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  Table of Contents  
CLINICOPATHOLOGICAL CHALLENGE
Year : 2021  |  Volume : 12  |  Issue : 2  |  Page : 368-370  

Hairless hyperhidrotic plaque in a young male


1 Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission18-Jun-2020
Date of Decision05-Aug-2020
Date of Acceptance05-Sep-2020
Date of Web Publication22-Feb-2021

Correspondence Address:
Keshavamurthy Vinay
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_467_20

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   Abstract 


An 18-year-old male presented with an erythematous plaque on the left side of face and neck since 4 years. The lesion was devoid of hair, had follicular prominences, and showed increased localized sweating. Histopathology revealed an increased number of eccrine glands in the myxoid stroma with multiple vascular elements in the dermis, favoring the diagnosis of eccrine angiomatous hamartoma. This case illustrates a relatively uncommon clinical presentation of eccrine angiomatous hamartoma as a hairless, hyperhidrotic plaque in the head and neck region.

Keywords: Eccrine angiomatous hamartoma, hamartoma, hyperhidrosis


How to cite this article:
Kaushik A, Chatterjee D, Vinay K. Hairless hyperhidrotic plaque in a young male. Indian Dermatol Online J 2021;12:368-70

How to cite this URL:
Kaushik A, Chatterjee D, Vinay K. Hairless hyperhidrotic plaque in a young male. Indian Dermatol Online J [serial online] 2021 [cited 2021 Apr 15];12:368-70. Available from: https://www.idoj.in/text.asp?2021/12/2/368/308916




   Clinical Findings Top


An 18-year old male presented with an erythematous plaque on the left side of face and neck of 4 years duration. The plaque had gradually increased in size, showed absence of hair and was associated with increased localized sweating. On local examination, a well-defined erythematous to brownish plaque was present on the left side of face, extending from angle of jaw to the lateral aspect of the neck [Figure 1]. There were increased follicular prominences and a complete absence of hair growth on the lesion. No pulsations were felt and no bruits were heard on auscultation. Systemic examination was unremarkable. Routine laboratory tests, including complete hemogram as well as acute phase reactants (like C-reactive protein) were within normal limits. Biopsy was taken from the lesion and evaluated.
Figure 1: Erythematous plaque on the left side of face, extending from angle of jaw to the lateral aspect of the neck, with follicular prominence and paucity of hair on the lesion

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   Histological Findings Top


Histopathological examination of the biopsy specimen from the lesion revealed an increased number of eccrine glands in myxoid stroma, multiple thick-walled blood vessels, and a few immature hair follicles in the dermis [Figure 2]a and [Figure 2]b]. Immunohistochemistry was performed using CD31 immunostaining to delineate the presence of vascular elements in the dermis [Figure 2]c.
Figure 2: Histopathology of skin biopsy from the lesion showing (a) increased number of eccrine glands in myxoid stroma, multiple thickened blood vessels and few immature hair follicles in the dermis (H and E, ×100). Similar findings are seen in (b) (H and E, ×200) while (c) shows CD31 immunostaining highlighting the blood vessels (×100)

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   Diagnosis Top


Eccrine angiomatous hamartoma (EAH).


   Clinical Course Top


Benign nature of the lesion was explained and available therapeutic options including surgical excision were offered to the patient. However, he decided against opting for any specific therapy.


   Discussion Top


EAH is a benign lesion of hamartomatous origin, characterized histopathologically by proliferation of eccrine sweat glands and capillary-like vessels in the dermis.[1] The disease shows no gender or racial predilection and primarily affects young children, though cases have been described in adolescents and adults as well.[2] The exact pathogenesis is unclear; however, faulty biochemical interactions between the differentiating epithelium and mesenchyme leading to abnormal eccrine and vascular proliferation have been proposed.[3] While distal extremities are a common site, occurrence on head and neck has also been reported. Clinical presentation is variable and can take the form of patches, plaques, or nodules in varying shades of discoloration including bluish-black, violaceous, erythematous, or flesh-colored. The lesions can be asymptomatic or associated with pain (42%) due to nerve compression, hyperhidrosis (32%) due to eccrine glandular proliferation and hypertrichosis.[1],[4],[5] Epidermis is mostly uninvolved although verrucous or ulcerative changes may sometimes be seen. Although hypertrichosis is described as a relatively common feature of EAH, our case had a novel presentation in the form of clinical absence of hair and the histopathological finding of immature hair follicles.

Histopathological examination is vital and to be performed in all cases, which will show the characteristic features as described. Other investigations that are sometimes useful in work-up include ultrasound Doppler (can indicate location of lesion in subcutaneous plane and to rule out vascular malformation prior to biopsy).[6] Although not commonly performed, dermatoscopy features have been described in some studies and may show popcorn or spitzoid patterns.[7] Differential diagnoses include tufted angiomas, eccrine nevus, vascular malformations, and sudoriparous angiomas. While considered to be part of the same spectrum, sudoriparous angiomas show predominant angiomatous proliferation affecting large-caliber vessels and minimal eccrine hyperplasia, whereas EAH shows vascular proliferation affecting capillaries and also shows eccrine proliferation, as described.[4] Clinicopathological correlation can reasonably differentiate EAH from other entities.

The natural history of EAH is benign and while asymptomatic lesions do not require any therapy, lesions associated with pain or debilitating hyperhidrosis benefit from surgical excision. In addition, botulinum toxin injection has been used for control of hyperhidrosis.[8] Some patients may benefit from laser therapy (pulsed-dye and CO2) and sclerosants.[4]

This case illustrates a relatively uncommon clinical presentation of EAH as hairless, hyperhidrotic plaque in the head and neck region and the importance of clinical and pathological correlation in such cases.

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.
Patterson AT, Kumar MG, Bayliss SJ, Witman PM, Dehner LP, Gru AA. Eccrine angiomatous hamartoma: A clinicopathologic review of 18 cases. Am J Dermatopathol 2016;38:413-7.  Back to cited text no. 1
    
2.
Tantanasrigul P, Kootiratrakarn T, Wessagowit V, Kattipathanapong P, Sudtikoonaseth P. Eccrine angiomatous hamartoma in an adolescent. Case Rep Dermatol 2015;7:233-6.  Back to cited text no. 2
    
3.
Smith SD, DiCaudo DJ, Price HN, Andrews ID. Congenital eccrine angiomatous hamartoma: Expanding the morphologic presentation and a review of the literature. Pediatr Dermatol 2019;36:909-12.  Back to cited text no. 3
    
4.
Gupta V, Bhatia S, Arava S, Sharma VK. A hairy port-wine stain with overlying hyperhidrosis on the thigh of a child. Int J Dermatol 2017;56:1349-51.  Back to cited text no. 4
    
5.
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. 5
[PUBMED]  [Full text]  
6.
Mendiratta V, Malik M, Agrawal M, Jain M, Gupta B. Congenital eccrine angiomatous hamartoma: A rare entity revisited. Indian Dermatol Online J 2018;9:188-90.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Kelati A, Chiaverini C, Giacchero D, Ilie M, Lacour J-P, Bahadoran P. Dermoscopy of eccrine angiomatous hamartoma: The spitzoid pattern. JAAD Case Rep 2018;4:835-6.  Back to cited text no. 7
    
8.
Barco D, Baselga E, Alegre M, Curell R, Alomar A. Successful treatment of eccrine angiomatous hamartoma with botulinum toxin. Arch Dermatol 2009;145:241.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]



 

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