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Table of Contents
CORRESPONDENCE
Year : 2022  |  Volume : 40  |  Issue : 2  |  Page : 124-125

Eruptive syringoma with unusual positional accentuation as erythematous papules of forearms


1 Department of Dermatology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan
2 Department of Dermatology, Linkou Chang Gung Memorial Hospital; College of Medicine, Chang Gung University, Taoyuan, Taiwan

Date of Submission15-Feb-2021
Date of Decision22-Mar-2022
Date of Acceptance05-Apr-2022
Date of Web Publication29-Jun-2022

Correspondence Address:
Dr. Yu Ting Lin
Department of Dermatology, Wan Fang Hospital, Taipei Medical University, No. 111, Sec. 3, Xinglong Road, Wenshan Dist., Taipei City 116
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ds.ds_22_22

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How to cite this article:
Yen H, Yen H, Lin YT. Eruptive syringoma with unusual positional accentuation as erythematous papules of forearms. Dermatol Sin 2022;40:124-5

How to cite this URL:
Yen H, Yen H, Lin YT. Eruptive syringoma with unusual positional accentuation as erythematous papules of forearms. Dermatol Sin [serial online] 2022 [cited 2022 Aug 13];40:124-5. Available from: https://www.dermsinica.org/text.asp?2022/40/2/124/349026



Dear Editor,

Syringomas are skin adnexal tumors with characteristic histopathological features that most commonly present as skin-colored or pigmented asymptomatic periorbital lesions in females.[1] These benign tumors are thought to arise from intraepidermal eccrine ducts.[2] Previously considered neoplastic in origin, there has been evidence that syringomas are a hyperplastic proliferative response as part of an inflammatory process of the upper eccrine duct.[3] Previously, a classification scheme with four main clinical variants was proposed: (1) localized, (2) generalized including multifocal and eruptive syringomas, (3) Down's syndrome-related, and (4) familial.[1] We report an unusual case of eruptive syringoma where the skin-colored papules on the forearms became erythematous with change of arm position.

A 59-year-old man presented to our outpatient clinic with numerous asymptomatic erythematous papules on his arms for 10 years. On examination, multiple erythematous small papules ranging from 2 mm to 3 mm in diameter were noted in symmetric distribution on both the extensor and dorsal sides of bilateral forearms. Of note, the papules on his forearms were only erythematous when his arms were relaxed and positioned straight down parallel to his trunk [Figure 1]a but would immediately become skin-colored and less perceptible when he raised his arms above his head [Figure 1]b. Close-up images are provided in [Figure 1]c and [Figure 1]d. Punch biopsy of an erythematous papule on the left arm in a relaxed position was arranged. Histopathology demonstrated epithelial cells arranged in cords and small ductal structures within the dermis with a “comma-shaped” tail [Figure 2]. There were no obvious dilated capillaries in the dermis. No increased mast cells were highlighted by CD117 and Giemsa stains. Taken together with the clinical presentation, the patient was diagnosed with eruptive syringoma. As the lesions were asymptomatic, the patient decided not to pursue further treatment.
Figure 1: Multiple erythematous papules are present on the right forearm in a relaxed position straight down the side of the body (a), and immediate change to less perceptible, skin-colored papules when the right forearm is raised above the head (b). Close-up highlighting one specific papule (arrow) that is erythematous when in a relaxed position (c) and transitioning to skin-colored when in the raised position (d). Clinical photographs have been rotated for ease of comparison.

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Figure 2: Skin biopsy demonstrating epithelial cells arranged in cords and small ductal structures within the dermis with a “comma-shaped” tail (H and E stain, ×400).

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One review of 239 syringoma cases noted that 92% of syringomas occurred in females and 70% presented during adolescence.[1] The most frequent variant of syringoma was the localized form (almost 89%), predominantly in the periorbital region.[1] In contrast, eruptive syringomas are less common and present as multiple groups of small papules on the trunk, neck, abdomen, and extremities.[1] Symmetric involvement of the distal extremities and acral regions is rare,[4] and to our knowledge, this is the first reported case of eruptive syringoma with positional accentuation of lesions.

