Lymphangioma Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 18, 2012
 

History

History findings are as follows:

  • Lymphangioma circumscriptum: The typical history involves a small number of vesicles on the skin at birth or shortly after. In subsequent years, they tend to increase in number, and the area of skin involved continues to expand. Vesicles or other skin abnormalities may not be noticed until several years after birth. Usually, lesions are asymptomatic, but, occasionally, patients may have spontaneous episodes of minor bleeding and copious drainage of clear fluid from ruptured vesicles.
  • Cavernous lymphangioma: Typically, during infancy, a solitary rubbery nodule with no skin changes becomes evident in a single location, such as the face, trunk, or extremity. These lesions often have a fast growth phase similar to that of raised hemangiomas. No family history of prior lymphangiomas is described.
  • Cystic hygroma: Soon after birth, the infant is noted to have a deep subcutaneous cystic swelling, usually in the axilla, base of the neck, or groin. If these lesions are drained, they tend to fill up rapidly with lymph fluid. The lesions tend to grow and increase to a large size if they are not completely excised at surgery.
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Physical

Physical findings are as follows:

  • Lymphangioma circumscriptum: Lymphangioma circumscriptum involves small clusters of vesicles measuring about 2-4 mm. These clear vesicles can vary from pink to red to black secondary to hemorrhage.[1] Although it may appear localized to the dermis, this neoplasm frequently extends deeply and laterally. The lesions can have a warty appearance on their surface; as a result, they are often confused with warts. Shah et al report a lymphangioma presenting on the penis.[5]
  • Cavernous lymphangioma: Typically, cavernous lymphangiomas appear as subcutaneous nodules with a rubbery consistency. They may have large dimensions. The overlying skin has no lesions or changes. The area of involvement varies, ranging from lesions smaller than 1 cm in diameter to larger lesions that involve an entire limb.
  • Cystic hygroma: Cystic hygromas are usually larger than cavernous lymphangiomas, and they more commonly occur in the neck and parotid area. Often, deep cavernous lymphangiomas are not evident on superficial examination, but cystic hygromas are detected with ease because of their size and location. These large cystic lesions are soft and translucent. Congenital and acquired lymphangiomas of the vulva are rare.[6] Diffuse lymphangiomas may go unnoticed, evident as asymptomatic, erythematous flat, indurated, or atrophic plaques with or without swelling with or without any surface changes.

Lymphangiomas may affect a number of sites, including the scrotum. Multilocular lymphangiomatosis of the scrotum is unusual.[7] The site of a lymphangioma may determine its clinical signs and symptoms. For example, a lacrimal gland lymphangioma may result in acute proptosis and inferonasal displacement of an eye.[8]

Note the images below.

A 44-year-old woman with plaque on her forearm sinA 44-year-old woman with plaque on her forearm since birth that clinically appeared to be typical lymphangioma circumscriptum. Histologically, however, it had features of both hemangioma and lymphangioma. Close-up demonstrating the clinical morphology to Close-up demonstrating the clinical morphology to better advantage.
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Causes

The reason that these embryonic lymph sacs remain disconnected from the rest of the lymphatic system is not known.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Geover Fernandez, MD, FAAD  Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School

Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Carrie L Kovarik, MD  Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Patel GA, Siperstein RD, Ragi G, Schwartz RA. Zosteriform lymphangioma circumscriptum. Acta Dermatovenerol Alp Panonica Adriat. Dec 2009;18(4):179-82. [Medline].

  2. Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol. Jul 2009;34(5):566-9. [Medline].

  3. Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol. May 1976;94(5):473-86. [Medline].

  4. Itakura E, Yamamoto H, Oda Y, Furue M, Tsuneyoshi M. VEGF-C and VEGFR-3 in a series of lymphangiomas: is superficial lymphangioma a true lymphangioma?. Virchows Arch. Mar 2009;454(3):317-25. [Medline].

  5. Shah A, Meacock L, More B, Chandran H. Lymphangioma of the penis: a rare anomaly. Pediatr Surg Int. Apr 2005;21(4):329-30. [Medline].

  6. Mehta V, Nayak S, Balachandran C, Monga P, Rao R. Extensive congenital vulvar lymphangioma mimicking genital warts. Indian J Dermatol. 2010;55(1):121-2. [Medline]. [Full Text].

  7. Wong R, Melnyk M, Tang SS, Nguan C. Scrotal lymphangiomatosis: a case report. Can Urol Assoc J. Feb 2012;6(1):E11-4. [Medline]. [Full Text].

