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Year : 2020  |  Volume : 21  |  Issue : 3  |  Page : 206-208

Primary lymphangioma circumscriptum around external urethral meatus in a male child: A rare presentation

Department of Dermatology, Pt. B.D. Sharma Postgraduate Institute of Medical Sciences, Rohtak, Haryana, India

Date of Submission10-Feb-2020
Date of Decision21-Feb-2020
Date of Acceptance29-Mar-2020
Date of Web Publication30-Jun-2020

Correspondence Address:
Dr. Jayanti Singh
House No. 3753, Urban Estate, Jind - 126 102, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_21_20

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Primary lymphangioma circumscriptum (LC) is a superficial lymphatic malformation, presenting as multiple fluid-filled grouped vesicular lesions which may be associated with slight hemorrhages. We report a rare case of primary LC with exclusive involvement of area around the external urethral meatus in a 10-year-old male child. We treated the patient with radiofrequency ablation under topical anesthesia with eutectic mixture of local anesthetics (EMLA). No recurrence has been observed in the past 1 year. LC must be included in differentials when presented with vesicular lesions near the external urethral meatus in male pediatric patients.

Keywords: External urethral meatus, lymphangioma circumscriptum, male child, radiofrequency ablation

How to cite this article:
Singh J, Dayal S, Mothsra J, Sahu P. Primary lymphangioma circumscriptum around external urethral meatus in a male child: A rare presentation. Indian J Paediatr Dermatol 2020;21:206-8

How to cite this URL:
Singh J, Dayal S, Mothsra J, Sahu P. Primary lymphangioma circumscriptum around external urethral meatus in a male child: A rare presentation. Indian J Paediatr Dermatol [serial online] 2020 [cited 2021 Jul 25];21:206-8. Available from: https://www.ijpd.in/text.asp?2020/21/3/206/288496

  Introduction Top

Lymphangiomas are benign malformations of lymphatic system, formed because of a focal abnormality in the lymphatic system. Based on the depth and size of the abnormal lymphatics, they are classified as either superficial or deep seated. The superficial lymph vessels are affected in lymphangioma circumscriptum (LC) and the involvement of deep lymphatics occurs in cavernous lymphangioma and cystic hygroma.[1]

We report a case of primary LC exclusively involving the area adjoining the opening of external urethral meatus, due to its rare presentation and diagnostic confusio. To the best of our knowledge, only one such case has been previously reported.[2]

  Case Report Top

A 10-year-old boy presented to our dermatology outpatient department with complaints of fluid-filled lesions near the urethral opening. These lesions had been developing for the past 5 years and during the past 2 months, were also associated with occasional burning micturition. On examination, we observed multiple grouped clear fluid-filled vesicles of size 0.1 mm–0.3 mm. The vesicles had slight hemorrhages and encircled the external urethral meatus [Figure 1]. There was no lymphedema of the penis or inguinal lymphadenopathy. Tzanck smear was negative. Routine investigations including complete hemogram, liver function test, renal function test, and urine examination were within normal limits. HBsAg, anti-hepatitis C virus antibody, and HIV were negative. Abdominal, pelvic, and penile ultrasonography were normal. Differential diagnosis of LC, angiokeratoma, and recurrent herpes genitalis were considered. Histopathological examination of the lesions showed dilated lymphatic vessels and mononuclear inflammatory cells throughout the dermis [Figure 2]. Based on these findings, patient was diagnosed with primary LC surrounding external urethral meatus. After topical anesthesia with EMLA, patient was treated with radiofrequency ablation (RFA). Patient was followed up for 1 year with no recurrence and complications.
Figure 1: Multiple clear fluid-filled vesicles with hemorrhages surrounding the external urethral meatus in a male child

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Figure 2: Dilated lymphatic vessels and mononuclear inflammatory cells throughout the dermis (H and E stain, ×40)

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  Discussion Top

LC is a superficial lymphatic malformation characterized by dilation of lymphatic vessels in dermis and subcutaneous tissue.[3] It can be classified into primary/congenital and acquired LC.[1]

