Dermatology Online Journal

A case of lymphangioma circumscriptum successfully treated with electrodessication following failure of pulsed dye laser
Jason Emer¹ MD, Jaime Gropper¹, Stephanie Gallitano² BA, Jacob Levitt¹ MD
Dermatology Online Journal 19 (3): 2

1. The Mount Sinai School of Medicine, Department of Dermatology, New York, New York
2. The University of Illinois at Chicago, College of Medicine, Chicago, Illinois

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Abstract

Lymphangioma circumscriptum is the most common cutaneous lymphatic malformation. It presents as a well-circumscribed, localized region of vesicles that can ooze and drain lymphatic and sanguineous fluid. The vesicles cause significant morbidity and psychological distress from itching, pain, drainage, and secondary infection. We report a case of a 16-year-old female with a life-long history of a weeping, hemorrhagic, and painful lesion on her right buttock causing significant emotional distress. We initially attempted therapeutic treatment with pulsed dye laser. The lesion appeared unchanged after two trials and we subsequently used three sessions of electrodessication to cauterize the aberrant vessels. The lesion largely resolved without complications and is no longer causing pain or emotional distress.

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Disease description

Lymphangioma circumscriptum (LC) was first described by Fox and Fox in 1879 as vesicle-like elements in groups as lymphangiectodes [1, 2]. It is now described as the most common cutaneous lymphatic malformation consisting of abnormal, dilated, and tortuous lymphatic vessels in dermis and subcutaneous tissue. Irregular vessel contraction with subsequent dilatation and fluid build-up manifests clinically as superficial vesicles. Two forms of LC are described: the classic variety that presents at birth and the localized variety that occurs secondary to an inciting event. Although malignant transformation is rare, LC can cause significant morbidity from pain, itching, drainage, and secondary infection.

Clinical presentation and etiology

Lymphangioma circumscriptum presents clinically as clusters of thin-walled, tense vesicles on a localized, well-circumscribed area of skin. Vesicles are most often filled with a clear or serosanguineous fluid giving a yellow or red-purple discoloration. Classic LC presents at a young age and 90 percent of lesions are detected by the end of the second year [3]. The most common site of presentation is on the head and neck, followed by the trunk, extremities, and internal organs. Most lesions are greater than 1 centimeter in size with larger lesions predisposing to a great morbidity from oozing, crusting, and swelling. The symptoms include chronic pruritus, pain, and secondary infections [4, 5, 6].

Classic LC is thought to arise from muscular lymphatic cisterns that failed to segment during embryonic development [7]. The cisterns lie just above the deep fascia and pulse at a rate of four to eight beats per minute, independent of the cardiovascular system. The rhythmic contractions create saccular outpouchings of lymph fluid into the dermis and subcutaneous tissue thereby forming lymph-filled vesicles on the skin [8]. The aberrant lymphatic cisterns and dermal lymphatics arising from the cisterns do not communicate with regular subcutaneous lymphatics. However, hemangiolymphatic connections may occur when blood capillaries lying adjacent to dilated lymphatics are stretched, leading to microtrauma and spillage of blood into the abnormal lymphatics [8]. Clinically, this pathology manifests as red, pink, or black crusted vesicles on the skin surface [2].

Differential diagnosis

The diagnosis of LC is most often made by clinical inspection, but skin biopsy can help differentiation from other vesicular disorders such as herpes infections (simplex, zoster), dermatitis herpetiformis, contact dermatitis, carcinoma metastasis, grouped or eruptive pyogenic granulomas, hemangioma, or malignant melanoma. Imaging by ultrasound, computed tomography (CT), and/or magnetic resonance imaging (MRI) can be used to determine the extent of the lymphatic cisterns [9].

