Follicular Papules in a Linear Distribution on a Child’s Fac
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A girl younger than 2 years presented with a 1-year history of slightly pruritic lesions involving her face. Physical examination revealed multiple skin-colored and erythematous follicular papules, 1 to 2 mm in diameter, distributed in a segmental pattern on the right side of the forehead, as well as the dorsum and tip of the nose. A small, excoriated plaque was also noted on the right malar area. Two skin punch biopsies were taken from the forehead and nose.

Histopathological examination revealed accumulation of a finely granular basophilic material within the follicular and sebaceous gland epithelium. Results of Alcian blue stain demonstrated the presence of mucin. Clinicopathological correlation supported the diagnosis of idiopathic follicular mucinosis (IFM).

The patient was treated with topical corticosteroid and tacrolimus, 0.03%, without improvement. Systemic corticosteroids at a daily dosage of 0.5 mg/kg were then initiated with complete resolution of the lesions but recurrence after withdrawal. The patients’ parents declined more intensive therapy, and the lesions resolved spontaneously. Eighteen months after diagnosis, the patient remained in clinical remission.

The present case is unusual as, IFM presenting in a segmental or blaschkoid distribution has not been previously described. Lymphoproliferative disorders showing this peculiar presentation are rare and represent a diagnostic challenge because it can be misdiagnosed as several inflammatory skin conditions. Other differential diagnosis include lichen striatus, follicular mucinous nevus, and trichodysplasia spinulosa. Several treatment modalities, with variable results, have been described for IFM, including corticosteroids, dapsone, hydroxychloroquine, and interferon alpha. Because IFD is a benign condition, no aggressive treatment is recommended. However, given the difficulty in distinguishing it from lymphoma, long-term follow-up is warranted.