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CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 2  |  Page : 125-128

Lymphangiectasia of varied etiology: Case series


Department of Dermatology, Vernerology and Leprology, Gandhi Medical College and Hospital, Hyderabad, Telangana, India

Date of Submission10-Feb-2016
Date of Acceptance07-May-2016
Date of Web Publication30-Jul-2019

Correspondence Address:
Dr. Sudha Rani Chintagunta
Plot No. #5, Jupiter Colony, Kakaguda, Karkhana, Secunderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.263632

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  Abstract 


Lymphangiectasias are dilated superficial lymphatic vessels following damage or obstruction to deep lymphatics leading to increased lymphatic pressure and formation of superficial vesicles. Lymphangiectasia clinically present as numerous translucent vesicles with chronic lymphedema, often associated with various conditions such as malignancies, radiotherapy, trauma, recurrent, and chronic infections and inflammatory conditions. The most common causes of lympangiectasia are surgery and radiotherapy. We herewith report six cases of lymphangiectasias of different etiology, of which five cases involving the genitalia following radiotherapy, malignancy, and blunt injury and another case involving the breast with carcinoma.

Keywords: Breast, genitalia, lymphangiectasia, malignancy, radiotherapy


How to cite this article:
Chintagunta SR, Arakkal G. Lymphangiectasia of varied etiology: Case series. J NTR Univ Health Sci 2019;8:125-8

How to cite this URL:
Chintagunta SR, Arakkal G. Lymphangiectasia of varied etiology: Case series. J NTR Univ Health Sci [serial online] 2019 [cited 2019 Aug 18];8:125-8. Available from: http://www.jdrntruhs.org/text.asp?2019/8/2/125/263632




  Introduction Top


Lymphangiectasia of the skin represents superficial lymphatic vessel dilatation caused by a spectrum of scarring processes that leads to obstruction of local lymphatic drainage. Lymphangiectasia arises due to structural damage to deep lymphatics secondary to radiation, surgery, malignancy, or infections such as filariasis, tuberculosis, lymphogranuloma venereum (LGV), trauma, or pregnancy. Lymphangiectasia clinically presents as thick-walled, translucent vesicles and papules with lymphedema, and histologically appear as dilated spaces with flattened endothelial cells in the papillary dermis. We here by report a retrospective analysis of six cases of Lymphangiectasis of varied etiology.


  Case Report Top


Case 1

A 45-year-old male presented with multiple swellings over the scrotum with serosanguinous discharge since 6 months. General examination showed pallor, pedal edema, and bilateral inguinal lymphadenopathy. Examination revealed multiple grouped and discrete vesicles of 0.5-1.5 cm and erosions present all over the scrotum with surrounding induration over the scrotum and penis. Skin biopsy revealed tumor tissue in lobules and nests with dilated lymphatic spaces secondary to squamous cell carcinoma of the scrotum [Figure 1].
Figure 1: Scrotum and penis showing multiple vesicles and papules and erosions with oozing and scrotal edema along with H&E 40× showing tumor tissue arranged in cords separated by dilated lymphatic spaces

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Case 2

A 35-year-old male presented with multiple, discrete, translucent vesicles on the scrotum since 2 years. There was scrotal and penile edema. History of right inguinal mass 15 years ago that was excised and diagnosed as Hodgkin's lymphoma, followed by radiotherapy is present. Systemic examination and general examination were normal. Biopsy revealed lymphangiectasi. Biopsy revealed lymphangiectasia.

Case 3

A 26-year-old male came with multiple vesicles over the genitalia with discharge of clear fluid since 3 years. There was a history of blunt injury to the genital region at the age of 12 years. Examination revealed numerous confluent and discrete translucent vesicles over the scrotum and the penis. Biopsy revealed dilated lymphatic spaces in the dermis, suggestive of lymphangiectasia.