Associated diseases

Fortunately, LC has no strong association with other systemic diseases and malignant transformation is rare. Only two cases of squamous cell carcinoma, one case of lymphosarcoma, and one case of a Dabska tumor (low-grade angiosarcoma in children) have been reported within an LC [10, 11, 12, 13]. Morbidity is from social embarrassment and psychological distress because of frequent uncontrolled oozing, pain, itching, and secondary infection requiring numerous dressing and/or clothing changes.

Case report

Figure 1
Figure 1. Clinical photograph of lymphangioma circumscriptum on the right buttock.

A 16-year-old female presented to the outpatient dermatology clinic with a weeping and painful lesion on her right buttock. The lesion had been present since early childhood and had caused significant pain and bleeding. It also frequently required oral and topical antibiotic therapy in addition to meticulous wound management for secondary infections. She reported no prior treatments and presented to our clinic related to the cosmetically displeasing nature of the skin lesion and its periodic bleeding and pain. The lesion appeared as a violaceous, verrucous, hemorrhagic plaque on the right buttock.

Histopathology

Figure 2	Figure 3
Figure 2. The superficial dermis with cystically dilated lymph vessels, which impinge on and appear to be enclosed by the epidermis (H&E x4). Figure 3. The dilated lymph vessels are lined by a simple endothelium and contain a proteinaceous fluid (H&E x10).

Figure 4
Figure 4. Immunohistochemical stain highlights the lymphoid spaces (D2-40 x4).

Punch biopsy of the right buttock vesicular plaque demonstrated a superficial dermis with cystically dilated lymph vessels (Figure 2). The vessels were lined by simple endothelium and immunohistochemical studies with D2-40 highlighted numerous lymphoid spaces (Figures 3 and 4). Altogether the biopsy was consistent with a diagnosis of LC, which classically demonstrates horizontally-lined lymphatics found below the papillary dermal ridge and numerous muscular lymphatic cisterns in the deeper dermis and subcutaneous tissue that communicate with the superficial sacculations [8].

Treatment

Various treatment modalities including lasers, destructive methods, and surgery have been used to provide symptomatic relief and cure of LC. After discussing all treatment options, our patient elected for a non-invasive approach to achieve therapeutic relief. We first attempted to damage underlying vessels and lymphatic channels with a 585 nm pulsed dye laser (PDL) (Vbeam, Candela Corporation, Wayland, Massachusetts, USA). A small spot size (7 mm), short pulse-width (0.45 msec) and high fluency (8 J/cm²) was used on the entire lesion at two treatment sessions, two weeks apart. Despite these aggressive laser settings, no clinical change in the lesion was appreciated.

Figure 5
Figure 5. Lymphangioma circumscriptum six months after electrodessication and cauterization.

A second therapy was attempted using electrodessication with cauterization of the visualized surrounding lymphatic communications. Intraoperatively, a magnifying glass was used to identify target vesicles of lymphatic dilatation for rigorous ablation of the lesion. The plaque was allowed to heal through secondary intention. Three treatments roughly four weeks apart showed marked improved results. The final result, six months later, demonstrated a much smoother, atrophic, indurated plaque with only scattered small, skin-colored vesicles in place of the original violaceous, verrucous, and hemorrhagic plaque (Figure 5). The patient currently reports no further hemorrhage from the lesion, is pain and itch-free, and is extremely content with the ultimate outcome. Further treatment of electrodessication with cauterization or fraxelated carbon dioxide (CO2) laser was offered, but the patient rejected further care. We recognize that short-term follow-up cannot conclusively demonstrate usefulness of this treatment and that longer-term observation is needed before definitive conclusions can be made. However, significant improvement was observed, leading to the improved well-being of our patient.

