Noor Al-Mozan, MD; Gillian Heinecke, BS; David Pompei, PharmD; Jason Emer, MD
The authors are from Mount Sinai School of Medicine, Departments of Dermatology and Dermatopathology, New York, New York. Disclosure: The authors report no relevant conflicts of interest.
A healthy 16-year-old girl from the Dominican Republic with no significant past medical history presented to the outpatient dermatology clinic with a cosmetically displeasing lesion over her right buttock, which had been present since early childhood. She reported occasional painful bleeding and previous episodes of superficial infections requiring topical and oral antibiotic therapy. Aside from wound management, she had received no prior treatments for this condition. The patient denied family history of similar lesions. Physical examination revealed a firm, verrucous and vesicular purpuric plaque with hemorrhage and satellite papules on the right buttock (Figure 1). Punch biopsy was done for histopathological analysis (Figure 2). Special staining on the skin biopsy was also performed for histological confirmation (Figure 3).
Microscopic Findings and Clinical Course
The skin biopsy revealed cystically dilated lymphatic vessels in the superficial dermis that impinged on and appeared to be enclosed by epidermis (Figure 2). These vessels were lined by simple endothelium and contained a proteinaceous fluid. Additionally, positive immunohistochemical staining for D2-40 highlighted lymphoid spaces, consistent with a diagnosis of lymphangioma circumscriptum (Figure 3).
After skin biopsy confirmation, treatment was initiated with pulsed dye laser (PDL; 585nm, Vbeam, Candela Corporation, Wayland, Massachusetts), which showed no response to therapy despite aggressive settings (7mm spot size, 0.45ms pulse width, 8J of fluence). Three sessions of electrodessication with lymphatic vessel cauterization four weeks apart gave a significant improvement. Six months post-treatment, the patient reports no bleeding or pain and is cosmetically pleased.
Cutaneous lymphangioma circumscriptum (CLC) is a benign disorder caused by malformation of lymphatic channels. In the United States, lymphatic malformations occur in 1.2 to 2.8 per 1,000 live births.1 In 90 percent of cases, the lesion is apparent by two years of age; however, CLC can appear spontaneously in adolescents or adults.
CLC occurs most frequently on the proximal extremities, trunk, axilla, oral mucosa, or genitalia. The clinical appearance of CLC lesions has been traditionally described as “frog spawn,” referencing the groups of vesicles filled with clear serous or hemorrhagic fluid of various colors, shapes, and sizes. Lesions can be anywhere from small (1–2mm in diameter) to large, conspicuous plaques and tend to gradually increase in size with age. The vesicles can be hyperkeratotic with a verrucous surface, which may mimic the appearance of warts. Under dermatoscopy, typical CLC lesions have a lacunar/saccular pattern while the clear fluid in the vesicles appears as light brown lacunas with pale septa. If there is marked blood in the vesicles, dermatoscopic findings are less useful as the lesions become indistinguishable from hemangiomas.
The underlying etiology of CLC is still unknown. In 1976, Whimster postulated a mechanism for the disease, suggesting that during embryonic development, the abnormal lymphatic cisterns in the subcutaneous tissue grow separately from and fail to communicate with the normal lymphatic system. The deep lymphatic cisterns communicate with the lymphatic channels in the dermis, producing significant dilation, which manifests as vesicles clinically
On histopathology, the vesicles of CLC are dilated lymphatic channels in the papillary dermis that may extend into the subcutaneous tissue. The channel lumens contain a?predominance?of lymphatic fluid mixed with red and white blood cells. The mechanism by which red blood cells enter these lymphatic channels is unknown, but may be due to microshunts between capillaries and abnormal lymphatics.
Although CLC is considered a benign condition and is frequently asymptomatic, patients may complain of minor bleeding or clear fluid exudate as well as cellulitis and secondary bacterial infection. When confronted with these findings, treatment is required to decrease the associated morbidity including, but not limited to, both medical and emotional concerns.
Treatment remains a challenge. The mainstay of therapy is surgical excision due to the deep extension of lymphatic channels that are rarely sufficiently removed with superficial therapies. Excision has a high risk of scarring and rate of recurrence (17%). Many others have sought other cosmetically conservative therapies in hopes of good clinical success.5 Some case reports describe therapeutic strategies using electrocautery (with or without desiccation), cryotherapy, sclerotherapy, intralesional bleomycin, CO2 laser, pulsed dye laser, diode laser, argon laser, radiofrequency therapy, liposuction, and superficial x-ray irradiation.[3,7–9] However, these modalities have been largely unimpressive and have higher rates of recurrence as compared to surgical excision. Unless treatment includes the entire superficial lesions as well as the deeper lymphatic connections, local recurrences will ensue. Currently, no one therapy is fully effective and treatment is best kept conservative unless there is associated morbidity.
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