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 Table of Contents  
ONLINE ONLY ARTICLES - CASE REPORT
Year : 2022  |  Volume : 6  |  Issue : 2  |  Page : 150

Vulval lymphangiectasia post-radiotherapy in cervical cancer: A rare case report


Department of Dermatology, Sri Lakshmi Narayana Institute of Medical Sciences, Pondicherry, India

Date of Submission27-Nov-2020
Date of Decision16-Jan-2021
Date of Acceptance12-Feb-2021
Date of Web Publication26-Aug-2022

Correspondence Address:
C Kanmani Indra
Senior Resident, Department of Dermatology, Sri Lakshmi Narayana Institute of Medical Sciences, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/cdr.cdr_131_20

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  Abstract 


Cutaneous lymphangiectasia is a benign cutaneous disorder of dilatation of the dermal and subcutaneous lymphatic channels, which occurs rarely on the vulva. It can be congenital or acquired developing secondary to tuberculosis, surgery, and radiotherapy. We report the case of a 58-year-old female patient who gives a history of cervical cancer, for which she underwent hysterectomy and postsurgery radiotherapy 3 years back, now presenting with multiple lumps over the vulva, which discharged clear fluid for 1 year. Acquired vulval lymphangiectasia develops 7–15 years postradiotherapy or surgery commonly, while in our case, it developed earlier. On local examination, there were multiple shiny translucent and hyperpigmented “frog-spawn like” vesicles and papules present over the lower midabdomen, mons pubis, and labia majora. Diagnosis of vulval lymphangiectasia was made on the clinical findings and biopsy. Biopsy revealed dilated lymphatic channels in the superficial and deep dermis. CO2 laser treatment was done for the patient.

Keywords: Acquired lymphangioma, cervical cancer, lymphangiectasia


How to cite this article:
Raj P A, Indra C K, Nasrin M I, Jacob NS. Vulval lymphangiectasia post-radiotherapy in cervical cancer: A rare case report. Clin Dermatol Rev 2022;6:150

How to cite this URL:
Raj P A, Indra C K, Nasrin M I, Jacob NS. Vulval lymphangiectasia post-radiotherapy in cervical cancer: A rare case report. Clin Dermatol Rev [serial online] 2022 [cited 2022 Sep 26];6:150. Available from: https://www.cdriadvlkn.org/text.asp?2022/6/2/150/354739




  Introduction Top


Cutaneous lymphangiectasia or acquired lymphangioma represents distended, dermal lymphatics engorged with lymph due to a failure of downstream drainage, which can occur secondary to tuberculosis (TB), surgery, radiotherapy, and trauma. It has to be differentiated from lymphangioma circumscriptum (LC), which is a lymphatic malformation of the deep dermal and subcutaneous lymphatics.

LC is usually present since birth or early childhood although clinically and histologically it resembles lymphangiectasia.[1],[2] There are very few cases of vulval lymphangiectasia reported in the literature.[3] Here, we describe a case of acquired cutaneous lymphangiectasia of the vulva, which developed 3 years postradiotherapy done for cervical cancer.


  Case Report Top


A 58-year-old female patient with no comorbidities presented with multiple lumps over the vulva, which discharged clear watery fluid for 1 year. She did not have any complaints of itching/burning sensation/bleeding/pain over the lesions. The patient gave a history of cervical cancer, for which she underwent a hysterectomy, lymph node dissection, and postsurgery radiotherapy 3 years back. There was no history of TB, trauma, sexually transmitted diseases, and genital ulcer. She is married and has three healthy children. She attained menopause 8 years back.

On examination, the patient is well built and nourished and the vitals are stable.

