Dermatology Online Journal

Lymphangioma circumscriptum
Marissa Heller MD, Stephanie Mengden MD
Dermatology Online Journal 14 (5): 27

Department of Dermatology, New York University

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Abstract

A 54-year-old man presented for evaluation of multiple 1- to-4-mm, clear, pink, red, and black vesicles overlying hyperpigmented and erythematous indurated plaques on his right thigh and extending to his right inguinal fold. Clinical presentation and histopathologic features were consistent with lymphangioma circumscription. Lymphangioma circumscriptum is the most common cutaneous form of lymphangioma. Typical skin findings are multiple clear vesicles that may appear pink, red, or black. The first-line treatment option is surgical excision, although other modalities, such as laser therapy and sclerotherapy, have also been used successfully.

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Clinical synopsis

A 54-year-old man presented to the Dermatology Clinic at Bellevue Hospital Center with a history since childhood of multiple, clear, pink, red, and black vesicles on his right thigh. He reports that he had experienced recurrent infections of this area that have been treated with multiple courses of antibiotics.

Physical Examination

Multiple, 1- to-4-mm, clear, pink, red and black vesicles overlying hyperpigmented and erythematous indurated plaques were present on the right thigh

Figure 1	Figure 2

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Histopathology

Within the papillary dermis in close approximation to the epidermis, there are multiple thin-walled, dilated lymphatic spaces that contain lymph with a few erythrocytes. The overlying epidermis is thin, and there are elongated rete ridges that appear to surround the lymphatic channels.

Comment

A lymphangioma or lymphatic malformation represents a congenital proliferation of lymphatic vessels. These conditions account for approximately 4 percent of vascular tumors and 26 percent of benign vascular tumors in children. The predominant types of lymphangiomas are lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, and benign lymphangioendothelioma [1].

The most common form of cutaneous lymphangioma is lymphangioma circumscriptum, which arises in infancy but may occur at any age. Clinical findings consist of multiple, clear vesicles that may be pink, red, or black, owing to serosanguinous fluid or hemorrhage. These vesicles contain lymph fluid and are often said to resemble frogspawn. They are caused by abnormal dilations of lymph vessels [2]. Most commonly, lymphangioma circumscriptum is found on the proximal extremities, trunk, axilla, and oral cavity but also may occur on the penis, vulva, and scrotum [3, 4]. Lymphangioma circumscriptum is asymptomatic; however, it can be complicated by excessive drainage and recurrent cellulitis.

The pathogenesis of lymphangioma circumscriptum was first described by Whimster in 1976. He postulated that lymphatic cisterns in the deep subcutaneous tissue arise from early lymph tissue sacs during embryonic development. Furthermore, he noted that these sacs were distinct entities that did not connect to the remainder of the lymphatic system. Finally, he proposed that these sacs are lined by muscle fibers that contract and induce pressure that causes outpouchings in the walls of the sacs. These outpouchings eventually protrude from the skin surface to become what are clinically described as vesicles [5].

Histolopathologic features of lymphangioma circumscriptum include dilated lymph vessels in the upper dermis that may extend into the subcutis. These dilated vessels cause expansion of the papillary dermis. There may be acanthosis and hyperkeratosis of the overlying epidermis [6]. The lymph fluid within the vesicles may contain erythrocytes, lymphocytes, macrophages, or neutrophils.

Indications for treatment of lymphangioma circumscriptum include both its cosmetic appearance and prevention of complications such as cellulitis. The definitive treatment for lymphangiomas is surgical excision of both the superficial and deep components [7]. However, the recurrence rates can be high, especially in the case of lesions with deeper components. Other palliative treatments that have been reported include superficial x-ray therapy, radiotherapy, argon laser, CO² laser, 900-nm diode laser, pulsed dye-laser, and sclerotherapy [8, 9, 10].

References

1. Calonje E, Wilson-Jones E. Vascular tumors. In Elder D, et al, eds. Lever's Histopathology of the Skin. 8th edition. Philadelphia: Lippincott-Raven 1997: 921

2. Fernandez G, Schwartz RA. Lymphangioma. E medicine. http://www.emedicine.com/derm/topic866.htm

3. Vlastos A-T, et al. Lymphangioma circumscriptum of the vulva: a review of the literature. Obstet Gynecol 2003; 101:946

4. Swanson DL. Genital lymphangioma with recurrent cellulitis in men. Int J Dermatol 2006; 45:800

5. Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol 1976; 94:473

6. Rapini RP. Vascular proliferations and neoplasms. In Rapini, ed. Practical Dermatopathology. 1st edition. Philadelphia: Mosby 2005: 332

7. Browse NL. Surgical management of 'lymphangioma circumscriptum'. Br J Surg 1986; 73:585

8. Eliezri YD, Sklar JA. Lymphangioma circumscriptum: review and evaluation of carbon dioxide laser vaporization. J Derm Surg Oncol 1988; 14:357

9. Lapidoth M. Treatment of lymphangioma circumscriptum with combined radiofrequency current and 900nm diode laser. Derm Surg 2006; 32:790

10. Bikowski JB, Dumont AMG. Lymphangioma circumscriptum: treatment with hypertonic saline sclerotherapy. J Am Acad Dermatol 2005; 53:442

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