Abstract
An otherwise healthy 13-year-old female presented with agminated flesh-colored to slightly brown papules on the inferior vermillion lip, with the largest measuring 5 mm. She reported no family history of similar lesions. These were present since birth, asymptomatic, and did not change with environmental exposures. Shave biopsy of the largest lesion showed a well circumscribed, moderately cellular neoplasm located in the upper and mid-reticular dermis, composed of nests and strands of epithelioid cells with pale eosinophilic granular cytoplasm, round to ovoid nuclei, and fine nuclear chromatin. No cytologic atypia, pleomorphism or mitoses were noted. Stroma showed increased vascularity with ectatic vessels and areas of hyalinization with focal collagen trapping. The differential diagnoses included granular variant of angiofibroma, granular cell tumor, PEComa and cellular neurothekeoma. The granular cells were positive for CD163, NKIC3, MITF, and negative for SOX-10, S-100, HMB45, SMA and NSE. The findings were consistent with granular angiofibroma. Agminated angiofibromas may represent a segmental form of tuberous sclerosis (TS). Multiple angiofibromas are also associated with multiple endocrine neoplasia type 1, Birt-Hogg-Dube syndrome, Neurofibromatosis-2 and Cowden disease. However, no other cutaneous lesions or stigmata of these diseases were noted in our patient. Granular cell change in angiofibroma is an uncommon histology, and its clinical presentation as agminated labial papules unassociated with genodermatoses has not been reported. For our patient, shave removal resulted in excellent cosmesis, and she continues to be followed for findings suggestive of syndromic involvement.
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