Case 4

A 65-year-old male presented with vesicles and scrotal swelling since 6 months. Systemic examination and general examination were normal. Histopathology revealed adenocarcinoma with dilated lymphatic spaces. Based on clinical and histopathology findings, a diagnosis of lymphangiectasia was established [Figure 2].
Figure 2: Scrotum showing multiple vesicles along with scrotal and penile edema and additionally H&E 40× showing tumor tissue along with dilated lymphatic spaces

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Case 5

A 50-year-old female presented with asymptomatic skin lesions in the vulvar region since 5 months with a history of radiotherapy of the lower abdomen 5 years back. Examination revealed multiple grouped translucent vesicles of 0.5-2 cm over the labia majora. Skin is indurated over the vulva and the lower abdomen with inguinal lymphadenopathy. Gynecological workup and ultrasound of the pelvis revealed no abnormality. Routine investigations were normal. Histopathology showed numerous lymphatic vessels of irregular size and shape in the upper dermis lined by the endothelium [Figure 3] and [Figure 4]. Based on clinical and histopathological findings, a diagnosis of lymphangiectasia of the vulva was made.
Figure 3: Multiple grouped vesicles over the labia

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Figure 4: H&E 10× Epidermis showing hyperkeratosis along with subepithelial dilated spaces filled with proteinaceous matter in the dermis along with fibrocollagenous tissues

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Case 6

A 32-year-old female presented with painful swellings over the right breast since 2 months. Examination showed multiple grouped vesicles and smooth, skin-colored papules and nodules of 0.5-3 cm with induration. The left breast was normal and there was no axillary lymphadenopathy. Biopsy showed tumor tissue arranged in sheets and cords showing pleomorphic hyperchromatic nucleus around dilated lymphatic spaces suggestive of lymphangiectasia secondary to underlying infiltrating ductal cell carcinoma of the breast [Figure 5] and [Figure 6].
Figure 5: Multiple grouped vesicles, papules, and nodules over the right breast

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Figure 6: H&E 10× showing stratified squamous epithelium with tumor tissue arranged in lobules with dilated lymphatic channels

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  Discussion Top


Lymphangiectasia are dilatation of normal lymphatic channels in the dermis due to damage to the lymphatic vessels secondary to various scarring processes. Lymphangiectasia and lymphangioma circumscriptum share similar clinical and histological features but pathogenesis is different. Lymphangioma circumscriptum is a congenital malformation of the deep lymphatic system and lymphangiectasia is the dilatation of normal superficial lymphatics following any surgery or trauma resulting in fibrosis and/or obstruction to the deep lymphatic vessels.[1] Lymphangioma circumscriptum has an early onset and histopathological examination shows deeper lymphatic abnormalities involving reticular dermis and subcutis. Lymphangiectasia develops later and is secondary to various causes, shows thin-walled ectatic vessels in the superficial and mid dermis. Typical cutaneous lesions in both conditions are grouped translucent vesicles, often compared with frog spawn.

Lymphangiectasia usually occurs between 40-60 years and its onset requires few months to several years after the insult. It can develop anywhere on the body and is common on the upper limbs, chest, axillae, and genital region.[2] There can be an associated underlying lymphedema. Lymphangiectasia is characterized by discrete or clusters of asymptomatic papules or thick-walled vesicles, translucent to flesh colored, and the surface may be smooth or verrucous. It can be asymptomatic or associated with chronic discharge, pain, recurrent infections, and cosmetic disfigurement.

Several cases have been reported in association with malignancies of the breast, cervix, vagina with or without radiotherapy, melanoma, trauma, infections such as filariasis, LGV, and tuberculosis, recurrent cellulitis, erysipelas, keloids, scleroderma, and rarely due to iatrogenic causes such as pencillamine therapy and chronic use of high potency topical steroids.[2],[3],[4]

Lymphangiectasia has an apparent predilection for the genital area, particularly the vulva, the penis, scrotum, and the pubic region after various operative procedures and other conditions where fibrosis and obstruction of lymphatics occur.[4],[5],[6]

In our series, we have observed four cases of lymphangiectasia of the scrotum and penis associated with squamous cell carcinoma [case 1], following radiotherapy [case 2], following blunt injury [case 3] and with adenocarcinoma. All the four cases showed underlying minimal lymphedema.