One possible explanation for the positional accentuation of the papules is the local release of histamine from mast cells causing an urticarial wheal and flare after stroking lesional skin, similar to Darier's sign in cutaneous mastocytosis. However, the erythematous change could be elicited simply by lowering the arms into a relaxed position without any other trigger, and no abnormal increase in mast cells was identified on skin biopsy. More likely, there is possibly a component of capillary ectasia accompanying the syringoma. This vascular component could become more apparent due to elevated blood pressure when the arms are lowered due to gravity.[5] Blood pressure measurements have been noted to be elevated by 8.8–14.4 mmHg when the arm is relaxed in a parallel position to the body compared to raised perpendicular to the body.[6] Although these vascular components were not readily identifiable in our pathological specimen, this could be attributable to the local anesthetic used during the biopsy procedure, which contains epinephrine and can cause vasoconstriction of the capillaries.

Differential diagnoses for syringoma include other adnexal tumors such as hidrocystoma and trichoepithelioma, cutaneous mastocytosis, angiofibroma, and fibroelastic papulosis.[1] Skin biopsy can be helpful as the distinctive histopathological features are diagnostic. These include multiple dermal small ducts and epithelial strands with some ductal structures demonstrating characteristic “comma-shaped” tails.[1],[4] Other skin diseases that can be affected by the positioning of the limbs include varicose veins, piezogenic pedal papules, and Bier spots, which may be differentiated clinically. The former two are more prominent on the lower limbs when standing,[7] while the latter may occur on both the upper and lower extremities and disappears with elevation.[8] Systemic associations of syringomas include Down syndrome and diabetes mellitus.[1] Treatment options include CO2 laser, surgical excision, or medical therapy such as topical retinoids.[1]

In conclusion, we report a case of eruptive syringoma which presented as erythematous papules of the forearms with an unusual transient positional presentation.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

Dr. Hsi Yen, an editorial board member at Dermatologica Sinica, had no role in the peer review process of or decision to publish this article. The other authors decalared no conflicts of interest in writing this paper.



 
  References Top

1.
Williams K, Shinkai K. Evaluation and management of the patient with multiple syringomas: A systematic review of the literature. J Am Acad Dermatol 2016;74:1234-40.e9.  Back to cited text no. 1
    
2.
Langbein L, Cribier B, Schirmacher P, Praetzel-Wunder S, Peltre B, Schweizer J. New concepts on the histogenesis of eccrine neoplasia from keratin expression in the normal eccrine gland, syringoma and poroma. Br J Dermatol 2008;159:633-45.  Back to cited text no. 2
    
3.
Guitart J, Rosenbaum MM, Requena L. 'Eruptive syringoma': A misnomer for a reactive eccrine gland ductal proliferation? J Cutan Pathol 2003;30:202-5.  Back to cited text no. 3
    
4.
Patel K, Lundgren AD, Ahmed AM, Soldano AC. Disseminated syringomas of the upper extremities in a young woman. Cureus 2018;10:e3619.  Back to cited text no. 4
    
5.
Byrd JB, Brook RD. Arm position during ambulatory blood pressure monitoring: A review of the evidence and clinical guidelines. J Clin Hypertens (Greenwich) 2014;16:225-30.  Back to cited text no. 5
    
6.
Hemingway TJ, Guss DA, Abdelnur D. Arm position and blood pressure measurement. Ann Intern Med 2004;140:74-5.  Back to cited text no. 6
    
7.
Ma DL, Vano-Galvan S. Piezogenic pedal papules. CMAJ 2013;185:E847.  Back to cited text no. 7
    
8.
Fan YM, Yang YP, Li W, Li SF. Bier spots: Six case reports. J Am Acad Dermatol 2009;61:e11-2.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]



 

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