A case report of generalised eruptive syringoma: a rare variant of syringoma
Department of Dermatology, Adichunchanagiri Institute of Medical Sciences, Mandya, Karnataka, India
Received: 05 June 2021
Accepted: 31 July 2021
*Correspondence:
Dr. Yogesh Devaraj,
E-mail: yogeshdevaraj86@gmail.com
Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Syringomas are benign adnexal neoplasm of eccrine sweat duct usually affecting adult women. They present as firm, skin coloured to yellowish papules in a symmetrical distribution over periorbital region. Eruptive syringomas are infrequent and usually appear at uncommon sites. Herein, we report a case of a 21-year old girl with eruptive syringomas with an extensive involvement.
Keywords: Syringoma, Eccrine sweat duct, Skin coloured
INTRODUCTION
Syringomas are benign tumors derived from intraepidermal portion of the eccrine sweat ducts. They are classified into four clinical variants: localized syringoma, familial syringoma, syringoma associated with Down syndrome, and generalized syringoma that encompasses multiple and eruptive syringomas. Generalized eruptive syringoma is a rare entity usually seen in women mainly during or after puberty.1
CASE REPORT
A 21-year-old girl who presented with multiple skin-coloured asymptomatic papules over both infra orbital areas of the face since 2 years. She developed similar lesions on neck, trunk and upper limbs. Lesions started on face 2 years back and gradually spread to involve the neck, dorsum of hands, forearm and abdomen. Lesions were asymptomatic. There were no similar complaints in the family members. On examination, there were multiple grouped skin coloured and angulated papules on bilateral infra orbital areas (Figure 1).
Similar papules were present on the neck, dorsum of fingers, flexor aspect of forearms (Figures 2, 3 and 4) and over lower abdomen. Systemic examination was normal.
Routine investigations were all within normal limits. Skin biopsy was advised but patient refused the procedure because of fear of scar formation on the face. Clinically a diagnosis of generalised eruptive syringoma was made. Patient was treated with oral Isotretinoin 10 mg, topical tretinoin 0.05% cream along with moisturisers to prevent irritation. Lesions over the face were treated by radiofrequency ablation.
DISCUSSION
Syringomas are benign adnexal tumors of intraepidermal portion of eccrine sweat ducts. They were first described by Kaposi. Friedman and Butler classified four clinical variants of syringoma- a localised form, a familial form, a form associated with Down syndrome and generalised form that includes multiple and eruptive syringomas.2 Eruptive syringoma is a rare, clinically distinct variant of syringoma and was first described in 1887 by Jacquet and Darier.1 It usually presents before or during puberty affecting about 0.6% of the general population. Females are more commonly affected. The pathogenesis of eruptive syringoma is not fully understood. According to one theory, a hyperplastic reactive process occurs in eccrine duct due to previous cutaneous inflammatory process, because many cases of eruptive syringomas were reported after cutaneous inflammatory process like contact dermatitis, shaving, laser hair removal, alopecia areata, radiation dermatitis.3 Clinically lesions are small, firm, smooth, skin coloured or slightly yellowish, 1 to 5 mm papules occurring in periorbital areas. They may also occur on thigh, axilla, abdomen and vulva. Estrogen and progesterone receptors have been detected within syringoma on histological studies. This explains why they are more common in females with a peak incidence during puberty. On histopathology, epidermis is unremarkable. Upper and mid dermis show multiple ducts and small solid epithelial nests, cords or tubules embedded in a sclerotic stroma. Lumina of the ducts are filled with amorphous debris. Ducts are lined by two rows of flat epithelial cells. Some of the ducts possess small comma like tails of epithelial cells giving them a tadpole appearance.4 Treatment of syringoma is generally unsatisfactory, as they are located in the dermis and often numerous. Physical techniques such as excision, electrofulguration, cryotherapy and dermabrasion yield poor cosmetic results. Oral isotretinoin and topical tretinoin and adapalene have been used. Ablative techniques such as the CO2 laser has been tried with variable success. However, recurrence rate is high. One study demonstrated good results with temporary tattooing, following Q-switched alexandrite laser. Unfortunately, all surgical interventions result in scarring.5
CONCLUSION
In our patient, the lesions developed a few years after puberty. She did not give any history of trauma or other precipitating factors. We treated her with oral Isotretinoin 10 mg, Tretinoin 0.05% cream and moisturiser for lesions over the body. Lesions over face were treated with Radiofrequency ablation. Patient had good improvement. She was asked to follow up regularly so that any recurrence of lesions can be picked up early and treated accordingly. This case is being reported for its rarity.
Funding: No funding sources
Conflict of interest: None declared
Ethical approval: Not required
REFERENCES
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