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CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 1  |  Page : 72-74

Verrucous lymphangioma circumscriptum in a child


Department of Dermatology and Venereology, Pramukshwami Medical College, Karamsad, Gujarat, India

Date of Submission10-Nov-2016
Date of Acceptance05-Mar-2019
Date of Web Publication26-Apr-2019

Correspondence Address:
Dr. Pragya A Nair
Department of Dermatology and Venereology, Pramukshwami Medical College, Karamsad - 388 325, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_113_16

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  Abstract 


Lymphangioma circumscriptum (LC) is characterized by dilation of lymphatic vessels in the skin and subcutaneous tissue. It presents as asymptomatic vesicles filled with lymphatic fluid giving characteristic frog spawn appearance, but does not progress into warty plaque. LC can also be divided into congenital and acquired form. The common sites are axillary folds, shoulder, upper arm, scrotum, penis, rectum, and vulva. It needs to be differentiated from certain common conditions such as herpes zoster, molluscum contagiosum, hemangioma, verruca vulgaris, and angiokeratoma. Surgical modality is the first choice of treatment. A case of a 5-year-old girl having verrucous plaque on the right knee joint diagnosed as LC is presented here.

Keywords: Lymphangioma circumscriptum, lymphatics, verrucous


How to cite this article:
Nair PA, Kota RK, Singhal RR, Gandhi SS. Verrucous lymphangioma circumscriptum in a child. J NTR Univ Health Sci 2019;8:72-4

How to cite this URL:
Nair PA, Kota RK, Singhal RR, Gandhi SS. Verrucous lymphangioma circumscriptum in a child. J NTR Univ Health Sci [serial online] 2019 [cited 2019 Jul 16];8:72-4. Available from: http://www.jdrntruhs.org/text.asp?2019/8/1/72/257156




  Introduction Top


Lymphangioma circumscriptum (LC) is a benign lymphatic malformation characterized by dilatation of lymphatic vessels in the skin and subcutaneous tissue.[1] It was first described by Fox and Fox in 1879 as lymphangiectodes and later on renamed as lymphangioma circumscriptum by Morris in 1889.[2]

It can be congenital or acquired. Approximately 90% of cases are diagnosed before 2 years of age. Congenital variety occurs due to malformation of lymphatics, whereas acquired variety has various causes. Clinically, it presents with asymptomatic vesicles filled with lymphatic fluid and has a characteristic frog spawn appearance. LC may resemble a number of disorders including lymphangiosarcoma, syringocystadenoma papilliferum, lupus vulgaris, actinomycosis, herpes zoster, viral warts, and molluscum contagiosum.[3] Diagnosis is mainly by clinical and histopathological examination. Definitive treatment is surgical removal, but various other modalities are also available such as scelotherapy, cryotherapy, laser, and electrocautery. We report a case of a 5-year-old girl with a verrucous plaque on the right knee joint diagnosed as LC on histopathology.


  Case History Top


A 5-year-old girl presented with elevated lesions over right knee joint since almost 6 months. History of gradual increase in lesions which extended to the leg and the adjacent thigh was present. The patient complained of bleeding and oozing from the lesion after any trauma. There was no history of pain or itching from the lesions. No history of fever or any respiratory complaint was present. On examination, multiple grouped papules forming a plaque having verrucous morphology almost 8 × 6 cm in size were present over the right knee joint extending to the adjacent lower thigh and leg [Figure 1]. Few discrete papules were seen at the margin with two hypopigmented ill-defined plaques over the right thigh. A biopsy was taken from one of the papule keeping tuberculous verrucosa cutis, common warts, and LC as differentials. Histopathology showed hyperkeratosis and moderate acanthosis with presence of numerous dilated lymphatics in superficial and papillary dermis containing many red blood cells and lymphocytes. Marked periappendageal and perivascular lymphoplasmacytic infiltration was seen [Figure 2]a and [Figure 2]b. Diagnosis of LC was made. The patient was advised cryotherapy for the same, but the patient wanted quick response and so was referred to a laser clinic as we did not have the facility for the same.
Figure 1: Multiple grouped papules, few verrucous forming a plaque over right knee joint

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Figure 2: (a) Hyperkeratosis, moderate acanthosis with presence of numerous dilated lymphatics in superficial and papillary dermis containing many red blood cells (H and E stain 4×). (b) 10×

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  Discussion Top


Lymphatic malformations or lymphangiomas are rare benign hamartomas composed of dilated lymphatic channels filled with a proteinaceous fluid. They generally do not have connections to the normal lymphatic system and result from maldevelopment of primitive lymphatic sacs.[4] They are commonly seen over chest, mouth, axilla, and tongue.[1] Lymphangioma can occur at any age, but majority are seen in children. Approximately 50% present at birth and 90% are diagnosed before the age of 2 years.[1] Lymphangiomas can be divided into LC, which are superficial cutaneous lesions, and cavernous lymphangioma, which are more deep-seated.

