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CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 4  |  Page : 431-434
Adult-onset Eccrine Angiomatous Hamartoma: Report of a Rare Case


1 Department of Dermatology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
2 Department of Pathology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
3 Iran University of Medical Sciences, Iran

Date of Web Publication17-Sep-2021

Correspondence Address:
Nader Pazyar
Department of Dermatology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_702_19

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How to cite this article:
Yaghoobi R, Pazyar N, Mostufi E, Hemmati E. Adult-onset Eccrine Angiomatous Hamartoma: Report of a Rare Case. Indian J Dermatol 2021;66:431-4

How to cite this URL:
Yaghoobi R, Pazyar N, Mostufi E, Hemmati E. Adult-onset Eccrine Angiomatous Hamartoma: Report of a Rare Case. Indian J Dermatol [serial online] 2021 [cited 2021 Dec 2];66:431-4. Available from: https://www.e-ijd.org/text.asp?2021/66/4/431/326139




Sir,

Eccrine angiomatous hamartoma (EAH) is a relatively rare hamartoma with unknown etiology. EAH usually originates from an abnormal interaction between the differentiated epithelium and the mesenchyme which results in atypical development of eccrine and vascular elements.[1]

A 28-year-old man was admitted to our hospital with a dark blue-purple lesion on his right wrist since 6 years ago. He noted that the lesion had grown gradually and steadily over the years. He denied any previous trauma to his wrist before the appearance of the lesion. The lesion did not have any pain or other symptoms. Familial history was negative, and he had not received any medication. Cutaneous examination showed a well-demarcated bluish-purple mass size 2 × 3 cm with an erythematous base on the flexural aspect of his right wrist. The lesion was soft and non-tender on palpation [Figure 1].
Figure 1: Dark blue - purple lesion on the flexural aspect of the right wrist

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A systemic review and physical examination were unremarkable. Routine laboratory tests were in normal limit. Ultrasound examination of the lesion showed a hypo-echoic lesion in the subcutaneous tissue sized 40 × 26 × 5 mm diameter without any invasion of underlying muscles. Clinically, differential diagnoses were angiokeratoma and hemangioma. A skin biopsy from the lesion was performed. The histopathological study revealed epidermal hyperkeratosis and papillomatosis with elongation of the rete ridges. The dermis showed an increased number of eccrine structures and numerous capillary channels surrounding them. Foci of hemorrhages were found in the deep dermis [Figure 2] and [Figure 3]. Alcian blue staining for mucin deposition was positive [Figure 4]. Histological diagnosis was consistent with eccrine angiomatous hamartoma. Immunohistochemistry (IHC) study showed S100 positivity in eccrine glands and CD31, CD34, and factor VIII-related antigen for delineation of vascular components [Figure 5]. After the confirmation of diagnosis, the patient was referred to a vascular surgeon for further treatment. No recurrence was seen at 6 months follow-up [Figure 6].
Figure 2: Hyperkeratosis and papillomatos with elongated epidermal rete ridges. The dermis shows increased number of eccrine structures and numerous capillary channels surrounding the eccrine structures (Hematoxylin and Eosin ×40)

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Figure 3: Abundant eccrine structures in the dermis with peri-adnexal capillary channels (Hematoxylin and Eosin ×100)

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Figure 4: Mucin deposition between eccrine gland structures and capillary blood vessels (Alcian blue staining ×400)

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Figure 5: Special stains showing S100 positivity of eccrine glands and CD 31, CD34, Factor VШ positivity of capillary vessels (×100 & 400)

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Figure 6: The view of EAH after total removal of the lesion

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Lotzbeck first explained EAH in 1859 and Hyman et al. coined the term EAH. It is characterized by the proliferation of eccrine glands and vascular structures. It may be congenital, appears at birth or during childhood, but rarely arise in adulthood period. There is no sex predilection.[1],[2] It clinically presents as a solitary nodule commonly affecting the extremities. Approximately 80% of the cases are reported on the extremities, with a predilection for palm and sole involvement.[2] When symptomatic, EAH can be related with hyperhidrosis or pain.[3]

