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Case Report Volume 9 Issue 1
Abdominal cystic lymphangioma: a rare case of cutaneous congenital malformation
Ilham Snoussi, Ouiame El Jouari, Faical Abbad, Salim Gallouj,
Received: February 14, 2025 | Published: March 7, 2025
Citation: Snoussi I, Jouari OE, Abbad F, et al. Abdominal cystic lymphangioma: a rare case of cutaneous congenital malformation. J Dermat Cosmetol. 2025;9(1):29-31. DOI: 10.15406/jdc.2025.09.00286
Abstract
Introduction: Lymphangioma circumscriptum (LC) is a rare benign lymphatic malformation caused by dilated lymphatic vessels in the epidermis and subcutaneous tissue. It frequently manifests in early childhood and can be either congenital or acquired. The diagnosis is established through clinical features, dermoscopy, and histopathology. Treatment remains challenging, as conventional approaches such as sclerotherapy and surgery have limited efficacy. Recent discoveries of PIK3CA mutations and the activation of the PIK3CA/AKT/mTOR pathway have opened the door to targeted therapy. Sirolimus (rapamycin) has emerged as a promising alternative despite not being FDA-approved. Case report: We report the case of a 1-year-old boy presenting with a congenital lesion on his right flank that has progressively increased in size. A dermatological examination revealed a 7-cm erythematous-violaceous plaque with hemorrhagic vesicles resembling “frog spawn.” Imaging studies identified an intra-abdominal cystic lymphangioma. Histopathology confirmed a microcystic lymphangioma. The patient was treated with oral rapamycin (0.8 mg/m²/dose, twice daily) to assess its efficacy in reducing the lesion’s size and improving skin appearance. Discussion: According to the International Society for the Study of Vascular Anomalies (ISSVA), LC can be classified as macrocystic, microcystic, or mixed. Dermoscopy, which shows lacunar patterns with blood-filled spaces, aids in diagnosis. Histopathologically, microcystic lesions exhibit erythrocyte extravasation and dilated lymphatic vessels. Standard treatments such as surgery, lasers, and sclerotherapy often fail for microcystic forms. Sirolimus, an mTOR inhibitor with antiangiogenic properties, has demonstrated promising outcomes in reducing lymphatic malformations. While emerging data support its potential use in LC treatment, further research is needed to confirm its long-term safety and efficacy.
Keywords: lymphangioma circumscriptum, microcystic lymphangioma, histopathology, dermoscopy, sirolimus (rapamycin)
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