Abstract

The clinical triad of vascular extremity lesions, hyperhidrosis, and episodic pain with histologic findings of a vascular malformation and eccrine gland changes correspond with the differential diagnoses of blue rubber bleb nevus syndrome (BRBNS), sudoriparous angioma (SA), and eccrine angiomatous hamartoma (EAH). Given that these three entities have eccrine gland associations with vascular proliferations, clinical and histological correlation is essential in these cases. Herein, we describe the case of a 15-year-old female with seven-year history of tender palpable lesions on the bilateral lower extremities with episodic hyperhidrosis and pain notably in stressful situations. Upon evaluation, multiple scattered dusky purple indurated papules were noted in bilateral lower legs. Punch biopsy of the medial calf revealed a complexed ectatic vascular growth lined by a single-layer endothelial cells and prominent dilated adjacent eccrine glands, overall consistent with venous malformation. At the time of this report, our patient was pending endoscopic evaluation for visceral involvement and thus blue-rubber bleb nevus syndrome. BRBN histologically manifests as venous malformations, and reportedly could comprise eccrine lesions. EAH is traditionally characterized by lobules of mature eccrine glands, intimately associated with capillary caliber vessels, in a hamartomatous fashion. Subsequent cases report a range of associated vascular lesions, including spindle cell hemangioma, arteriovenous malformation and verrucous hemangioma. Considered nowadays as a variant of EAH, SA is another painful, eccrine-vascular lesion, which reportedly perspirate when pinched or stroked. All these lesions reportedly could present as either solitary or multiple lesions. We review the literature and highlight these important clinicopathologic clues in parsing the diagnosis of these eccrine-vascular entities.

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