- Email: [email protected]
Lymphangioma circumscriptum: Treatment with hypertonic saline sclerotherapy
THERAPY
Lymphangioma circumscriptum: Treatment with hypertonic saline sclerotherapy Joseph B. Bikowski, MD,a and Anna Margarita G. Dumont, MS-IIIb Columbus, Ohio, and Erie, Pennsylvania A 38-year-old woman came for treatment of multiple clear vesicles and hemorrhagic papules on the posterior aspect of the right shoulder and the right axillary vault of 5 years’ duration. These lesions would spontaneously manifest as clear or blood-filled vesicles (or both) and appear to be exacerbated by physical contact from certain articles of clothing. A biopsy was done and the results revealed lymphangioma circumscriptum. The purpose of this case study was to evaluate a new form of treatment of lymphangioma circumscriptum with the use of 23.4% hypertonic saline sclerotherapy. The patient’s lymphangioma circumscriptum significantly resolved with minimal side effects, such as mild hyperpigmentation. Decreased sensitivity was noted and no further treatment was indicated. This case showed that hypertonic saline 23.4% solution can be effective in treating the appearance of vesicles containing clear fluid or lymph and those containing red blood cells in superficial lymphangiomas and that this treatment can be considered for longterm management of lymphangioma circumscriptum. ( J Am Acad Dermatol 2005;53:442-4.)
A
38-year-old woman presented with multiple clear vesicles and hemorrhagic papules on the posterior aspect of the right shoulder of 5 years’ duration. The pertinent medical history included increased sensitivity to touch of the affected area. These lesions would spontaneously manifest as clear and/or blood-filled vesicles and appear to be exacerbated by physical contact from certain articles of clothing. The patient had no known drug allergies, was not taking any medications, and did have a family history of acne and skin cancer. One month before the initial dermatologic consultation, her primary care physician performed a biopsy of the lesion; it was diagnosed as lymphangioma circumscriptum with associated mild chronic inflammation that was consistent with mechanical irritation. Examination revealed multiple minute discrete and confluent vesicles filled with either clear fluid or blood encompassing an area of approximately 16 3 16 cm across the posterior aspect of the right shoul-
From the Department of Dermatology, Ohio State University, Columbus,a and Lake Erie College of Osteopathic Medicine.b Funding sources: None. Conflicts of interest: None. This study has been accepted for the Philadelphia College of Osteopathic Medicine MEDNet Poster Session held at the 2005 Annual Pennsylvania Osteopathic Medical Association Clinical Assembly/Convention on May 12, 2005. Reprint requests: Joseph B. Bikowski, MD, 500 Chadwick St, Sewickley, PA 15143. E-mail: [email protected]. 0190-9622/$30.00 ª 2005 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2005.04.086
442
der, extending from the medial right scapular border to the right posterior axillary fold and from the superior to the inferior border of the right scapula. A repeat biopsy was performed from the center of the involved area at that visit; it confirmed the original histologic findings. Imiquimod 5% cream (Aldara) was prescribed to be used nightly as a therapeutic trial because of its ability to induce antiangiogenic cytokines.1 At the 6-week follow-up visit there was no improvement and within the preceding 24 to 48 hours the lesions had become more prominent (Fig 1). Because of the dilation of lymphatic and vascular elements, sclerotherapy was considered. Three of the most prominent central anastomosing vascular lesions were injected with 0.5 mL of 23.4% hypertonic saline solution. At the 1-month follow-up visit, complete resolution of these previously treated lesions was determined (Fig 2). Therefore an additional 10 to 15 lymphatic channels were injected with 23.4% hypertonic saline solution. At the most recent follow-up visit, lymphangiomas could not be detected and the patient claimed: ‘‘this is the best I have been in three years.’’ The patient was able to touch her right shoulder without experiencing increased sensitivity. Postinflammatory erythema and hyperpigmentation of the previously involved area were minimal. No further treatment was indicated.
