Eccrine Angiomatous Hamartoma: A Clinicopathological Study of 26 Cases

Abstract

Background: Eccrine angiomatous hamartoma (EAH) is a rare benign cutaneous tumor characterized by the proliferation of eccrine glands and capillaries. Objective: The aim of this study was to summarize the clinicopathological characteristics of EAH. Methods: A retrospective chart review was performed on all patients diagnosed with EAH from 1977 to 2012 in the Union Hospital, Wuhan, P.R. China, and the clinicopathological features were compared with the cases reported in the literature. Results: A total of 26 patients with EAH were identified. The male:female ratio was 1.2:1. EAH most commonly presents as a solitary (80.8%) plaque (50.0%) on the lower extremities (61.5%). Most patients presented with hyperhidrosis localizing to the lesion. Although most patients did not have major pain or anatomic deformity, one patient had severe pain as well as difficulty walking and moving, necessitating leg amputation. The histopathological findings showed typical features of EAH. Conclusion: EAH is a rare but characteristically benign skin hamartomatous condition. In rare occasions it can be associated with severe structural and functional impairment.

A hairy port‐wine stain with overlying hyperhidrosis on the thigh of a child

“…Pain and hyperhidrosis is reported in roughly one‐half and one‐third of the cases, respectively. Pain could be attributed to the presence of infiltrating small nerves in the lesion, while hyperhidrosis is probably due to the increased eccrine sweat glands . Although its exact etiopathogenesis is not known, it is hypothesized that a defective biochemical interaction between the epidermis and mesenchyme during development leads to an abnormal proliferation of eccrine and vascular structures.…”

Large eccrine angiomatous hamartoma: a novel clinical presentation of disease

“…7 The most common presentation is a painful, hyperhidrotic and solitary element on the distal extremities with a median size of 4-15 mm. 6,7 Several things differ in our case; localisation on the upper back, no pain or tenderness nor hypertrichosis and a size far surpassing that of preceding reports.…”

“…7 The most common presentation is a painful, hyperhidrotic and solitary element on the distal extremities with a median size of 4-15 mm. 6,7 Several things differ in our case; localisation on the upper back, no pain or tenderness nor hypertrichosis and a size far surpassing that of preceding reports. Hyperhidrosis of EAH is presumably an expression of its eccrine components and occurs in one third of all patients with EAH, as it was the case in our patient, it is seen in response to the typical triggers of eccrine secretion such as heat or emotional stimuli.…”

“…For most cases, surgical removal is the first choice and the definitive treatment. 2,3,[5][6][7] Non-invasive treatments comprise of topical antiperspirants or intralesional injections of botulinum toxin type A. 8 As surgery was rejected due to size and topical ointments due to location, botulinum toxin type A was preferred.…”

“…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. 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.…”