affect vision. The lesion was surgically removed. Histopathology confirmed the diagnosis of juvenile xanthogranuloma (JXG). In view of the rare possibility that there may be systemic involvement, she was referred to the paediatric medical team for further systemic investigations, which proved negative. Follow-up at 4 months did not show recurrence.

Figure 1
figure 1

A spherical 6-mm-diameter central umbilicated painless lesion on the left upper eyelid.

Full size image

Comment

JXG is frequently a self-limiting dermatological disorder, rarely linked to systemic manifestations.1 JXG presents with single or multiple yellowish, firm, and slightly raised papulonodular skin lesions, several millimetres in size. This usually occurs in the head and neck region. It also has a predilection for the ocular structures, especially the uveal tract and, occasionally, the orbits and the eyelids.2, 3, 4 The present case was an unusual eyelid presentation, wherein the lesion was atypical (Figure 1). It was the only lesion found. Histopathology confirmed the lesion to be JXG. It is therefore important to recognise an unusual JXG presentation, to perform a full ocular examination, and then to refer the case to the paediatric medical team for a systemic work-up to rule out the unlikely event of latent systemic manifestations.