DISCUSSION Lymphatic malformations, also known as lymphangiomas, are a hyperproliferation of the lymphatic vessels that constitute approximately 4% of all
J AM ACAD DERMATOL
Bikowski and Dumont 443
VOLUME 53, NUMBER 3
Fig 1. Multiple clear vesicles and hemorrhagic papules on posterior aspect of right shoulder.
vascular tumors and approximately 26% of benign vascular tumors in children.2 In this case report, the patient presented with lymphangioma circumscriptum, which is characterized by subcutaneous lymphatic cisterns that communicate through dilated channels with superficial thin-walled vesicles on skin surfaces. The vesicles typically contain lymph, but may also contain red blood cells, such as in our patient. Lymphangiography has been used to diagnose lymphangioma circumscriptum. It shows the presence of a normal lymphatic network surrounding the sacs and confirms the absence of any communication between the sacs and the normal lymphatic network. Magnetic resonance imaging has also proven to be useful in evaluating lymphangioma circumscriptum; it can be used before surgery to limit the risk of recurrence, but may not be reliable for detecting smaller lymphatic cisterns.3 The ‘‘gold’’ standard of noninvasive examination of the venous system is duplex ultrasound, which allows direct visualization of veins and allows identification of flow through the venous valves. Duplex ultrasound is often used to uncover hidden sources of reflux for pretreatment evaluation of sclerotherapy for peripheral veins and to delineate reflux sources when patients experience poor results from sclerotherapy.4 For the treatment of lymphangioma circumscriptum, the main indications are usually cosmetic but can also be to control complications such as infection, hemorrhage, and pain. If the disease is asymptomatic, it is often advised to use the ‘‘wait and watch’’ approach. The only cure is to remove the superficial component as well as the deeper lymphatic cisterns through surgical destruction or laser ablation of lesions. All other treatments are palliative to control symptoms. Electrocautery, cryotherapy, and carbon dioxide laser can also be used to reduce the risk of infection and to reduce lymphorrhea. Another palliative treatment reported to be helpful is sclerotherapy injection.3 Sclerotherapy and resection can be
Fig 2. A, Prominent central anastomosing vascular lesions and dilation of lymphatic channels on posterior aspect of right shoulder. B, Complete resolution of vascular lesion and lymphatic dilation.
used for long-term management for appearance and functional issues of lymphatic malformations.4 The optimal sclerosing agent is one that induces panendothelial destruction and possesses no systemic toxicity. It should produce local endothelial destruction extending to the entire adventitia with minimal thrombus formation, which can induce an inflammatory reaction leading to fibrosis and eventually obliteration of the vessel lumen.5 Sclerosing agents are classified into 3 groups on the basis of chemical structure and mechanism of injury to the endothelium. Hyperosmotic agents, such as hypertonic saline solution or hypertonic saline and dextrose, involve endothelial cell damage through dehydration.5 Detergent sclerosants (eg, polidocanol, sodium tetradecyl sulfate, sodium morrhuate) cause vascular injury by altering the surface tension around endothelial cells.5 Chemical irritants (eg, polyiodinated iodine) injure cells by acting as corrosives, and their cauterizing effect may be due to the associated heavy metal.5 Side effects of sclerotherapy can be minimized by a fastidious technique, understanding appropriate maneuvers if something is believed to go wrong, and appreciating management options available for adverse sequelae.5 Minor side effects and complications of sclerotherapy include the following: purple- brown hyperpigmentation after sclerotherapy,
444 Bikowski and Dumont
J AM ACAD DERMATOL SEPTEMBER 2005
telangiectatic matting, cutaneous ulcerations and necrosis, superficial thrombophlebitis, pulmonary embolism, and arterial injection.
containing red blood cells in superficial lymphangiomas. Treatment with hypertonic saline sclerotherapy can be considered for long-term management of lymphangioma circumscriptum.
CONCLUSION Sclerotherapy can be an effective palliative treatment of lymphangioma circumscriptum. The key factor in producing successful sclerotherapy is to determine the minimal volume and minimal concentration of the appropriate sclerosing agent. The only sclerosing agents in the United States approved by the Food and Drug Administration are sodium tetradecyl sulfate (Sotradecol), sodium morrhuate (fatty acids in cod liver oil), and hypertonic saline (18%-30%).5 To our knowledge ours is the first reported case showing that hypertonic saline solution 23.4% is effective in treating the appearance of vesicles containing clear fluid or lymph and those
REFERENCES 1. Diego ME, Haynes HA, Li VW. Antiangiogenic treatment of pyogenic granulomas [poster abstract 217]. J Am Acad Dermatol 2004;50(Suppl):S57. 2. Elder D, Elenitsas R, Jaworsky C, Johnson B Jr, editors. Lever’s histopathology of the skin. 8th ed. Philadelphia: LippincottRaven; 1997. 3. Lebwohl MG, Heymann WR, Berth-Jones J, Coulson L. Treatment of skin disease: comprehensive therapeutic strategies. St Louis: Mosby; 2002. 4. Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI. Fitzpatrick’s dermatology in general medicine. 6th ed. New York: McGraw-Hill; 2003. 5. Bolognia JL, Jorizzo JL, Rapini RP. Dermatology. New York: Mosby; 2003.