Management

The literature regarding treatment of LC is characterized mainly by case reports and limited case series. Various laser treatments have been used to treat LC including CO2 laser, pulsed dye laser, and argon laser. Carbon dioxide laser ablation (10,600 nm) converts light energy to heat vaporization on impact with water-bearing tissues (chromophore). The laser vaporizes underlying tissue and seals the lymphatic channels. If the laser energy does not penetrate deep enough into the dermis and/or subcutaneous tissue, the patient will only achieve short-term palliative symptomatic relief and will have lesion recurrence. Further, energy delivered into deeper structures requires large amounts of local anesthesia (injection or tumescent) because of pain during delivery and may be followed by prolonged healing times with the potential for scarring, prolonged erythema, or post-inflammatory hyperpigmentation [7, 14].

Two case reports have demonstrated successful treatment of LC with PDL [7, 15]. Pulsed dye laser emits high-energy laser light in ultrashort pulse durations, allowing for specific targeting of the chromophore hemoglobin (585-595 nm) in and around vessels without damaging the surrounding tissues. The effectiveness of PDL in the treatment of LC can be limited by the minimal hemoglobin as a chromophore, because the dilated lymphatic channels contain serosanguineous fluid in dilated lymphatic channels [7]. In our patient PDL was unsuccessful as an initial therapy for hemorrhagic LC despite aggressive fluency and short pulse-width; likely related to an insufficient amount of hemoglobin in the lymphatic tissue.

Other methods have been reported to treat LC. Sclerotherapy involves injecting detergent sclerosants, chemical irritants, or hyperosmolar agents into the lymphatic malformations to destroy the aberrant vessels. Injectable corticosteroids, tetracycline, 50 percent dextrose solution, and hyperosmolar saline have all been used in case reports and preliminary trials. Depending on the study, recurrence rates vary from 58 percent to 100 percent [3, 16, 17, 18]. Complete resolution of LC by treatment with suction-assisted lipectomy (liposuction) was reported in one patient [19]. Suction-assisted lipectomy involves inserting a cannula into the subcutaneous fat and suctioning the deep cisterns of LC while leaving the superficial lymphatic vesicles untouched. Without the deep cisterns there is no longer any aberrant superficial connections and vesicles will flatten and dry out. This method involves minimal scarring and minimal contour defects when done by a trained practitioner. Cryotherapy utilizes very low temperatures to cause immediate vasoconstriction followed by reactive vasodilation, in turn producing cellular necrosis and healing by secondary intension. Electrodessication (hyfrecation) emits low-power, high frequency, high-voltage AC electrical pulses, via an electrode mounted on a handpiece to cause protein denaturation. Three treatments of electrodessication ablation proved efficacious in smoothing and reducing the number of superficial vesicles as well as decreasing the oozing, bleeding, and pain.

Recently, the use of imiquimod cream has successfully treated LC in two patients. Imiquimod induces cellular production of endogenous interferons (IFNs) and interleukins (IL). Some ILs inhibit endothelial proliferation and formation and IFNs can inhibit vascular motility and invasion. Efficacy in these patients is likely related to the ability to inhibit vessel formation and induce endothelial cell apoptosis [20].

Surgical excision is regarded as the most definitive treatment, giving the highest chance of cure with a recurrence rate of 17 percent to 23 percent [2, 3, 5]. By completely excising the subcutaneous cisterns and removing the source of the vesicles, it is possible to eliminate the cutaneous manifestations. Whereas surgical excision offers definitive treatment, it also involves significant risks including scarring, keloid formation, seroma, hematoma, wound infection, and nerve injury [3, 4, 21].

Conclusion

Lymphangioma circumscriptum is the most common lymphatic malformation marked by a circumscribed area of vesicles that can ooze, bleed, and become infected. As demonstrated, treatment is challenging. Ablative and/or destructive techniques provide less invasive treatments options as compared to surgery. However, the inability to destroy the deeper lymphatic cisterns results in a high recurrence rate. Surgery gives the lowest rates of recurrence, but has the highest risk of complications. Clinicians should discuss goals of treatments with patients when designing a therapeutic plan. The clinical and psychological improvement that occurred in our patient suggests that ablative, non-surgical techniques should not be overlooked when patients are seeking alternative treatments.

References

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