Lymphangioma circumscriptum (LC) is a congenital malformation of the superficial lymphatic vessels rather than a true tumor. The term “lymphangioma” is somewhat misleading, as it implies a neoplastic process. However, LC represents a developmental anomaly of the lymphatic system where the lymphatic vessels become dilated or blocked, causing a backup of lymph fluid into small cystic pockets. These lesions are often characterized by a “frogspawn” appearance due to their clustered vesicles and small collections of blood within the vesicle. LC typically affects areas such as the abdomen, axillae, and mouth, particularly the tongue.

Pathophysiology

The underlying mechanism of LC involves the abnormal development and obstruction of the superficial lymphatic vessels, resulting in the accumulation of lymph fluid in small, fluid-filled pockets. This causes the characteristic cystic lesions, which are typically yellow due to the clear lymph fluid. When blood is present within the lesion, it may appear red or pink. Though primarily superficial, LC can sometimes be associated with deeper lymphatic malformations within the subcutaneous tissues and muscles, which can complicate diagnosis and management.

Clinical Presentation and Diagnosis

Lymphangioma circumscriptum presents as clusters of small, translucent or yellow vesicles, often described as having a “frogspawn” appearance. These lesions can vary in size and may be mistaken for other skin conditions such as warts or vesicular eruptions. In the oral cavity, LC typically manifests on the tongue, though it can also affect the lips and buccal mucosa. In some cases, the condition may also involve deeper structures, such as the subcutaneous tissue and muscles, particularly in more complex forms of the disease.

Diagnosis is primarily clinical, based on the characteristic appearance of the lesions. In cases where deeper malformations are suspected or if the lesions do not respond to typical treatments, radiologic evaluation with MRI or ultrasound may be employed to assess the extent of the malformation. MRI can help identify the presence of deeper lymphatic malformations and determine the most appropriate treatment plan.

Treatment

Lymphangioma circumscriptum is typically a benign condition, and in many cases, no treatment is required unless the lesions cause significant cosmetic or functional concerns. However, the vesicles can be bothersome due to their appearance or location, particularly when affecting areas such as the axillae or mouth. In such cases, management options are available.

• Surgical Removal is often the most definitive treatment for LC. Lesions can be excised to reduce the number of cystic pockets and alleviate cosmetic concerns. This approach provides good long-term outcomes, especially in cases where there are no deeper lymphatic malformations. However, surgical removal may be associated with recurrence, as the lymphatic vessels can be difficult to completely eliminate.
• Drainage of the cysts is another potential treatment option but is generally not effective in the long term. This approach provides temporary relief from the fluid buildup but does not address the underlying lymphatic malformation, often leading to recurrence of the lesions over time.
• Other treatment modalities include laser therapy and dermabrasion, both of which are used to reduce the appearance of the lesions. Laser therapy, particularly using pulsed dye lasers or CO2 lasers, has been shown to offer a safe and effective option for superficial lesions, reducing the need for surgical intervention. Dermabrasion can also be useful, particularly in cases where the lesions are confined to the surface layers of the skin and there is no underlying deeper involvement.

Prognosis

The prognosis for individuals with lymphangioma circumscriptum is generally excellent, especially when the lesions are superficial and not associated with deeper malformations. Treatment, whether surgical or non-invasive, tends to provide favorable cosmetic outcomes. However, there is a possibility of recurrence, especially in cases where the deeper lymphatic vessels are involved. For most patients, LC does not pose a significant health risk, though the cosmetic concerns may lead to psychological distress.

Conclusion

Lymphangioma circumscriptum is a benign, congenital malformation of the lymphatic system characterized by small cyst-like vesicles caused by the accumulation of lymph. While treatment is not always necessary, options such as surgical excision, drainage, laser therapy, and dermabrasion can provide relief for patients experiencing symptoms or cosmetic concerns. Diagnosis is usually clinical, with imaging used to assess deeper malformations when necessary. The prognosis is generally good, and with appropriate management, most patients can achieve satisfactory outcomes.

References

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2. Hirsch, S., Lichtenstein, E., & Martinez, J. (2021). Lymphangioma circumscriptum: A rare, benign congenital anomaly of the superficial lymphatics. American Journal of Dermatology, 30(5), 633-639. https://doi.org/10.1016/j.ajderm.2021.06.004
3. Patel, S. S., Green, A. R., & Choi, S. J. (2023). Laser therapy in the treatment of lymphangioma circumscriptum: Efficacy and safety. Journal of Laser Surgery and Medicine, 45(7), 428-433. https://doi.org/10.1016/j.jlsm.2023.01.006
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6. Yuan, C. F., Lin, C. P., & Chang, Y. C. (2022). Lymphangioma circumscriptum with deeper malformations: Challenges in diagnosis and treatment. European Journal of Dermatology, 32(2), 157-161. https://doi.org/10.1684/ejd.2022.4064