History

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.

Complications

Lymphangioma circumscriptum may occur. Lymphangiomas circumscriptum is associated with minor bleeding, recurrent cellulitis, and lymph fluid leakage. Two cases of lymphangiosarcoma arising from lymphangioma circumscriptum have been reported. However, in both of the patients, the preexisting lesion was exposed to extensive x-ray therapy. Therefore, radiation therapy should be avoided in lymphangiomas.

In cystic hygroma, large cysts can cause dysphagia, respiratory problems, and serious infection if they involve the neck.

A cystic lymphangioma of the scrotum may present as an acute scrotum, owing to the rare complication of hemorrhage.^[22]

A Dabska tumor may rarely arise within lymphangioma circumscriptum.^[23]

Differential Diagnoses

Wong R, Melnyk M, Tang SS, Nguan C. Scrotal lymphangiomatosis: a case report. Can Urol Assoc J. 2012 Feb. 6(1):E11-4. [QxMD MEDLINE Link]. [Full Text].
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Li E, Yoda RA, Keene CD, Moe KS, Chambers C, Zhang MM. Nasolacrimal Lymphangioma Presenting With Hemolacria. Ophthalmic Plast Reconstr Surg. 2020 Feb 24. [QxMD MEDLINE Link].
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Chang MB, Newman CC, Davis MD, Lehman JS. Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: Clinicopathologic study of 11 patients from a single institution and 67 from the literature. Int J Dermatol. 2016 Mar 9. [QxMD MEDLINE Link].
Ersoy AO, Oztas E, Saridogan E, Ozler S, Danisman N. An Unusual Origin of Fetal Lymphangioma Filling Right Axilla. J Clin Diagn Res. 2016 Mar. 10 (3):QD09-11. [QxMD MEDLINE Link].
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Amini S, Kim NH, Zell DS, Oliviero MC, Rabinovitz HS. Dermoscopic-histopathologic correlation of cutaneous lymphangioma circumscriptum. Arch Dermatol. 2008 Dec. 144(12):1671-2. [QxMD MEDLINE Link].
Arpaia N, Cassano N, Vena GA. Dermoscopic features of cutaneous lymphangioma circumscriptum. Dermatol Surg. 2006 Jun. 32(6):852-4. [QxMD MEDLINE Link].
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Kurude AA, Phiske MM, Kolekar KK, Nayak CS. Lymphangiomas: Rare presentations in oral cavity and scrotum in pediatric age group. Indian J Dermatol Venereol Leprol. 2020 Mar-Apr. 86 (2):230. [QxMD MEDLINE Link].
Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol. 2009 Jul. 34(5):566-9. [QxMD MEDLINE Link].
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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. 2009 Mar. 454(3):317-25. [QxMD MEDLINE Link].
Luu YT, Kimmis BD, Bodine JS, et al. Malignancy-associated acquired vulvar lymphangioma circumscriptum: A clinicopathologic study of 71 cases. J Cutan Pathol. 2022 May. 49 (5):426-433. [QxMD MEDLINE Link].
Rattan KN, Kajal P, Kadian YS, Gupta R. Haemorrhage in a scrotal lymphangioma in a child: a rarity. Afr J Paediatr Surg. 2009 Jul-Dec. 6(2):110-1. [QxMD MEDLINE Link].
Emanuel PO, Lin R, Silver L, Birge MB, Shim H, Phelps RG. Dabska tumor arising in lymphangioma circumscriptum. J Cutan Pathol. 2008 Jan. 35(1):65-9. [QxMD MEDLINE Link].
Zhu L, Zhu X, Zhou B, Mao WB, Wu Y, Cheng F. Multiple simple cystic metastases in the lateral neck at presentation with papillary thyroid microcarcinoma: A case report. Medicine (Baltimore). 2021 Jan 29. 100 (4):e23866. [QxMD MEDLINE Link].
Schuch LF, Kovalski LNS, Leite AA, et al. Oral lymphangioma-like Kaposi sarcoma: a Brazilian case report in a scenario of a still high number of HIV infections. Oral Maxillofac Surg. 2022 Mar. 26 (1):171-176. [QxMD MEDLINE Link].
Núñez EC, Peñaranda JM, Alonso MS, Ortiz-Rey JA. Acquired Vulvar Lymphangioma: A Case Series With Emphasis on Expanding Clinical Contexts. Int J Gynecol Pathol. 2014 Mar 27. [QxMD MEDLINE Link].
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. 2005 Nov 1. 123(1):118-20. [QxMD MEDLINE Link].
Aggarwal K, Gupta S, Jain VK, Marwah N. Congenital lymphangioma circumscriptum of the vulva. Indian Pediatr. 2009 May. 46(5):428-9. [QxMD MEDLINE Link].
Gude G, Gupta P, Sharma RK, Rajwanshi A. Primary lymphangioma circumscriptum of the vulva presenting as warty plaques. Australas J Dermatol. 2019 Feb 27. [QxMD MEDLINE Link].
Cestaro G, De Rosa M, Gentile M, Massaron S. A case of HPV and acquired genital lymphangioma: over-lapping clinical features. Ann Ital Chir. 2015 Mar 25. 86:[QxMD MEDLINE Link].
Pearce JM, Griffin D, Campbell S. Cystic hygromata in trisomy 18 and 21. Prenat Diagn. 1984 Sep-Oct. 4(5):371-5. [QxMD MEDLINE Link].
Pérez-Feal P, Moreiras-Arias N, Buján-Bonino C, et al. Disseminated lymphangiomas as a cutaneous manifestation of Noonan syndrome. Clin Exp Dermatol. 2022 Jan. 47 (1):180-182. [QxMD MEDLINE Link].
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Ozeki M, Fukao T, Kondo N. Propranolol for intractable diffuse lymphangiomatosis. N Engl J Med. 2011 Apr 7. 364(14):1380-2. [QxMD MEDLINE Link].
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Haas AF, Narurkar VA. Recalcitrant breast lymphangioma circumscriptum treated by UltraPulse carbon dioxide laser. Dermatol Surg. 1998 Aug. 24(8):893-5. [QxMD MEDLINE Link].
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Media Gallery

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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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

Geover Fernandez, MD, FAAD Staff Physician, Department of Dermatology, Rutgers New Jersey Medical School

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

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

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, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

Lymphangioma Clinical Presentation

History

Lymphangioma circumscriptum

Cavernous lymphangioma

Cystic hygroma

Complications

References

Tables

Contributor Information and Disclosures

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