Literature survey showed few case reports of lymphangiectasia involving the scrotum and the penis following radiotherapy, recurrent infections, trauma, and radical surgery for malignancies.[1],[4],[7]

Our case of vulvar lymphangiectasia presented 5 years after radiotherapy and the patient could not give the details of the underlying disease. Vulval lymphangiectasia is usually reported following surgery or radiotherapy for carcinoma of the cervix.[8] Other underlying causes are urogenital tuberculosis, Crohn's disease, recurrent bacterial infections, or radical pelvic surgery for nongynecological tumors.[6]

Additionally, we report a case of lymphangiectasia involving the breast with underlying ductal cell carcinoma, which is rare. Soo et al. reported a case of lymphangioma and lymphangiectasia of the breast mimicking inflammatory breast cancer.[9] Many cases of lymphangiectasia has been reported in the literature with upper limb lymphedema secondary to mastectomy, radiotherapy, keloids, or scleroderma.[10]

Treatment of lymphangiectasia includes complete surgical excision, cryotherapy, sclerotherapy, electrodessication, and CO2 laser vaporization.[2] The preferred treatment is complete surgical excision but recurrences are common without any malignant transformation. Case 1, 2, 4, 6 were sent to the surgery department for further evaluation and management. Case 3 showed partial improvement with cryotherapy and is under follow-up. Case 5 showed moderate improvement CO2 laser.

Genital lymphangiectasia often present as verrucous papules mimicking condylomata lata, warts, molluscum, and syringomas. Patients with lymphangiectasia need to be evaluated thoroughly for underlying malignancies, radiotherapy, trauma, and infections. Awareness about various etiological factors of lymphangiectases, detailed evaluation, and appropriate investigations are important to avoid misdiagnosis and for better management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mortimer PS. Disorders of lymphatic vessels. In: Burns T, Breathnach S, Cox N, Griffliths C, editors. Rook's Textbook of Dermatology. Vol. 3. 7th ed. Malden, Massachusetts, USA: Blackwell Science Ltd.; 2004. p. 51.2-51.4.  Back to cited text no. 1
    
2.
Celias AV, Gaughf CN, Sangueza OP, Gourdin FW. Acquired lymphangiectasis. South Med J 1999;92:69-72.  Back to cited text no. 2
    
3.
Giannelli V, Rockley PF. Acquired lymphangiectasias following mastectomy and radiation therapy-report of a case and review of the literature. Cutis 1996;58:276-8.  Back to cited text no. 3
    
4.
Arnold HR Jr. Dermal and subcutaneous tumors. In: James WD, Berger TG, Elston DM, Odom RB, editors. Andrews' Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia: Saunders Elsevier; 2006. p. 590-1.  Back to cited text no. 4
    
5.
Verma SB. Lymphangiectasias after penectomy, inguinal lymph node dissection, urethrostomy and radiation. Acta Derm Venereol 2006;86:175-6.  Back to cited text no. 5
    
6.
Vlastos AT, Malpica A, Follen M. Lmphangioma circumsriptum of the vulva: A review of the literature. Obstet Gynecol 2003;101:946-54.  Back to cited text no. 6
    
7.
Di Leonardo M, Jacoby RA. Acquired cutaneous lymphangiectasias secondary to scarring from scrofuloderma. J Am Acad Dermatol 1986;14:688-90.  Back to cited text no. 7
    
8.
Haneef NS, Ramachandra S, Metta AK, Haritha K. Lymphangiectasias of vulva. Indian Dermatol Online J 2011;2:40-2.  Back to cited text no. 8
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9.
Hahn SY, Choi HY, Park SH, Jang J. Lymphangioma and lymphangiectasia of the breast mimicking inflammatory breast cancer. J Ultrasound Med 2011;30:863-5.  Back to cited text no. 9
    
10.
Valdés F, Peteiro C, Toribio J. Acquired lymphangiectases and breast cancer. Actas Dermosifiliogr 2007;98:347-50.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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