LC, also known as “capillary lymphangioma,” “lymphangiectasia,” and “dermal lymphangioma,” is a rare benign skin disorder involving hamartomatous lymphatic malformation of deep dermal and subcutaneous lymphatic channels.

Peachey et al.[5] classified LC into two main forms: classic and localized. The classic form usually appears at or soon after birth and involves proximal limbs. It is thought to be derived from muscular lymphatic cisterns which failed to segment during embryonic development. Clinically, lesions appear as vesicular and do not progress into warty plaque. On the contrary, the localized form is seen at any age and has no site predilection.

LC can also be divided into congenital and acquired forms. Congenital LC results from local malformation of lymphatics and manifests at birth or before 5 years of age, whereas acquired form occurs secondarily due to obstruction of lymphatics commonly in the vulvar region and can manifest at any age secondary to pelvic surgery, radiation therapy, and infection such as tuberculosis, Crohn's disease, and so on.[6] The common sites are axillary folds, shoulder, upper arm, scrotum, penis, rectum, and vulva.

LC develops as a result of collection of subcutaneous lymph cisterns during embryonic development, which are not connected to the lymphatic system and therefore unable to drain the lymph received from the surrounding tissue. The cisterns are lined with muscle that contracts and, by applying pressure, produces protrusions on the skin. Acquired LC develops due to injury to deep collecting lymphatics, caused by radiotherapy damage or infections such as filariasis, lymphogranuloma venereum, or tuberculosis in advanced age. The exact etiology of LC is unknown, but various growth factors such as vascular endothelial growth factor-C (VEGF-C) and VEGF-D and their receptors on the lymphatic endothelial cells may have a role.[7]

LC is usually asymptomatic. Vesicles that contain lymphatic fluid are compared with frog spawn which are pink to copper-colored while secondary hemorrhage gives it red or black color. Less commonly, it can present as diffuse swelling or with verrucous morphology as was seen in our case.

If there is significant hyperkeratosis, the swelling may clinically resemble condyloma acuminate. It can be complicated by excessive drainage and recurrent cellulitis.

Differential diagnosis of LC includes genital warts, herpes zoster, molluscum contagiosum leiomyoma, hemangioma, verruca vulgaris, angiokeratoma, and lymphangioendothelioma.[8] As the clinical presentation of LC may vary from pseudovesicles to nodules or wart-like lesions, it requires histopathology.

Histology shows dilated lymphatics which contain red and white blood cells in the epidermis and the papillary dermis, lined by flat endothelial cells. Sometimes, the epidermis shows acanthosis and hyperkeratosis with widening of papillary dermis. The deeper dermis shows wide ectatic channels with a lining of endothelium containing lymph.[9]

LC is treated for cosmetic reasons and to prevent complications such as cellulitis. The definitive treatment for lymphangiomas is surgical excision.[10] It has highest cure rate with recurrence rate of 17%–23% which is usually due to an improper surgical approach or inadequate excision of the tumor. Surgery has the highest risk of complications such as scarring, keloid formation, hematoma, wound infection, and nerve injury. Other treatment options include scelotherapy, cryotherapy, pulsed dye laser, electrocoagulation, CO2 laser, and topical imiquimod cream. Recurrence is a rule with other forms of destructive therapies.[10]

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.
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. 1
    
2.
Morris M. Lymphangioma circumscriptum. In: Unna PG, Morris M, Duhring LA, Leloir H, editors. International Atlas of Rare Skin Diseases. London: Lewis; 1889. p. 1-4.  Back to cited text no. 2
    
3.
Kura MK, Rane VK. Cervicofacial actinomycosis mimicking lymphangioma circumscriptum – A case report. Indian J Dermatol 2011;56:321-3.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Cahill AM, Nijs ELF. Pediatric vascular malformations: Pathophysiology, diagnosis, and the role of interventional radiology. Cardiovasc Intervent Radiol 2011;34:691-704.  Back to cited text no. 4
    
5.
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. 5
    
6.
Mu XC, Tran TA, Dupree M, Carlson JA. Acquired vulvar lymphangioma mimicking genital warts. A case report and review of literature. J Cutan Pathol 1999;26:150-4.  Back to cited text no. 6
    
7.
Roy KK, Agarwal R, Agarwal S, Kumar S, Malhotra N, Gopendru N. Recurrent vulvar congenital lymphangioma circumscriptum – A case report and literature review. Int J Gynecol Cancer 2006;16:930-4.  Back to cited text no. 7
    
8.
Mehta V, Nayak S, Balachandran C, Monga P, Rao R. Extensive congenital vulvar lymphangioma mimicking genital warts. Indian J Dermatol 2010;55:121-2.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol 1976;94:473-86.  Back to cited text no. 9
    
10.
Puri N. Treatment options of lymphangioma circumscriptum. Indian Dermatol Online J 2015;6:293-4.  Back to cited text no. 10
[PUBMED]  [Full text]  


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