Lymphangioma circumscriptum: Treatment with hypertonic saline sclerotherapy Joseph B. Bikowski, MD,a and Anna Margarita G. Dumont, MS-IIIb Columbus, Ohio, and Erie, Pennsylvania A 38-year-old woman came for treatment of multiple clear vesicles and hemorrhagic papules on the posterior aspect of the right shoulder and the right axillary vault of 5 years’ duration. These lesions would spontaneously manifest as clear or blood-filled vesicles (or both) and appear to be exacerbated by physical contact from certain articles of clothing. A biopsy was done and the results revealed lymphangioma circumscriptum. The purpose of this case study was to evaluate a new form of treatment of lymphangioma circumscriptum with the use of 23.4% hypertonic saline sclerotherapy. The patient’s lymphangioma circumscriptum significantly resolved with minimal side effects, such as mild hyperpigmentation. Decreased sensitivity was noted and no further treatment was indicated. This case showed that hypertonic saline 23.4% solution can be effective in treating the appearance of vesicles containing clear fluid or lymph and those containing red blood cells in superficial lymphangiomas and that this treatment can be considered for longterm management of lymphangioma circumscriptum. ( J Am Acad Dermatol 2005;53:442-4.)
A
38-year-old woman presented with multiple clear vesicles and hemorrhagic papules on the posterior aspect of the right shoulder of 5 years’ duration. The pertinent medical history included increased sensitivity to touch of the affected area. These lesions would spontaneously manifest as clear and/or blood-filled vesicles and appear to be exacerbated by physical contact from certain articles of clothing. The patient had no known drug allergies, was not taking any medications, and did have a family history of acne and skin cancer. One month before the initial dermatologic consultation, her primary care physician performed a biopsy of the lesion; it was diagnosed as lymphangioma circumscriptum with associated mild chronic inflammation that was consistent with mechanical irritation. Examination revealed multiple minute discrete and confluent vesicles filled with either clear fluid or blood encompassing an area of approximately 16 3 16 cm across the posterior aspect of the right shoul-
From the Department of Dermatology, Ohio State University, Columbus,a and Lake Erie College of Osteopathic Medicine.b Funding sources: None. Conflicts of interest: None. This study has been accepted for the Philadelphia College of Osteopathic Medicine MEDNet Poster Session held at the 2005 Annual Pennsylvania Osteopathic Medical Association Clinical Assembly/Convention on May 12, 2005. Reprint requests: Joseph B. Bikowski, MD, 500 Chadwick St, Sewickley, PA 15143. E-mail: [email protected]. 0190-9622/$30.00 ª 2005 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2005.04.086
442
der, extending from the medial right scapular border to the right posterior axillary fold and from the superior to the inferior border of the right scapula. A repeat biopsy was performed from the center of the involved area at that visit; it confirmed the original histologic findings. Imiquimod 5% cream (Aldara) was prescribed to be used nightly as a therapeutic trial because of its ability to induce antiangiogenic cytokines.1 At the 6-week follow-up visit there was no improvement and within the preceding 24 to 48 hours the lesions had become more prominent (Fig 1). Because of the dilation of lymphatic and vascular elements, sclerotherapy was considered. Three of the most prominent central anastomosing vascular lesions were injected with 0.5 mL of 23.4% hypertonic saline solution. At the 1-month follow-up visit, complete resolution of these previously treated lesions was determined (Fig 2). Therefore an additional 10 to 15 lymphatic channels were injected with 23.4% hypertonic saline solution. At the most recent follow-up visit, lymphangiomas could not be detected and the patient claimed: ‘‘this is the best I have been in three years.’’ The patient was able to touch her right shoulder without experiencing increased sensitivity. Postinflammatory erythema and hyperpigmentation of the previously involved area were minimal. No further treatment was indicated.
DISCUSSION Lymphatic malformations, also known as lymphangiomas, are a hyperproliferation of the lymphatic vessels that constitute approximately 4% of all
J AM ACAD DERMATOL
Bikowski and Dumont 443
VOLUME 53, NUMBER 3
Fig 1. Multiple clear vesicles and hemorrhagic papules on posterior aspect of right shoulder.
