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Epidermal hyperplasia, an increased number of vessels in the superficial dermis, and eccrine structures with intermingled vascular channels in the deep dermis (H&E, ×20).
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Eccrine angiomatous hamartomas are benign vascular and eccrine malformations often accompanied by hyperhidrosis or pain, increased eccrine glands, and aggregates of vessels. Verrucous hemangiomas are congenital vascular malformations presenting as unilateral grouped papules. Histologically, they show verrucous epidermal change and proliferation of...
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We report a 42-year-old woman with an asymptomatic solitary targetoid patch on her shin of 1 month duration. Histopathologic evaluation revealed intact epidermis with vascular proliferation in the papillary dermis. Vascular channels were irregular, thin walled, and dilated. They were lined by prominent endothelial cells having plumped protruding nu...
Citations
... In our case adipose tissue element was found with eccrine glands, proliferating blood vessels with nerve fragments. Pele et al proposed criteria for diagnosis of EAH 13 15,16 The Differential Diagnosis is broad because of wide spectrum of clinical variants of this disorder . Differential Diagnosis should include angioleiomyoma, eccrine nevus, vascular lesion , childrens fibrous hamartoma, smooth muscle hamartoma. ...
... EAH. [4][5][6][7][8] We present two cases of vascular hamartoma showing a histopathological combination of EAH with SAK/VVM or VH. We also report a complete and detailed review of the literature to clarify the clinical, epidemiological, and histopathological features of this unique entity. ...
... In addition, EAH often extends into the superficial dermis, making it impossible to identify correctly the boundary between the deep dermis and subcutaneous fat. For example, in our first case, there were some areas that could be interpreted as a deep extension of the superficial vascular component (therefore supporting a diagnosis of VVM); however, these areas 5 In our circumstance, it could be due to the pubertal hormones, as already described for other related skin lesions, including EAH. 6,13 In the other case, the rapid growth was related to a trauma. 5 Neither of the patients had a significant family history or associated systemic and chronic disease. ...
... For example, in our first case, there were some areas that could be interpreted as a deep extension of the superficial vascular component (therefore supporting a diagnosis of VVM); however, these areas 5 In our circumstance, it could be due to the pubertal hormones, as already described for other related skin lesions, including EAH. 6,13 In the other case, the rapid growth was related to a trauma. 5 Neither of the patients had a significant family history or associated systemic and chronic disease. 1-8 EAH usually appears at birth or in childhood as a papule, plaque, or nodule on the extremities, especially the legs. ...
First reported in 2006, eccrine angiokeratomatous hamartoma is a very rare vascular malformation of the skin, with only few described cases. It has a peculiar histology with features deriving from the combination of two different vascular malformations of the skin: solitary angiokeratoma and eccrine angiomatous hamartoma. In the past, other authors described similar hamartomatous lesions with features deriving from verrucous venous malformation and eccrine angiomatous hamartoma. We believe that these lesions are clearly overlapping from clinical, histological and immunohistochemical points of view and the term “eccrine angiokeratomatous hamartoma” should be used to indicate the whole spectrum of these lesions as suggested by Kanitakis et al. Herein we present two cases of this rare vascular hamartoma, with clinical, histological and immunohistochemical characterization. In addition, for the first time we report a complete and detailed review of the literature to clarify the clinical, epidemiological and histological features of this unique entity. This article is protected by copyright. All rights reserved.
... Interestingly, coexistence of eccrine angiomatous hamartoma and verrucous hemangioma has been found [12]. Eccrine angiomatous hamartoma has been reported in combination with other vascular tumors in only a few instances, such as verrucous hemangioma or overlying verrucous-hemangioma-like features [13]. ...
Verrucous hemangioma is a rare vascular malformation consisting of a proliferation of dilated blood vessels of different sizes that occupy the dermis and hypodermis. The epidermis of the affected area shows a strong proliferative reaction that presents as a warty appearance. Here, we report the case of a 17-year-old male who presented with an asymptomatic warty growth above the lateral malleolus on the right leg, which healed after electrocautery.
... Smith et al. [6] also designated three histological variants of EAH; follicular, lipomatous and mucinous. Some anecdotal cases of EAH with verrucous epidermal hyperplasia as well as features overlapping with hemangioma have been reported [7]. ...
Eccrine angiomatous hamartoma is a rare hamartoma that usually affects childhood and adolescence. In this report we describe a typical onset and clinical presentation of eccrine angiomatous hamartoma. Histopathological analysis and immunohistochemical staining of the sections were done to confirm the diagnosis.
... To our knowledge, only few cases have been reported showing the coexistence of EAH and verrucous hemangioma-like features. (5,6) ...
Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous tumor. It is characterized by the proliferation of the eccrine gland elements that are closely associated with capillary proliferation. Patients usually present with a solitary nodule on the extremities that appeared at birth or during the prepubertal years. We report a rare case of EAH in a 13-year-old boy, with clinical features suggesting melanoma. Histologically, an EAH with changes resembling a verrucous hemangioma was noted.
