Diagnostic Considerations

Lymphangioma circumscriptum may resemble warts, metastatic carcinoma of the skin, lymphangiectasis, herpes simplex, and herpes zoster.^[3] Metastasis of a papillary thyroid microcarcinoma may appear as a cystic lymphangioma of the neck.^[24]

Other cysts and soft subcutaneous masses should be considered. Oral and cutaneous lymphangioma-like Kaposi sarcoma may require distinction.^[25]

Occasionally, acquired lymphangioma circumscriptum of the vulva may mimic genital warts, appearing as verrucous plaques,^{[26, 27, 28, 29]}although these common viral infections may occur with the lymphangiomas and display overlapping clinical features.^[30]Resulting from interference in the lymph drainage, acquired lymphangioma circumscriptum is often related linked to oncological surgical procedures.

Genital lymphangiomas may be complicated by cellulitis.^[1]

Cystic hygroma can be associated with the following congenital disorders:

Turner syndrome
Chromosomal aneuploidy
Hydrops fetalis
Down syndrome and other trisomy disorders^[31]
Fetal alcohol syndrome
Noonan syndrome^[32]
Several other congenital syndromes

Patients with cystic hygroma should undergo cytogenetic analysis for chromosomal aneuploidy. Parents should receive genetic counseling because aneuploidic conditions can recur in subsequent pregnancies.^[33]

Differential Diagnoses

Workup

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].
Bagheri A, Amoohashemi N, Salour H, Yazdani S. Lacrimal Gland Lymphangioma: Report of a Case and Review of Literature. Orbit. 2012 Feb 16. [QxMD MEDLINE Link].
Patel GA, Siperstein RD, Ragi G, Schwartz RA. Zosteriform lymphangioma circumscriptum. Acta Dermatovenerol Alp Panonica Adriat. 2009 Dec. 18(4):179-82. [QxMD MEDLINE Link].
Shah A, Meacock L, More B, Chandran H. Lymphangioma of the penis: a rare anomaly. Pediatr Surg Int. 2005 Apr. 21(4):329-30. [QxMD MEDLINE Link].
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].
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. [QxMD MEDLINE Link]. [Full Text].
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].
Saccone G, Di Meglio L, Di Meglio L, Zullo F, Locci M, Zullo F, et al. Prenatal ultrasound diagnosis of fetal chest wall cystic lymphangioma: An Italian case series. Eur J Obstet Gynecol Reprod Biol. 2019 Mar 18. 236:139-142. [QxMD MEDLINE Link].
Park SH, Moon SK, Sung JY. Sonographic findings in a case of scrotal lymphangioma. J Clin Ultrasound. 2014 May. 42(4):234-6. [QxMD MEDLINE Link].
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].
Massa AF, Menezes N, Baptista A, Moreira AI, Ferreira EO. Cutaneous Lymphangioma circumscriptum - dermoscopic features. An Bras Dermatol. 2015 Mar-Apr. 90(2):262-4. [QxMD MEDLINE Link]. [Full Text].
Jha AK, Lallas A, Sonthalia S. Dermoscopy of cutaneous lymphangioma circumscriptum. Dermatol Pract Concept. 2017 Apr. 7 (2):37-38. [QxMD MEDLINE Link].
Werner JA, Eivazi B, Folz BJ, Dünne AA. [State of the art of classification, diagnostics and therapy for cervicofacial hemangiomas and vascular malformations]. Laryngorhinootologie. 2006 Dec. 85(12):883-91. [QxMD MEDLINE Link].
Kolay SK, Parwani R, Wanjari S, Singhal P. Oral lymphangiomas - clinical and histopathological relations: An immunohistochemically analyzed case series of varied clinical presentations. J Oral Maxillofac Pathol. 2018 Jan. 22 (Suppl 1):S108-S111. [QxMD MEDLINE Link].
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].
Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol. 1976 May. 94(5):473-86. [QxMD MEDLINE Link].
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].
Marchese C, Savin E, Dragone E, et al. Cystic hygroma: prenatal diagnosis and genetic counselling. Prenat Diagn. 1985 May-Jun. 5(3):221-7. [QxMD MEDLINE Link].
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].
Jenkinson HA, Wilmas KM, Silapunt S. Sodium Tetradecyl Sulfate: A Review of Clinical Uses. Dermatol Surg. 2017 Apr 19. [QxMD MEDLINE Link].
Ekelem C, Thomas L, Van Hal M, Valdebran M, Lotfizadeh A, Mlynek K, et al. Radiofrequency Therapy and Noncosmetic Cutaneous Conditions. Dermatol Surg. 2019 Mar 18. [QxMD MEDLINE Link].
Mirza B, Ijaz L, Saleem M, Sheikh A. Different Modalities Used to Treat Concurrent Lymphangioma of Chest wall and Scrotum. J Cutan Aesthet Surg. 2010 Sep. 3(3):189-90. [QxMD MEDLINE Link]. [Full Text].
Porwal PK, Dubey KP, Morey A, Singh H, Pooja S, Bose A. Bleomycin Sclerotherapy in Lymphangiomas of Head and Neck: Prospective Study of 8 Cases. Indian J Otolaryngol Head Neck Surg. 2018 Mar. 70 (1):145-148. [QxMD MEDLINE Link].
Emer J, Gropper J, Gallitano S, Levitt J. A case of lymphangioma circumscriptum successfully treated with electrodessication following failure of pulsed dye laser. Dermatol Online J. 2013 Mar 15. 19(3):2. [QxMD MEDLINE Link].
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].
Sasaki R, Negishi K, Akita H, Suzuki K, Matsunaga K. Successful Treatment of Congenital Lymphangioma Circumscriptum of the Vulva with CO2 and Long-Pulsed Nd:YAG Lasers. Case Rep Dermatol. 2014 Jan. 6(1):1-4. [QxMD MEDLINE Link]. [Full Text].
Torezan LA, Careta MF, Osorio N. Intra-Oral Lymphangioma Successfully Treated Using Fractional Carbon Dioxide Laser. Dermatol Surg. 2013 Feb 22. [QxMD MEDLINE Link].
Bikowski JB, Dumont AM. Lymphangioma circumscriptum: treatment with hypertonic saline sclerotherapy. J Am Acad Dermatol. 2005 Sep. 53(3):442-4. [QxMD MEDLINE Link].
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. 2006 Nov. 55(5 Suppl):S106-7. [QxMD MEDLINE Link].
Okazaki T, Iwatani S, Yanai T, et al. Treatment of lymphangioma in children: our experience of 128 cases. J Pediatr Surg. 2007 Feb. 42(2):386-9. [QxMD MEDLINE Link].
Yoon KR, Mo AJ, Park SH, Na YS, Park ST. Carvenous lymphangioma of the vulva. Obstet Gynecol Sci. 2015 Jan. 58(1):77-9. [QxMD MEDLINE Link]. [Full Text].
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. 2012 Feb. 47(2):367-70. [QxMD MEDLINE Link].

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.

of 3

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 Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

Tables

Contributor Information and Disclosures

What would you like to print?