After taking patient's consent, local genital examination, there were multiple firm to hard nontender, nonmobile, smooth, shiny translucent or flesh-colored and hyperpigmented vesicles and papules resembling the characteristic “frog-spawn like” measuring 0.5 mm × 0.5 mm –2 mm × 2 mm present over the lower midabdomen, mons pubis, and bilateral labia majora and minora discharging clear watery nonfoul-smelling fluid [Figure 1]. Abdominal and per vaginal pelvic examination were normal. There was no vulval lymphoedema/lower limb edema/regional lymphadenopathy. Systemic examination was within normal limits. Complete analyses of liver and renal function tests, blood, urine, and stool were within normal limits. Mantoux test was negative. Screening tests for HIV, hepatitis B surface antigen, hepatitis C virus, and venereal disease research laboratory were nonreactive. Histopathological examination of the lesions revealed thin-walled and dilated lymphatic channels in the papillary dermis lined by a thin wall of endothelial cells. A diagnosis of vulval lymphangiectasia was made based on the clinical findings and histopathology [Figure 2]. The patient was treated with CO2 laser vaporization. The result was favorable with a good cosmetic result and no signs of recurrences were seen during follow-up.
Figure 1: Multiple firm to hard non-tender, non-mobile, smooth, shiny, translucent or flesh-coloured and hyperpigmented vesicles and papules resembling “frog-spawn

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Figure 2: Thin-walled and dilated lymphatic channels in the papillary dermis lined by a thin wall of endothelial cells, along with diffuse inflammatory infiltrate

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  Discussion Top


Cutaneous lymphangiectasias are not true neoplasms or hamartomas. It is a rare disease, occurring secondary to obstruction of lymphatics due to radiation or surgery for cervical cancer or vulva, tubercular lymphadenitis, and Crohn's disease of the vulva.[1],[2],[3] However, there is no difference clinically in lymphangiectasia due to radiation and that due to other causes. Vulval presentation is uncommon and may be misdiagnosed as genital warts, molluscum contagiosum, or TB verrucosa cutis.[2],[3]

The lymphatic vessels of the superficial dermal plexus drain skin through the vertical collecting lymphatics to the deep plexus. Hence, any damage to the deep lymphatics causes back pressure and dermal backflow, with subsequent dilatation of the upper dermal lymphatics, resulting in lymphangiectasia.[2] It usually develops approximately 7–15 years after lymph node dissection and radiotherapy,[4] but in our case, it developed earlier, i.e., within 3 years postradiotherapy. The cause for the early occurrence of lymphangiectasia in this patient is unknown. The disease affects females aged 22–75 years with a mean age of 48 years.[5] Here, we report a case of vulval lymphangiectasia in a 58-year-old female patient who developed it 3 years postradiotherapy done for cervical cancer.

Vulval lymphangiectasia can be either asymptomatic or sometimes associated with pruritus, burning sensation, or pain. It is a benign condition.[6] On clinical examination, it is characterized by thin-walled translucent vesicles filled with clear colorless fluid or sometimes blood-tinged, which may be either scattered or grouped-like frog spawn. Sometimes, they can appear as smooth flesh-colored nodules or rarely has a firm hyperkeratotic appearance.[2],[3],[6] They can also develop lymphoedema or recurrent cellulitis over the vulva.[3],[7] The skin in advanced lymphoedema often becomes thickened and warty and may show lymphangiectases, eventually developing into a malignant change as in postmastectomy lymphangiosarcoma of Stewart and Treves; hence, early detection is critical.[8] Some cases of squamous cell carcinoma are also reported.[9] Rarely, the patient can develop cellulitis, as part of complication.

Histologically, dilated lymphatic channels are seen in the superficial and middermis with few dilated lymphatics in the deep dermis. The overlying epidermis may show varying degrees of hyperkeratosis, papillomatosis, and acanthosis. These dilated lymphatic channels may contain scattered lymphocytes and red blood cells, giving a purplish tinge to the lesion. Other conditions such as mucin secreting metastatic adenocarcinoma mimicking acquired lymphangioma, condyloma, syringoma, and benign lymphangioendothelioma, should be ruled out.[1]

Vulval lymphangiectasis has to be differentiated from LC. LC is a congenital condition in which there is dilatation of superficial lymphatics as a consequence of transmitted pressure from a congenital malformation of deep dermal lymphatics. Clinically and histopathologically, lymphangiectasia and LC of the vulva are very similar. However, history of congenital or early onset of the disease and the presence of lymphatic abnormalities in the deep reticular dermis and involvement of subcutis on histopathology are suggestive of congenital LC.[10] Diagnosis is mainly clinically aided by histopathology.

