Acquired lymphangiectasia with hemangiomatous transformation in a child: A report

Dear Sir,

Acquired lymphangiectasias are common in the adult population, following mastectomy and radiation therapy, clinically manifesting with translucent vesicles in a chronic lymphedematous area after surgical intervention with or without radiation therapy. We hereby report a case of acquired lymphangiectasia with hemangiomatous transformation in a child, following excision of a congenital hamartoma.

A 14-year-old, otherwise healthy boy, presented with multiple, painless, coalescing, verrucous papules, plaques, and nodules on his left shoulder, which had been present for the past 12 years. The lesions had variable dimensions, had a pink to purple color, with a translucent appearance in some, which had gradually increased in size and number over the years [Figure 1]. The lesions occasionally bled on touch. The patient had a history of excision of a hamartoma on his left shoulder at the age of 2½ years, followed by sclerotherapy. Following excision and sclerotherapy, the patient started to develop these lesions. Dermoscopy revealed red-colored lacunae with a few yellowish-white lacunae [Figure 1]. Punchy biopsy followed by histology showed dilated vascular structures in the epithelium and papillary dermis, containing erythrocytes and proteinaceous fluid in some. The surrounding epithelium had a mild increase in melanocyte ratio and focal acanthosis. The dermis had perivascular inflammation and fibrocollagenous thickening [Figure 2]. Adnexal structures were unremarkable. The findings were consistent with lymphangioma circumscriptum (LC) with hemangiomatous transformation. The patient has been offered laser ablation of lesions as subsequent management, and he is under periodic follow-up.

Close

Figure 1: Multiple coalescing papules and nodules on the left shoulder. These lesions, measuring approximately 6 × 4 cm, exhibited a pink to purple color with a translucent appearance in some areas (inset showing dermoscopic image, red-colored lacunae with a few yellowish-white lacunae).

Export to PPT

Close

Figure 2: Photomicrograph showing dilated vascular structures in the epithelium and papillary dermis, with erythrocytes (yellow stars) and proteinaceous fluid. Mild epithelial melanocyte increase, focal acanthosis, perivascular inflammation, and dermal fibrocollagenous thickening were noted. Adnexal structures were normal. (Heamotoxylin & eosin, 40X).

Export to PPT

LC, or microcystic lymphatic malformation, is a benign hamartomatous malformation affecting the skin’s lymphatic channels. It comprises a visible dermal vesicular component and a less apparent subcutaneous cisternal element. The primary treatment is radical surgery. Typically asymptomatic when localized, LC can manifest through spontaneous or trauma-induced oozing of a clear fluid mixed with blood. Common complications include cellulitis and lymphatic fluid leakage, with rare incidences of squamous cell carcinoma, verruciform xanthoma, and lymphangiosarcoma developing within the malformation. Although acquired lymphangiomas generally lack malignant potential, chronic lymphedema associated with LC increases the risk of developing lymphangiosarcoma, a highly aggressive tumor with poor prognosis.^[1,2] Podoplanin (D2-40) is a commonly employed immunohistochemistry stain specific for an O-linked sialoglycoprotein distributed over the lymphatic endothelium.^[3] Management options include surgical excision, laser therapy, sclerotherapy, and cryosurgery, each with its own benefits and drawbacks. Surgical excision is the most definitive option but carries the highest risk of complications such as scarring and nerve injury. Laser therapy, utilizing carbon dioxide or pulsed dye lasers, can provide relief but may not always prevent recurrence. Sclerotherapy offers a minimally invasive alternative, although recurrence rates vary. Cryosurgery shows promise, particularly when combined with imiquimod cream. Despite the array of treatments, recurrence remains a challenge.^[4,5] Acquired cutaneous lymphangiectasias in the pediatric age group are uncommon, and this prompted us to report the case.