A 4-year-old female child, born out of nonconsanguineous marriage, presented with multiple reddish-brown asymptomatic lesions on her right lower limb since birth. No history of similar complaints was present in the family. On cutaneous examination, few well-defined hyperpigmented to reddish-brown plaques arranged in a linear distribution were present over the lateral aspect of the right lower limb [Figure 1]. The plaques were firm, nontender, noncompressible, and nonpulsatile with no bruit on auscultation. Differential diagnoses of verrucous hemangioma and angiokeratoma circumscriptum neviformae (ACN) were considered. Dermoscopic examination revealed red and black lacunae which represent dilated vessels with thrombosis and whitish veil which represent acanthosis or hyperkeratosis [Figure 2]. Histopathological examination showed hyperkeratotic epidermis with numerous thin-walled dilated blood vessels in the papillary dermis [Figures 3a, b and 4]. Ultrasonography of the lesion showed no evidence of calcification or any deeper vascular connection. Underlying vessels, muscles, and bone cortices were intact, and mild subcutaneous edema was noted.
Based on clinical, dermoscopic, and histopathological correlation, a final diagnosis of ACN was made. No deep angiomatous component was seen; hence, the possibility of verrucous hemangioma was ruled out.
ACN is an extremely rare cutaneous disorder, it represents a group of vascular ectasias occurring due to congenital malformation of the blood vessels in the papillary dermis, and it may produce hyperkeratosis, acanthosis, and papillomatosis of the epidermis. There are reports of the coexistence of ACN with other vascular malformations; however, in our patient, after doing thorough systemic examination and evaluation, we did not find any such abnormalities. For treatment of this condition, diathermy, curettage, electrocautery, and cryosurgery are sufficient for smaller lesions. Laser ablation or deep surgical excision are deemed suitable for larger lesions.
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