Many underlying related conditions have been described in the literature. Lin et al. reported that EAH arose as a single lesion on a lower extremity in most cases. The symptoms and signs most commonly associated with EAH were pain, hypertrichosis, itching, and hyperhidrosis, but our patient did not report any of all of them. Additional findings related to EAH were hemangioma, verrucous hemangioma, arteriovenous malformation, and angiokeratoma.[4]

The precise pathogenesis of EAH is unidentified, but numerous theories such as abnormal induction of heterotypic dependency with resultant malformation of adnexal, also mesenchymal elements have been suggested. It has also been reported in association with radiation therapy and frequent trauma.[5] EAH has been documented in a patient with neurofibromatosis, Cowden syndrome, nevus depigmentosus, and nevus spilus.[3]

Diagnosis of EAH is based upon the histologic criteria described as[1] proliferation of mature, normal or dilated eccrine glands,[2] the intimate relationship of the eccrine structures with benign vascular proliferation,[3] and varying occurrence of pilar, lipomatous, mucinous, and/or lymphatic structures.[6]

The differential diagnoses of EAH includes tufted angioma, vascular malformations, macular telangiectatic mastocytosis, nevus flammeus, glomus tumor, smooth muscle hamartoma, congenital hamartoma of the eccrine sweat gland, eccrine nevus, and blue rubber bleb syndrome. The differentiation and confirmation of each of them can be performed based on histopathology and IHC studies.[7] In our case, in addition to full filling histological criteria, IHC confirmed specific positive staining for eccrine glands, and vascular elements.

The prognosis of EAH is usually considered beneficial.[4] The course of EAH is benign without spontaneous regression or malignant transformation. The lesion may expend in proportion with the patient's growth.[1] Surgical excision is curative and reserved for cases accompanying local pain or cosmetically disfiguring lesions, but recurrence is likely to occur.[1],[4],[7]

In summary, our patient was a typical case of EAH that demonstrated an increased number of eccrine glands and capillary blood vessels in histopathology and immunohistochemistry studies. Compared to the previously reported cases, EAH in our case appeared in adult age without any symptoms.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Eccrine Angiomatous Hamartoma in an Adolescent. Case Rep Dermatol 2015;7:233-6.  Back to cited text no. 1
    
2.
Hernot S, Wadhera R, Kaintura M, Bhukar S, Pillai DS. Angiomatous hamartoma-A rare presentation. J Clin Diagn Res 2016;10:MD04-5.  Back to cited text no. 2
    
3.
Kelati A, Chiaverini C, Giacchero D, Ilie M, Lacour JP, Bahadoran P. Dermoscopy of eccrine angiomatous hamartoma: The spitzoid pattern. JAAD Case Rep 2018;4:835-6.  Back to cited text no. 3
    
4.
Yamamoto T, Hirano M, Ueda K. Eccrine angiomatous hamartoma in a patient with nevus depigmentosus and nevus spilus. Indian J Dermatol 2017;62:99-100.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Sanusi T, Li Y, Sun L, Wang C, Zhou Y, Huang C. Eccrine angiomatous hamartoma: A clinicopathological study of 26 cases. Dermatology 2015;231:63-9.  Back to cited text no. 5
    
6.
Nygaard U, Dalager S, Spaun E2, Hedelund L. Large eccrine angiomatous hamartoma: A novel clinical presentation of disease. J Dermatol Case Rep 2015;9:58-61.  Back to cited text no. 6
    
7.
Verma P, Kaur M, Narula V, Ramesh V, Singh A, Saxena AK. Verrucous eccrine angiomatous hamartoma. Indian J Dermatol Venereol Leprol 2017;83:367-9.  Back to cited text no. 7
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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