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Year : 2022 | Volume
: 40
| Issue : 2 | Page : 124-125
Eruptive syringoma with unusual positional accentuation as erythematous papules of forearms
Hsuan Yen1, Hsi Yen2, Yu Ting Lin1
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
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
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ds.ds_22_22
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.
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).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
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