Dermatology Online Journal

Verrucous lymphangioma circumscriptum
Vitaly Terushkin MD, Shoshana Marmon MD PhD, Max Fischer MD, Rishi R Patel MD, Miguel R Sanchez MD
Dermatology Online Journal 18 (12): 9

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York

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Abstract

A 19-year-old woman with a seven-year history of pruritic, grouped, verrucous papules and plaques on the left arm presented for consultation. The lesion slightly flattened in appearance with topical glucocorticoid treatment. The histopathological features were consistent with lymphangioma circumscriptum. This entity is the most common subtype of lymphangioma and presents with grouped, clear vesicles that may appear pink to purple. However, a rare verrucous type can present a diagnostic challenge. Whereas the definitive treatment option is surgical excision, other treatments, which include sclerotherapy, radiotherapy, and laser therapy, have been reported to cause resolution or improvement. In the future, propranolol may hold promise as a medical therapy for lymphangioma.

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History

Figure 1	Figure 2

Figure 3	Figure 4

A 19-year-old woman presented to the Dermatology Clinic at Bellevue Hospital Center with a seven-year history of grouped, verrucous papules and plaques on the left arm. The patient complained of intermittent pruritus of the lesion; several papules developed a violaceous color after scratching. The lesion was initially diagnosed as a hemangioma by a primary care physician and treated with 1 percent hydrocortisone ointment with no improvement. Imiquimod cream also was prescribed by an outside provider but discontinued because of erythema, itching, and burning. At Bellevue Hospital Center, the lesion was treated with clobetasol for possible epidermal nevus. This treatment produced slight flattening after one month of treatment. Consequently, it was biopsied.

Physical examination

Multiple, grouped 2 to 4 mm, skin-colored and purple, verrucous papules coalescing into a linear plaque were present on the proximal aspect of the left lateral arm.

Laboratory data

None.

Histopathology

There is a proliferation of ectatic, thin-walled lymphatic vessels in the upper part of the dermis. Some vessels contain blood, which is probably secondary to trauma.

Discussion

Lymphangiomas are malformations of the lymphatic system that are located in the skin and subcutaneous tissues. Depending on the size and depth of the lymph vessels, lymphangiomas are separated into two categories; the superficial group contains only lymphangioma circumscriptum, whereas the deeper group is comprised of cavernous lymphangioma and cystic hygroma [1].

Lymphangiomas account for 4 percent of vascular tumors and 25 percent of benign vascular tumors in children [2]. In the Unites States, there are between 1.2 to 2.8 new cases per 1000 live births each year [3]. Although one-half of these cases will be clinically evident at birth, 90 percent will be visible by two years of age [2]. Infrequently, lymphangiomas appear spontaneously in adolescents and adults [1].

Of the three types of lymphangioma, lymphangioma circumscriptum is the most common [2]. The newer term for this entity is superficial lymphatic malformation [4]. Clinically, the lesion presents as a cluster of small, cutaneous, translucent vesicles, which resemble frog spawn [4]. Upon trauma and subsequent hemorrhage, the vesicles may develop a pink, red, purple, or black hue. If punctured, blood tinged lymphatic fluid may drain from the affected vesicles. Although uncommon, lymphangioma circumscriptum may have a tan, brown, verrucous appearance [4]. The most common sites of involvement are proximal extremities, abdomen, axillae, genitalia, and mouth. Complications associated with lymphangioma circumscriptum include bleeding and cellulitis.

On histopathologic examination, in the papillary dermis there is a proliferation and dilation of thin-walled lymphatic channels that may communicate with thick-walled vessels with a narrow lumen that is located in the subcutaneous tissue [1, 5]. These vessels are filled with lymphatic fluid and commonly, red and white blood cells, although it is unclear how red blood cells enter the lymphatic system. For diagnostic purposes, staining with lymphatic vessel endothelial receptor I and vascular endothelial growth factor C can help distinguish lymphatic from blood vessels [6]. The epidermis may show hyperkeratosis and acanthosis.

In 1976, Whimster et al provided a model of pathogenesis for lymphangioma circumscriptum development [7]. During embryogenesis, abnormal lymphatic cisterns grow independently from normal lymphatic vessels in the deep subcutaneous tissue. Contraction of smooth muscle cells that line these cisterns causes dilatation and protrusion of lymphatic vesicles from the skin and results in the appearance of vesicles [7]. Evidence to support this hypothesis is supported by lymphangiographic and radiographic studies, which show that large multi-lobular cisterns are located deep in the dermis and do not show any communication with adjacent normal lymphatics [8].

The differential diagnosis includes lymphangiectasis, metastatic carcinoma to the skin, verrucae, molluscum, hemangioma, angiokeratoma, or lymphangioendothelioma [1]. Lymphagiectases are acquired dilatations of lymph vessels, which may develop on the arms, axillae, chest, and back following lymph node dissection in women treated for breast cancer. These may also occur within the pubic region in men treated with radiation for prostate cancer. Scrofuloderma and recurrent erysipelas can lead to progressive scars of the lymphatics and also produce lymphangiectasia [4].

Surgical excision is the definitive treatment for lymphangioma circumscriptum [1]. Magnetic Resonance Imaging may be used to define the extent of the lesion and minimize recurrence rate [8]. Other destructive techniques used with variable success include hypertonic saline sclerotherapy, carbon dioxide laser, pulse dye laser/intense pulse light system and radiotherapy [9-13]. Propranolol has been used to treat a patient with diffuse lymphangiomatosis that was associated with chylothorax and significantly reduced the drainage volume [14]. In another report, propranolol was used to successfully minimize hemorrhage of a lymphangioma that was located on the tongue [15]. However, determination of propranolol’s efficacy in the treatment of cutatenous lymphangioma will require evaluation through clinical trials.

References

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5. Rapini RP. Practical Dermatopathology 2005, Philadelphia: Elsevier

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11. Yildiz F, et al. Radiotherapy in congenital vulvar lymphangioma circumscriptum. Int J Gynec Cancer: official journal of the International Gynecological Cancer Society. 2008; 18: 556 [PubMed]

12. Haas AF, VA Narurkar. Recalcitrant breast lymphangioma circumscriptum treated by UltraPulse carbon dioxide laser. Derm Surg: official publication for American Society for Dermatologic Surgery [et al.]. 1998; 24:893 [PubMed]

13. Lapidoth M, et al. Treatment of lymphangioma circumscriptum with combined radiofrequency current and 900 nm diode laser. Derm Surg: official publication for American Society for Dermatologic Surgery [et al.]. 2006; 32:790 [PubMed]

14. Ozeki M, et al. Propranolol for intractable diffuse lymphangiomatosis. New Eng J Med. 2011; 364:1380 [PubMed]

15. Leboulanger N, et al., Propranolol therapy for hemorrhagic lymphangioma of the tongue. Archives of otolaryngology--head & neck surgery. 2011; 137:813 [PubMed]

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