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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 123-125

Lymphangioma circumscriptum of scrotum: a rare case


1 Department of Pathology, Rampurhat Government Medical College & Hospital, Rampurhat, India
2 Department of Pathology, Deben Mahata Government Medical College & Hospital, Hatuara, India
3 Department of Dermatology, Rampurhat Government Medical College & Hospital, Rampurhat, India
4 Department of Ophthalmology, Rampurhat Government Medical College & Hospital, Rampurhat, West Bengal, India

Date of Submission15-Jul-2021
Date of Acceptance12-Jan-2022
Date of Web Publication23-Mar-2022

Correspondence Address:
Dr. Jyoti Prakash Phukan
Department of Pathology, Rampurhat Government Medical College & Hospital, Rampurhat 731224, West Bengal.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mgmj.mgmj_51_21

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  Abstract 

Lymphangioma circumscriptum (LC) is a form of lymphatic hamartoma, which mainly involves the skin and subcutaneous tissues. The mucosa of the mouth, tongue, groin, trunk, and axilla are the most common locations of LC, and the scrotum is a rare site of occurrence. Here we report a case of LC of scrotum occurring in a 58-year-old man without any history of surgery and radiation. The man was presented with multiple small vesicles containing clear fluid in the scrotum and subsequent histopathology confirmed the diagnosis. We are presenting this case for its rare presentation.

Keywords: Lymphangioma circumscriptum, lymphatic hamartoma, scrotum


How to cite this article:
Phukan JP, Sinha A, Das KD, Mazumdar M. Lymphangioma circumscriptum of scrotum: a rare case. MGM J Med Sci 2022;9:123-5

How to cite this URL:
Phukan JP, Sinha A, Das KD, Mazumdar M. Lymphangioma circumscriptum of scrotum: a rare case. MGM J Med Sci [serial online] 2022 [cited 2022 May 17];9:123-5. Available from: http://www.mgmjms.com/text.asp?2022/9/1/123/340586




  Introduction Top


Lymphangioma circumscriptum (LC) is a rare, hamartomatous lymphatic malformation of deep dermal and subcutaneous lymphatics, which is characterized by dilation of lymphatic vessels in the skin and subcutaneous tissue.[1],[2] LC usually presents as small clusters of fluid-filled vesicles. The most common sites of LC are axillary folds, shoulders, chest, tongue, and buccal mucous membrane.[2] The scrotum is a rare site of occurrence. To date, only a few cases of scrotal LC have been described. Here, we are presenting an interesting case LC involving a scrotum in a 58-year-old man.


  Case report Top


A 58-year-old man attended the dermatology outpatient department of our medical college with multiple clusters of small vesicles filled with clear watery fluid involving scrotal skin for a 1-year duration. The patient developed these vesicles a year back, however, there was a recent history of rupture and discharge from some of the vesicles, for which he attended the medical clinic. On examination, the scrotum was studded with tiny, translucent vesicles containing clear fluid, grouped like frogspawn [Figure 1]. He had no history of surgery or radiotherapy in that area nor any significant medical history of sexually transmitted disease.
Figure 1: Clinical picture showing scrotum of the patient, studded with tiny, translucent vesicles containing clear fluid, grouped like frog spawn

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Routine blood investigations and serological tests for syphilis, hepatitis B, hepatitis C, and human immunodeficiency virus (HIV) were done and found to be non-reactive. Routine blood examination reports were within normal limits. A clinical diagnosis of LC was made. A small punch biopsy of one of the vesicles was done and sent to the Department of Pathology for histopathological examination. Histopathology of the excised specimen showed hyperkeratosis and acanthosis of the superficial epidermis with numerous dilated lymphatic channels lined by a thin wall of endothelial cells in the epidermis and papillary dermis filled with eosinophilic proteinous material [Figure 2]. Thus, the diagnosis of LC was confirmed.
Figure 2: Photomicrograph of the lesion showing dilated lymphatic channels containing proteineous material in the epidermis and papillary dermis with areas of acanthosis and hyperkeratosis of overlying epithelium (H&E, ×10)

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The patient was consulted regarding various methods of treatment like surgery, carbon dioxide laser (CO2 laser), and electrocautery. As only surgery was available in our setup, we referred the patient to the surgery department for further treatment.