Treatment is aimed at reducing underlying lymphoedema, preventing the chances of infection, providing a good cosmetic result, and preventing recurrences. Daily compression bandages yield good results.[3] Excisional surgery and carbon dioxide laser are two major modalities in addition to cryotherapy, sclerotherapy, and electrocoagulation. Excisional surgery corrects the esthetic appearance of the edematous vulva.[4] There can be recurrences, but resection can be repeated several times.[8] Carbon dioxide laser is the main alternative to excisional surgery as it vaporizes the lesions superficially and seals the underlying lymphatics, reducing the chances for recurrences.[11] This can also be repeated if any recurrences occur.[4] It is effective in both LC and also lymphangiectasia, but focal recurrences are more commonly seen in LC, which means that laser cannot treat deeper lymphatic abnormalities.[11] However, the adverse effects of laser therapy are an aggravation of the lesions, keloid formation, and pain over the lesions. Sclerotherapy is a new modality of medical treatment effective in macrocystic lesions.[9] We did CO2 laser therapy on our patient. The treatment was well tolerated with a good cosmetic outcome, and no recurrences were seen during follow-up.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chang M, Shiao G, Tseng C. Lymphangiectasia – Report of one case and review of the literature. Dermatol Sin 1997;15:275-9.  Back to cited text no. 1
    
2.
Singh N, Kumari R, Thappa DM. Vulvallymphangiectasia secondary to tubercular lymphadenitis. Indian J Sex Transm Dis AIDS 2007;28:38.  Back to cited text no. 2
    
3.
Haneef NS, Ramachandra S, Metta AK, Haritha K. Lymphangiectasias of vulva. Indian Dermatol Online J 2011;2:40-2.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Okur MI, Köse R, Yildirim AM, Cobanoğlu B. Lymphangiectasia of the vulva accompanying congenital lymphedema. Dermatol Online J 2009;15:13.  Back to cited text no. 4
    
5.
Horn LC, Kühndel K, Pawlowitsch T, Leo C, Einenkel J. Acquired lymphangioma circumscriptum of the vulva mimicking genital warts. Eur J Obstet Gynecol Reprod Biol 2005;123:118-20.  Back to cited text no. 5
    
6.
Amouri M, Masmoudi A, Boudaya S, Amouri A, Ali IB, Bouassida S, et al. Acquired lymphangiomacircumscriptum of the vulva. Dermatol Online J 2007;13:10.  Back to cited text no. 6
    
7.
Sharma R, Tomar S, Chandra M. Acquired vulval lymphangiectases mimicking genital warts. Indian J Dermatol Venereol Leprol 2002;68:166-7.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
King DT, Duffy DM, Hirose FM, Gurevitch AW. Lymphangiosarcoma arising from lymphangioma circumscriptum. Arch Dermatol 1979;115:969-72.  Back to cited text no. 8
    
9.
Huilgol SC, Neill S, Barlow RJ. CO(2) laser therapy of vulval lymphangiectasia and lymphangioma circumscriptum. Dermatol Surg 2002;28:575-7.  Back to cited text no. 9
    
10.
Vignes S, Arrault M, Trévidic P. Surgical resection of vulva lymphoedemacircumscriptum. J Plast Reconstr Aesthetic Surg 2010;63:1883-5.  Back to cited text no. 10
    
11.
Avci ME. Lymphangiomacircumscriptum of the vulva: A case report and review of literature. Anatol J Obstet Gynecol 2010;24:2.  Back to cited text no. 11
    


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