... [2,3] EAH have a preference for distal extremities and some cases have shown an association with other vascular tumors. [4] The pain can be a result of hormonal influence, but in general is attributed to involvement of local nerve fibers while hyperhidrosis appears to be a result of stimulation of the sweat glands. [2,3] The differential diagnosis includes various vascular malformations, hamartoma of the smooth Eccrine angiomatous hamartoma: A rare entity LETTER TO EDITOR ...
... Although there are no specific immunohistochemical markers for VH, 5 it shares some characteristics with infantile hemangioma. Glucose transporter-1 (Glut-1), considered the definitive marker of infantile hemangioma 6 and not seen in other vascular malformations, 3,6 has been shown on the endothelium of VH. 3,5,7 There is evidence of the expression of Wilms tumor protein (WT-1) in VH, a marker normally associated with vascular tumors and not vascular malformations. 8 We have recently shown the expression of primitive and stem cell markers 9 -13 and provided evidence of a placental chorionic villous mesenchymal core cell origin of infantile hemangioma. ...
... Furthermore, both cases showed no immunoreactivity for the lymphatic endothelial marker, D2-40, which is against the diagnosis of angiokeratoma. 3,5 The focal expression of Glut-1 of these two cases is also characteristic of VH 3,5,7 and we show that Glut-1 being more commonly expressed in the more superficial regions of the lesion, and absent in the deeper dermis and subcutis. VH represents an intriguing vascular anomaly that has been difficult to categorize. ...
Verrucous hemangioma (VH) presents clinically as a vascular malformation but has similar histopathologic features to infantile hemangioma. This study characterized the cell population within VH.
Paraffin-embedded sections from two male patients with VH were processed for immunohistochemistry. The expression of SMA, CD34, glucose transporter-1 (Glut-1), D2-40, brachyury, angiotensin converting enzyme (ACE), Oct-4, hemoglobin ζ chain (HBZ), Wilms tumor protein (WT-1) and CD45 was examined.
The lymphatic marker, D2-40, was not expressed in VH, whereas Glut-1 was widely expressed in infantile hemangioma, it was only focally expressed by the endothelium of VH. The endothelium of VH expressed the primitive markers, Oct-4, brachyury and ACE. The primitive marker, WT-1, was expressed predominantly on the pericyte layer of both VH and infantile hemangioma. However, HBZ was only expressed in infantile hemangioma. CD45, a mature hematopoetic marker, was expressed by cells within the interstitium, away from the endothelium of VH and infantile hemangioma.
The expression of the primitive markers, Oct-4, brachyury and ACE on the endothelium, and WT-1 predominantly on the pericyte layer of VH shows a primitive microvascular phenotype similar to infantile hemangioma. However, the absence of the embryonic marker, HBZ, expressed only in first trimester placenta and in proliferating infantile hemangioma, suggests a different cellular origin. HBZ could be used to distinguish between the two conditions.
... EAH has been reported in combination with other vascular tumors in only a few instances, such as arteriovenous malformation, spindle cell hemangioma, verrucous hemangioma, or overlying verrucous hemangiomalike features. (5,14,29,(30)(31)(32)(33) It is interesting that in our cases we found additional pathological findings including hemangioma (13.3%), verrucous hemangioma (6.7%), arteriovenous malformation (6.7%), and angiokeratoma (6.7%). To the best of our knowledge, no previous studies have reported the coexistence of EAH and other vascular tumors. ...
Eccrine angiomatous hamartoma (EAH) comprises a rare nevoid proliferation of normal eccrine glands and small blood vessels and occasionally other elements in the middle and deep dermis with variable clinical manifestations. Case series have rarely been published except for case reports and literature reviews. The aims of this article were to investigate the clinical and pathologic features of patients with EAH in Taiwan and to compare our results with the results of previous studies.
A retrospective review of medical records and histopathological findings was performed on patients diagnosed with EAH in a medical center in Taiwan between 1994 and 2010.
Fifteen patients with pathologically diagnosed EAH were collected. The mean age at the time of diagnosis was 38.6 years (range, birth to 67 years). The male to female ratio was 3 to 2. In most cases, EAH arose as a single lesion on a lower extremity. The symptoms and signs most commonly associated with EAH were pain (60%), hypertrichosis (13.3%), itching (13.3%) and hyperhidrosis (6.7%). Additional pathological findings included hemangioma (13.3%), verrucous hemangioma (6.7%), arteriovenous malformation (6.7%), and angiokeratoma (6.7%). None of the patients experienced spontaneous regression of the lesions before excision. Excisions were done in one patient under general anesthesia, and ten patients with local anesthesia. Four patients were kept under observation. Tumor recurrences were noted in two out of the eleven patients whose lesions were excised.
Compared with cases in the literature, we found additional histopathological findings and an increased tumor recurrence risk in our cohort. EAH remains a benign and uncommon hamartomatous condition. Further multi-center, retrospective studies with larger case numbers are needed to better characterize the disease presentation in Asian populations.
A 30-year-old male presented with purple-red plaque exhibiting focal hyperhidrosis and mild tenderness involving right hand (Figs. 64.1 and 64.2). The symptoms started 1 year prior to presentation.