  8. Bagheri A, Amoohashemi N, Salour H, Yazdani S. Lacrimal Gland Lymphangioma: Report of a Case and Review of Literature. Orbit. Feb 16 2012;[Medline].

  9. Horn LC, Kuhndel K, Pawlowitsch T, Leo C, Einenkel J. Acquired lymphangioma circumscriptum of the vulva mimicking genital warts. Eur J Obstet Gynecol Reprod Biol. Nov 1 2005;123(1):118-20. [Medline].

  10. Aggarwal K, Gupta S, Jain VK, Marwah N. Congenital lymphangioma circumscriptum of the vulva. Indian Pediatr. May 2009;46(5):428-9. [Medline].

  11. Amini S, Kim NH, Zell DS, Oliviero MC, Rabinovitz HS. Dermoscopic-histopathologic correlation of cutaneous lymphangioma circumscriptum. Arch Dermatol. Dec 2008;144(12):1671-2. [Medline].

  12. Arpaia N, Cassano N, Vena GA. Dermoscopic features of cutaneous lymphangioma circumscriptum. Dermatol Surg. Jun 2006;32(6):852-4. [Medline].

  13. Ozeki M, Fukao T, Kondo N. Propranolol for intractable diffuse lymphangiomatosis. N Engl J Med. Apr 7 2011;364(14):1380-2. [Medline].

  14. Werner JA, Eivazi B, Folz BJ, Dünne AA. [State of the art of classification, diagnostics and therapy for cervicofacial hemangiomas and vascular malformations]. Laryngorhinootologie. Dec 2006;85(12):883-91. [Medline].

  15. Mirza B, Ijaz L, Saleem M, Sheikh A. Different Modalities Used to Treat Concurrent Lymphangioma of Chest wall and Scrotum. J Cutan Aesthet Surg. Sep 2010;3(3):189-90. [Medline]. [Full Text].

  16. Haas AF, Narurkar VA. Recalcitrant breast lymphangioma circumscriptum treated by UltraPulse carbon dioxide laser. Dermatol Surg. Aug 1998;24(8):893-5. [Medline].

  17. Bikowski JB, Dumont AM. Lymphangioma circumscriptum: treatment with hypertonic saline sclerotherapy. J Am Acad Dermatol. Sep 2005;53(3):442-4. [Medline].

  18. Ahn SJ, Chang SE, Choi JH, Moon KC, Koh JK, Kim DY. A case of unresectable lymphangioma circumscriptum of the vulva successfully treated with OK-432 in childhood. J Am Acad Dermatol. Nov 2006;55(5 Suppl):S106-7. [Medline].

  19. Okazaki T, Iwatani S, Yanai T, et al. Treatment of lymphangioma in children: our experience of 128 cases. J Pediatr Surg. Feb 2007;42(2):386-9. [Medline].

  20. Katz MS, Finck CM, Schwartz MZ, Moront ML, Prasad R, Timmapuri SJ, et al. Vacuum-assisted closure in the treatment of extensive lymphangiomas in children. J Pediatr Surg. Feb 2012;47(2):367-70. [Medline].

  21. Rattan KN, Kajal P, Kadian YS, Gupta R. Haemorrhage in a scrotal lymphangioma in a child: a rarity. Afr J Paediatr Surg. Jul-Dec 2009;6(2):110-1. [Medline].

  22. Emanuel PO, Lin R, Silver L, Birge MB, Shim H, Phelps RG. Dabska tumor arising in lymphangioma circumscriptum. J Cutan Pathol. Jan 2008;35(1):65-9. [Medline].

  23. Pearce JM, Griffin D, Campbell S. Cystic hygromata in trisomy 18 and 21. Prenat Diagn. Sep-Oct 1984;4(5):371-5. [Medline].

  24. Marchese C, Savin E, Dragone E, et al. Cystic hygroma: prenatal diagnosis and genetic counselling. Prenat Diagn. May-Jun 1985;5(3):221-7. [Medline].

 
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A 44-year-old woman with plaque on her forearm since birth that clinically appeared to be typical lymphangioma circumscriptum. Histologically, however, it had features of both hemangioma and lymphangioma.
Close-up demonstrating the clinical morphology to better advantage.
A 16-year-old obese boy with large unilateral verrucous lymphangioma.
 
 
 
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