Sclerotherapy with Polidocanol by Tessari Technique in a Case of Lymphangioma Circumscriptum over Axilla: A Rare Case Report

INTRODUCTION

Lymphangioma circumscriptum (LC) is a rare clinical entity, with benign proliferation of cutaneous lymphatics, presenting clinically as translucent vesicular lesions.^[1] Most cases reported are over vulva and scrotum. However, any part of the skin including axilla, neck, trunk and thighs can be involved. Treatment is a challenge, considering the recurrences after all modalities of treatment. Although mostly asymptomatic, for fear of complications such as rupture and infections, cases should be treated. Here, we report a case of a large axillary LC, successfully treated by a sclerosant, 3% polidocanol, using the Tessari technique.

CASE REPORT

A 13-year-old male, presented with multiple grape clusters - like grouped vesicles over the right axilla, which started 3 months back and had been gradually increasing in size, with occasional rupture, probably due to friction, causing discomfort and staining clothes. Clinical examination showed a group of translucent, minimally compressive, soft, multilobulated, vesicular lesions with verrucous crusted surface and haemorrhagic spots at the top of nearly half of the vesicles [Figure 1a]. Dermoscopic examination revealed multiple clear to pink and a few yellowish coloured lacunae, vascular structures, white lines, scales and hypopyon sign, overall exhibiting a frog-spawn-like appearance [Figure 1b and c].^[2,3] The history and clinical examination were suggestive of maicrocystic lymphatic malformation (LM) of the skin, also called as LC. Histopathology of skin biopsy was consistent with LC showing a hyperkeratotic epidermis, dilated lymphatic channels and cystic spaces containing clear, proteinaceous fluid with moderate lymphocytic infiltrate, and occasional neutrophils in superficial and deep dermis. Adnexal structures were unremarkable [Figure 2]. A diagnosis of LC was made and sclerotherapy was done using the Tessari technique, with polidocanol 3% solution, using a 3 way stop cock. For the procedure, two 5 mL syringes were taken. One filled with 3% polidocanol solution, another with room air. Both syringes were connected to the 3 way stop cock [Figure 3]. Foam was produced by repeated to-and-fro movement of the syringes. The foam generated was injected intralesionally at 12–15 sites at 0.05–0.1 mL/injection site. The patients father was informed about the probable complications such as pain, redness, swelling, fever or other cutaneous symptoms and asked to report immediately at any point of time in case of any undue event. The patient was reviewed weekly. Reduction in size of the lesions was noted on each visit. Second session was repeated after 1 month. Significant reduction in size of the lesion was noted after the end of the 2^nd month [Figure 4a-c]. No recurrence was detected up to 1 year of follow-up. No complication was reported.

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Figure 1:

(a) (i) Axillary translucent, multilobulated,vesicular lesions with (ii) verrucous crusted surface and (iii) haemorrhagic spots; (b-c) Dermoscopic frog-spawn-like appearance with (iv) multiple clear to pink and a few yellowish coloured lacunae, (v) vascular structures, (vi) white lines, (vii) scales and (viii) hypopyon sign.

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Figure 2:

Histology at 20x magnification showing: (i) hyperkeratotic epidermis, (ii) acanthosis, (iii) dilated lymphatic channels, (iv) cystic spaces containing clear, proteinaceous fluid, (v) inflammatory infiltrates, mostly lymphocytes and occasional neutrophils in superficial and deep dermis.

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Figure 3:

Production of foam for sclerotherapy by Tessari technique: (a) Polidocanol, (b) 3 way stop cock with attached syringes.

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Figure 4:

A 13 year old male with Lymphangioma circumscriptum treated with sclerotherapy with 3% Polidocanol using Tessari technique: (a) Pretreatment, (b) After two weeks of treatment, (c) After 4 weeks of treatment.

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DISCUSSION

LMs are congenital conditions that may involve organs rich in lymphatic drainage system, like intestine, subcutaneous tissues and skin, collectively called as lymphangiomas. Our case can be stated as LC or cutaneous atruncular LM, as we could not get evidence of lymphedema, that is seen in truncular LM, which occurs if the LM lesions are connected to nearby normal lymphatic channels. It can be congenital or acquired due to disruption of normal anatomy by malignancies, trauma, surgery or radiotherapy. Usually, congenital cases occur before 5 years of age. In contrast, our case had onset at 13 years of age. They need to be differentiated from angiokeratomas, molluscum contagiosum and warts.

Various treatment options include medical management such as sildenafil, systemic and topical rapamycin, surgical excision, electrocoagulation, radiofrequency ablation, CO₂ laser ablation, cryotherapy and sclerotherapy. A systematic review by Savas et al. of 16 studies has found CO₂ laser as efficacious and safe treatment modality, especially for large LC lesions.^[4] Aspiration of cystic fluid followed by injection of sclerosing agents such as bleomycin, doxycycline, ethanol and picibanil shows beneficial results.^[5] Recurrences are common with any treatment modality. Eradication of deep lymphatics decreases recurrences. LC may be complicated by lymphorrhoea, ulcerations, infections and bleeding. Therefore, treatment for reduction in size or complete cure needs to be encouraged.

Polidocanol was originally used as a local anaesthetic in the 1950s. It was United States Food and Drug Administration approved as sclerosant for incompetent veins which were used in foam or liquid forms in a concentration of 1% or 3% solution. Of late, it has been used for various dermatologic conditions such as pyogenic granuloma, acne cysts, glomuvenous malformations, mucous cysts and mucoceles.^[6] Foam sclerotherapy with polidocanol shows better efficacy than liquid form, which is highly echogenic and facilitates ultrasonography-guided sclerotherapy. Erythema, oedema, pain, tenderness, increased local temperature and skin pigmentation are anticipated complications. A 70-years-old female with vulvar lymphangiectasia with previous history of squamous cell carcinoma was treated successfully with 3 mL of 1% polidocanol solution given intralesionally at 20 sites with 0.1–0.2 mL solution in a single session by Stull et al.^[7]

Sclerotherapy for axillary LC with 3% polidocanol has not been reported till date.

CONCLUSION

We present a case of axillary LC, successfully managed by sclerotherapy with 3% polidocanol using the Tessari.