A 5-year-old girl had confluent, translucent papules on her tongue for 3 years (Fig. 1A and B). Biopsy revealed dilated lymphatic vessels with mononuclear and polymorphonuclear leukocytes (Fig. 1C). Dermoscopy showed irregular translucent lakes with clear and serosanguineous content (Fig. 2A and B). After the biopsy, macroglossia worsened, and the papules showed necrotic content (Fig. 3A, B and C). Treatment with triamcinolone acetonide ointment to reduce inflammation and systemic antibiotics was proposed. However, imaging exams to verify the extension of the condition and appropriate clinical treatment were not performed because the patient was lost to follow-up.

Discussion: Lingual lymphangioma circumscriptum (LC) is a benign condition that results from the formation of muscle-lined cisterns, not connected to the lymphatic drainage system, projecting bumps on the skin or mucosa1,2. Congenital or acquired, LC targets the pediatric population and is diagnosed in children under 2 years of age in about 90% of cases2,3. The oral cavity is rarely involved, with the more common site being the tongue and, more rarely, palate, buccal mucosa, gingiva, the floor of the mouth, and lips3. Clinically, LC is asymptomatic and has a good prognosis. However, cases in the head, neck, and oral cavity may be serious due to obstruction of the airways and death2. Lymphangioma circumscriptum (LC) treatment remains challenging because current therapeutic options are limited and have to be adapted according to the size of the lesion, its location and the range of anatomical structures and surroundings that are involved. Treatment includes excision, cryotherapy, sclerotherapy, laser, radiofrequency, radiotherapy, and local steroids. Also, topical imiquimod with oral propranolol had success4. Topical sirolimus in lingual microcystic lymphatic malformation [TOPGUN] protocol, ongoing until 2025, is studying the efficacy and safety of topical sirolimus in the case of lymphangioma, which can pave the way into the first-line treatment for lingual microcystic lymphatic malformations5.

TBA