In 1976, Whimster described the pathogenesis of congenital LC. During the embryonic development of lymphatic system, lymphatic cisterns arising from primitive lymph sac fail to connect with the rest of the lymphatic system, and lie in the deep subcutaneous plane. A thick coat of muscle fibers surrounds the sequestered lymphatic cisterns which causes rhythmic contractions. Out-pouching of these channels occurs due to increased intramural pressure, which is seen as vesicles in LC on the surface of the skin.[4] Mutations in the PIK3CA gene have been found in association with lymphatic malformations.[5]

LC is usually present as superficial clear grouped vesicles, sometimes with reddish tinge due to hemorrhage. Other clinical features can include the leakage of lymph, hemorrhage, and occasional ulcerations leading to secondary infections. It favors the proximal limbs and chest but can occur in any cutaneous site including the oral cavity and genitalia.[3] Over genitalia, these vesicles can mimic molluscum contagiosum, genital warts, angiokeratomas, and genital herpes. A definitive diagnosis is reached histopathologically which shows dilated flat endothelium-lined channels in the dermis, it may also be seen in subcutaneous and deeper tissue. The dilated lymphatics may contain red blood cells, lymphocytes, and neutrophils. There may be acanthosis and hyperkeratosis of the overlying epidermis.[3] On immunohistochemistry, it is positive for CD31 and D2-40.[1] In our case, lesions occurred in the first decade of patient's life. It was not associated with any underlying pathology. Hence, the patient was diagnosed with primary LC.

If the condition is asymptomatic, wait, and watch policy is advised.[6] Treatment is done with the aim of reducing complications and cosmetic concerns. Palliative treatment has been done using electrocautery, electrofulgration, RFA, ablative CO2 laser, pulse dye laser, intense pulsed light, sclerotherapy using hypertonic saline, and topical imiquimod.[7],[8] Definite treatment is surgical removal of superficial and deep malformation of lymphatics. However, the postsurgical recurrence rate is reported in up to 20%–50% of cases.[9] We treated our patient with RFA due to the high recurrence rate and postoperative discomfort associated with the surgery. The use of EMLA obviated the need of injectable local anesthetic, allaying the fear of pain in the pediatric patient. Till date, no recurrence has occurred in the patient.

To the best of our knowledge, only a single case of primary LC with exclusive involvement of area near the external urethral meatus has been described.[2] We report this case of primary LC due to its rare presentation near the external urethral meatus in a 10-year-old Indian male child. Lesions over genitalia may lead to significant psychological distress in the child and parents. Arriving at a definitive diagnosis and proper counseling of the patient and parents regarding the nature and recurrence of the lesions is necessary. Treatment with RFA is safe and can be done under topical anesthesia with EMLA as an outpatient procedure with minimal discomfort in pediatric patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Peachey RD, Lim CC, Whimster IW. Lymphangioma of skin. A review of 65 cases. Br J Dermatol 1970;83:519-27.  Back to cited text no. 1
Daklan S, Daryakar A. Lymphangioma circumscriptum of glans penis: A report of two cases. Dermatol Online J 2018;24. pii: 13030/qt9pr342qv.  Back to cited text no. 2
Mortimer PS, Gordan K. Disorders of Lymphatic Vessels. In: Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. Oxford: John Wiley and Sons; 2016. p. 105.34-105.37.  Back to cited text no. 3
Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol 1976;94:473-86.  Back to cited text no. 4
Luks VL, Kamitaki N, Vivero MP, Uller W, Rab R, Bovée JV, et al. Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA. J Pediatr 2015;166:1048-540.  Back to cited text no. 5
Kokcam I. Lymphangioma circumscriptum of the penis: A case report. Acta Dermatovenerol Alp Pannonica Adriat 2007;16:81-2.  Back to cited text no. 6
Wang JY, Liu LF, Mao XH. Treatment of lymphangioma circumscriptum with topical imiquimod 5% cream. Dermatol Surg 2012;38:1566-9.  Back to cited text no. 7
Puri N. Treatment options of lymphangioma circumscriptum. Indian Dermatol Online J 2015;6:293-4.  Back to cited text no. 8
[PUBMED]  [Full text]  
Sheu JY, Chung HJ, Chen KK, Lin AT, Chang YH, Wu HH, et al. Lymphangioma of male exogenital organs. J Chin Med Assoc 2004;67:204-6.  Back to cited text no. 9


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