Cutaneous lymphangioma circumscriptum presenting as warty plaque

A 16-year-old female presented to our outpatient department of dermatology with warty raised skin lesions on the left upper arm and axilla for 5 years. No similar history in the family or in the past. On clinical examination, multiple well-defined discrete hyperpigmented verrucous plaques noted over left axilla extending to medial aspect of arm, lesions were non-tender. Otherwise her general physical and systemic examination was normal [Figure 1a]. Polarized dermoscopy of the lesions reveals numerous clods surrounded by white septa having different colors yellow to orange color at the superior part and purple red at the base, referred to as the “hypopyon sign” [Figure 1b]. Histopathology, epidermis showed irregular acanthosis with thin walled, dilated lymphatics in the papillary dermis impinging on epithelium. The findings were consistent with the clinical diagnosis of lymphangioma circumscriptum [Figure 1c]. Cutaneous lymphangioma circumscriptum is uncommon, hamartomatous malformations of the lymphatic system that involve the skin, and subcutaneous tissues are identified by clusters of translucent, occasionally bloody vesicles. Dermoscopic features include reddish to violaceous lacunar structures, pink diffuse colouration and focal reddish areas. Depending on the blood content, this can present in dermoscopy as reddish regions in certain lagoons or brownish lacunas bordered by pale septa. The microshunts between lymphatic channels and small blood arteries have been proposed as a potential explanation for the mechanisms causing the extravasation of erythrocytes into the dilated lymphatics. Blood settling in the lower portion of the lacuna causes the lymphangioma circumscriptum to exhibit the hypopyon sign. This sign is seen in cutaneous angiosarcoma as well. Red lacunae are seen in other vascular lesions such as angiokeratoma and verrucous hemangioma.

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Figure 1:

(a) Multiple well-defined discrete hyperpigmented verrucous plaques noted over left axilla extending to medial aspect of arm, (b) polarized dermoscopy of the lesions reveals numerous clods surrounded by white septa having different colors yellow to orange color at the superior part and purple-red at the base, referred to as the “hypopyon sign”, and (c) epidermis showed irregular acanthosis with thin walled, dilated lymphatics in the papillary dermis impinging on epithelium (H and E stain ×10).

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