vascular tumors and approximately 26% of benign vascular tumors in children.2 In this case report, the patient presented with lymphangioma circumscriptum, which is characterized by subcutaneous lymphatic cisterns that communicate through dilated channels with superficial thin-walled vesicles on skin surfaces. The vesicles typically contain lymph, but may also contain red blood cells, such as in our patient. Lymphangiography has been used to diagnose lymphangioma circumscriptum. It shows the presence of a normal lymphatic network surrounding the sacs and confirms the absence of any communication between the sacs and the normal lymphatic network. Magnetic resonance imaging has also proven to be useful in evaluating lymphangioma circumscriptum; it can be used before surgery to limit the risk of recurrence, but may not be reliable for detecting smaller lymphatic cisterns.3 The ‘‘gold’’ standard of noninvasive examination of the venous system is duplex ultrasound, which allows direct visualization of veins and allows identification of flow through the venous valves. Duplex ultrasound is often used to uncover hidden sources of reflux for pretreatment evaluation of sclerotherapy for peripheral veins and to delineate reflux sources when patients experience poor results from sclerotherapy.4 For the treatment of lymphangioma circumscriptum, the main indications are usually cosmetic but can also be to control complications such as infection, hemorrhage, and pain. If the disease is asymptomatic, it is often advised to use the ‘‘wait and watch’’ approach. The only cure is to remove the superficial component as well as the deeper lymphatic cisterns through surgical destruction or laser ablation of lesions. All other treatments are palliative to control symptoms. Electrocautery, cryotherapy, and carbon dioxide laser can also be used to reduce the risk of infection and to reduce lymphorrhea. Another palliative treatment reported to be helpful is sclerotherapy injection.3 Sclerotherapy and resection can be
Fig 2. A, Prominent central anastomosing vascular lesions and dilation of lymphatic channels on posterior aspect of right shoulder. B, Complete resolution of vascular lesion and lymphatic dilation.
used for long-term management for appearance and functional issues of lymphatic malformations.4 The optimal sclerosing agent is one that induces panendothelial destruction and possesses no systemic toxicity. It should produce local endothelial destruction extending to the entire adventitia with minimal thrombus formation, which can induce an inflammatory reaction leading to fibrosis and eventually obliteration of the vessel lumen.5 Sclerosing agents are classified into 3 groups on the basis of chemical structure and mechanism of injury to the endothelium. Hyperosmotic agents, such as hypertonic saline solution or hypertonic saline and dextrose, involve endothelial cell damage through dehydration.5 Detergent sclerosants (eg, polidocanol, sodium tetradecyl sulfate, sodium morrhuate) cause vascular injury by altering the surface tension around endothelial cells.5 Chemical irritants (eg, polyiodinated iodine) injure cells by acting as corrosives, and their cauterizing effect may be due to the associated heavy metal.5 Side effects of sclerotherapy can be minimized by a fastidious technique, understanding appropriate maneuvers if something is believed to go wrong, and appreciating management options available for adverse sequelae.5 Minor side effects and complications of sclerotherapy include the following: purple- brown hyperpigmentation after sclerotherapy,
444 Bikowski and Dumont
J AM ACAD DERMATOL SEPTEMBER 2005
telangiectatic matting, cutaneous ulcerations and necrosis, superficial thrombophlebitis, pulmonary embolism, and arterial injection.
containing red blood cells in superficial lymphangiomas. Treatment with hypertonic saline sclerotherapy can be considered for long-term management of lymphangioma circumscriptum.
CONCLUSION Sclerotherapy can be an effective palliative treatment of lymphangioma circumscriptum. The key factor in producing successful sclerotherapy is to determine the minimal volume and minimal concentration of the appropriate sclerosing agent. The only sclerosing agents in the United States approved by the Food and Drug Administration are sodium tetradecyl sulfate (Sotradecol), sodium morrhuate (fatty acids in cod liver oil), and hypertonic saline (18%-30%).5 To our knowledge ours is the first reported case showing that hypertonic saline solution 23.4% is effective in treating the appearance of vesicles containing clear fluid or lymph and those
REFERENCES 1. Diego ME, Haynes HA, Li VW. Antiangiogenic treatment of pyogenic granulomas [poster abstract 217]. J Am Acad Dermatol 2004;50(Suppl):S57. 2. Elder D, Elenitsas R, Jaworsky C, Johnson B Jr, editors. Lever’s histopathology of the skin. 8th ed. Philadelphia: LippincottRaven; 1997. 3. Lebwohl MG, Heymann WR, Berth-Jones J, Coulson L. Treatment of skin disease: comprehensive therapeutic strategies. St Louis: Mosby; 2002. 4. Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI. Fitzpatrick’s dermatology in general medicine. 6th ed. New York: McGraw-Hill; 2003. 5. Bolognia JL, Jorizzo JL, Rapini RP. Dermatology. New York: Mosby; 2003.