Abstract
Eccrine angiomatous hamartoma (EAH) is a rare malformation presenting with the proliferation of eccrine glands and other dermal structures. It typically presents as a solitary lesion on extremities that appears at birth or during childhood. It is generally asymptomatic but may be associated with hyperhidrosis or pain. Here we report a case of verrucous EAH located on the heel of a 27-year-old man, which is an uncommon presentation of this tumor, and review the clinical characteristics, histologic findings, and prognosis of this rare condition.
Introduction
Eccrine angiomatous hamartoma is a rare hamartoma that comprises the proliferation of the eccrine glands within a vascular stroma and the proliferation of other dermal structures, such as hair and adipose tissue [1, 2] along with epidermal hyperplasia [3]. This tumor mostly occurs at birth or childhood as a solitary plaque or nodule on the extremities [2]. Its manifestations range from simple angiomatous nodules to erythematous plaques [1]. Generally, the lesion is asymptomatic, but pain, hyperhidrosis, and hypertrichosis have also been reported [4, 5]. In this article, we report a case of verrucous EAH, which is located in the heel of a 27-year-old man.
Case Report
A 27-year-old man without any past medical history presented with a lesion on his right heel during the past 2 years. The lesion has grown gradually with no pain. The patient only complained about the symptom of sweating on the lesion in hot places. He was otherwise developmentally normal and had no musculoskeletal abnormalities. He had no drug history and there was not any history of similar lesions in close family members. Upon physical examination, a solitary bruise-like verrucous 4 × 6 cm palpable plaque was seen on the right heel (Fig. 1). The lesion was well circumscribed without any tenderness.
Solitary bruise-like verrucous 4×6 cm palpable plaque in the right heel.
Histopathologic examination of the skin biopsy showed a lobular compartment with increased eccrine glands and vascular channels juxtaposed to the nerve bundles in the deep dermis (Fig. 2a, b). The vascular component was composed of tiny capillaries with variable dilation associated with mature-appearing eccrine glands in a lobular arrangement. The overlying epidermis revealed hyperkeratosis, moderate acanthosis, and low papillomatosis with irregular rete ridges (Fig. 2c). The patient declined any surgical treatment.
Histopathology of lobular compartment with increased eccrine glands and vascular channels juxtaposed to the nerve bundles in deep dermis. a Hyperkeratosis, moderate acanthosis, and low papillomatosis with irregular rete ridges. b, c Vascular component of small capillaries with variable dilation associated with mature-appearing eccrine glands in a lobular arrangement.
Histopathology of lobular compartment with increased eccrine glands and vascular channels juxtaposed to the nerve bundles in deep dermis. a Hyperkeratosis, moderate acanthosis, and low papillomatosis with irregular rete ridges. b, c Vascular component of small capillaries with variable dilation associated with mature-appearing eccrine glands in a lobular arrangement.
Conclusion
Primarily, Lotzbeck reported an angiomatous lesion of EAH in 1859 located on the cheek of a child [1]. This tumor presents equally in males and females. Approximately a third of known cases occur from birth to childhood. However, a few studies have reported the lesions that appear in adulthood [1, 2]. It typically manifests as a single, flesh-colored, blue-brown, or reddish papule, plaque, or nodule, although uncommonly multiple lesions or hyperkeratotic verrucous variants have also been reported [4]. The tumor is usually located on acral sites, especially on palms and soles of the feet [5, 6]. Other areas include the face, neck, abdomen, scrotal region, and back [4].
Hyperhidrosis and pain are common symptoms that can be spontaneous or caused by applying gentle pressure. The pain may be due to nerve fiber involvement. Besides, local temperature elevation of the lesion may stimulate eccrine glands, which can cause hyperhidrosis [1].
The lesions’ size is often enlarged along with the body size growth. However, sudden enlargement can also occur during pregnancy and puberty that may be due to the change in hormonal status of the body [4, 7].
A biopsy is necessary for the definite diagnosis of EAH. Histopathologic criteria are defined as eccrine glands and vascular elements proliferation along with variable apocrine glands and pilar, mucinous, lipomatous, and lymphatic structures [6]. The tumor is often well defined in the middle or deep dermis with an unremarkable overlying epidermis [4, 6]. Cases of EAH with overlying verrucous epiderm have been rarely reported [3, 6, 8]. Here we report another rare case in this article with an interesting presentation. The epiderm of verrucous EAH shows hyperkeratosis, acanthosis, and papillomatosis [3].
Immunohistochemical stains with pancytokeratin and CD31 may show eccrine glands and capillaries, respectively [2]. The principal differential diagnosis are other vascular malformations, smooth muscle hamartoma, glomus tumor, and tufted angioma [1, 6].
Treatment options comprised simple excision for pain, cosmetic reasons, and small lesions [1, 9], deep excision for larger acral lesions followed by full-thickness graft [9], laser depilation for hypertrichosis and botulinum toxin for hyperhidrosis [6]. There are also reports of intralesional Nd-YAG laser usage leading to satisfactory results after two sessions [10]. The CARE Checklist has been completed by the authors for this case report, which is attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000533667).
Statement of Ethics
Ethical approval is not required for this study in accordance with local or national guidelines. Written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.
Conflict of Interest Statement
The authors have no conflicts of interest to declare.
Funding Sources
This case report had no funding sources from any sponsors.
Author Contributions
A.S., Y.S., A.G., and E.M. revisited the manuscript and critically evaluated the intellectual contents. All authors participated in preparing the final draft of the manuscript, revised the manuscript, and critically evaluated the intellectual contents. All authors have read and approved the content of the manuscript and confirmed the accuracy or integrity of any part of the work.
Data Availability Statement
The data that support the findings of this study are available on request from the corresponding author, E.M. (Elham Mazaherpour). The data are not publicly available due to their containing information that could compromise the privacy of research participants.