  Discussion Top


Lymphangioma is a hamartomatous lesion that develops within the lymphatic system. Lymphangiomas can be divided into various subtypes such as LC, cavernous lymphangioma, cystic hygroma, and lmphangioendothelioma.[3] LC is the most common form of cutaneous lymphangioma and it may be congenital or acquired in origin.[3] In the year 1879, Fox and Fox described this condition and called lymphangiectodes and the term LC was coined by Morris in the year 1889.[4] LC mainly occurs in axillary folds, shoulders, proximal part of limbs, tongue, and buccal mucus membranes.[2] The scrotum is one of the rarest sites for LC.[5] They mainly occur spontaneously, but may also occur after irradiation, local infections, and after vasectomy.[6]

The exact cause of LC is not known. The pathogenesis of LC was first described by Fox and Fox in 1879. Whimster, in 1976, described that LC results from the collection of lymphatic reservoirs which receive lymphatics from nearby tissues, but they are not connected to the lymphatic system and thus fail to drain to the normal lymphatic system.[7] Acquired LC can occur probably due to injury to deep collecting lymphatics, which may be caused injury to deep collecting lymphatics.[3] The causes of injury vary from radiotherapy-related damage, infections like filariasis, lymphogranuloma venereum, tuberculosis, previous surgery like a vasectomy. However, in our patient, there is no such history of infections, radiotherapy, or surgical intervention.

Clinical presentation of LC varies from translucent to hazy vesicles of different sizes which are grouped like frogspawn, or less commonly diffuse swelling of the area. The vesicles usually contain clear lymphatics but may become hemorrhagic. The vesicles sometimes give a warty appearance.[8] LC generally remains asymptomatic but may be complicated by rupture leading to secondary infection. Our patient presented with multiple translucent vesicles of varying sizes involving almost the whole scrotum with rupture of a few vesicles. Although the clinical presentation is quite diagnostic, diagnostic confirmation is done by excision of the mass and histopathological examination.

Histopathology of the lesion shows dilated lymphatic vessels in the epidermis and papillary dermis often with accompanying acanthosis and hyperkeratosis of the epidermis. Other ancillary investigations like ultrasonography and dermoscopy also help in the diagnosis. Ultrasonography helps to determine the extent of the disease. Dermoscopy usually reveals a lacunar or saccular pattern which is due to the presence of lymphatic fluid varying from light brown to purple, which shows a hypopyon-like appearance due to the accumulation of blood in the lowest part of a lacuna.[9]

The indication of definitive treatment for LC is mainly cosmetics and prevention of infection. There is no standard therapy for LC. Traditional treatment of LC is the surgical removal of the lesion. Various other treatment modalities are also available. They include sclerotherapy, superficial radiotherapy, cryotherapy, pulse dye laser, CO2 laser, intense pulse light, and electrocautery.[10] As we do not have the newer treatment facilities, we referred the patient to the surgery department. However, we lose the follow-up of the patient after referral.


  Conclusion Top


This case has been presented due to the rare site of occurrence. Although clinical features are much diagnostic, a clinician must be aware of this condition and should send tissue for biopsy to confirm diagnosis before commencing definitive treatment.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gopal AP, Robertez AS Cutaneous lymphangioma circumscriptum: Frog spawn on the skin. Int J Dermatol 2009;48:1290-5.  Back to cited text no. 1
    
2.
Esquivias Gómez JI, Miranda-Romero A, Cuadrado Vallés C, Bajo del Pozo C, Sánchez Sambucety P, Martínez Fernández M, et al. Lymphangioma circumscriptum of the vulva. Cutis 2001;67:229-32.  Back to cited text no. 2
    
3.
Haroon S, Hasan SH Lymphangioma circumscriptum in the scrotum: A case report. J Med Case Rep 2012;6:233.  Back to cited text no. 3
    
4.
Patel GA, Siperstein RD, Ragi G, Schwartz RA Zosteriform lymphangioma circumscriptum. Acta Dermatovenerol Alp Panonica Adriat 2009;18:179-82.  Back to cited text no. 4
    
5.
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. 5
    
6.
Gomides MDA, Costa LD, Berbert ALCV, Janones RS Cutaneous lymphangioma circumscriptum: The relevance of clinical, dermoscopic, radiological, and histological assessments. Clin Case Rep 2019;7:612-5.  Back to cited text no. 6
    
7.
Sara O, Rassoasmae, Hanane B, Elloudi S, Mernissi RZ Scrotal microcystic lymphangioma: A case report with clinical and dermoscopic aspects. Urol Androl Open J 2020:4:43-5.  Back to cited text no. 7
    
8.
Fakhfakh H, Bouhlel A, Ketata H, Sahnoun A, Bahloul A, Mhiri MN Le lymphangioma kystique scrotal: A propos d’un cas. Andrologie 2005;15:242-4.  Back to cited text no. 8
    
9.
Jha AK, Lallas A, Sonthalia S Dermoscopy of cutaneous lymphangioma circumscriptum. Dermatol Pract Concept 2017;7:37-8.  Back to cited text no. 9
    
10.
Lai CH, Hanson SG, Mallory SB Lymphangioma circumscriptum treated with pulsed dye laser. Pediatr Dermatol 2001;18:509-10.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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