Eccrine angiomatous hamartoma (nevus):

Abstract

Eccrine angiomatous hamartoma (nevus) is a rare form of congenital tumorous malformation with proliferation of eccrine secretory coils and ducts, surrounding capillary angiomatous channels and occasionally other minor elements. To date, there have been only about 24 cases reported in the literature. We report an additional case with more detailed description of the microscopic findings, including immunohistochemical observations. The patient was a 28-year-old female who presented with painless, flesh- to reddish brown-colored, violaceous or bluish subcutaneous nodules on the extremities and trunk. The tumors did not show sweating following exertion. The histologic features were comparable to the previously reported cases. The hamartomatous eccrine sweat glands and ducts and a few apocrine glands demonstrated qualitatively diminished antigens commonly found in the eccrine sweat apparatuses, such as carcinoembryonic antigen (CEA) and S-100 protein. The findings of CD34, CD44, human nerve growth factor receptor and Ulex europaeus antigens have not been previously reported. The histologic features suggested a "hamartomatous" growth rather than a true neoplastic process.

Large eccrine angiomatous hamartoma: a novel clinical presentation of disease

“…Immunohistochemical analyses have demonstrated no definite difference between normal eccrine glands and those in EAH. 4,6,12 We found a similar pattern with the addition CD31 lined vessels, CEA and KL-1 positive ducts and SMA and KL-1 positive glands. Importantly, the immunohistochemical profiles of EAH and nonlesional skin were comparable on these parameters as well.…”

“…4 The histologic criteria for EAH include hyperplasia of normal or dilated eccrine glands; close association of the eccrine structures with capillary angiomatous foci; and the variable presence of pilar, lipomatous, mucinous, and/or lymphatic structures. 1,2,4,10 Our histopathological conclusion matched all criteria and included mucin deposition. Mild fibroblastic hyperplasia can present in the dermis, a sign we also might have encountered.…”

“…2,3,[5][6][7][8][9] While clinical examination can give meaningful clues, the diagnosis is based on the histologic criteria for EAH, which is defined as (1) proliferation of mature, normal or dilated eccrine glands, (2) intimate association of the eccrine structures with benign vascular proliferation, and (3) varying occurrence of pilar, lipomatous, mucinous, and/or lymphatic structures. 1,2,4,10 We present a case of EAH disclosing distinct histopathological features of the previously reported, though rare, mucinous EAH variant but of extraordinary size several hundred times larger than commonly seen.…”

“…[1][2][3] EAH is characterized by the proliferation of eccrine glands and numerous vascular channels, and a variable presence of pilar structures, fatty tissue, mucin deposition, and/or lymphatic configurations. 4 It is important to recognize the hamartoma as a benign clinical entity, for which aggressive management is not necessary, yet treatment including surgery or topical therapies is occasionally needed to alleviate pain or hyperhidrosis. 2,3,[5][6][7][8][9] While clinical examination can give meaningful clues, the diagnosis is based on the histologic criteria for EAH, which is defined as (1) proliferation of mature, normal or dilated eccrine glands, (2) intimate association of the eccrine structures with benign vascular proliferation, and (3) varying occurrence of pilar, lipomatous, mucinous, and/or lymphatic structures.…”

Eccrine angiomatous hamartoma with sudden enlargement and pain in an adolescent girl after menarche

“…[1][2][3][4] It usually arises at birth or during early infancy and childhood as a nodule or a plaque predominantly involving the distal extremities. Although some EAH cases are asymptomatic, pain and hyperhidrosis are common.…”