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Generalised Eruptive Syringomas: a Diagnostic and Cosmetic Conundrum

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Generalised Eruptive Syringomas: a Diagnostic and Cosmetic Conundrum

Chetna Singla,1MD, Pritika Sharma,2 MD

Address: 1Senior Resident, Sri Guru Ram Das University of Medical Sciences and Research, 2M.B.B.S. student, Government Medical College, Amritsar.

E-mail: singhal.chetna18@gmail.com

*Corresponding Author:Dr. Senior Resident, Sri Guru Ram Das University of Medical Sciences and Research, Amritsar.

Case Report DOI: 10.6003/jtad.18122c3

Published:

J Turk Acad Dermatol 2018; 12 (2): 18122c3

This article is available from: http://www.jtad.org/2018/2/jtad18122c3.pdf

Key Words: Generalised Eruptive Syringomas, Verruca Plana, Epidermodysplasia Verruciformis.

Abstract

Observation: Syringomas are benign, adnexal tumours that are derived from the intraepidermal portion of the eccrine sweat ducts. Syringomas are usually sporadic but familial forms have been seen rarely.

Lesions are in the form of bilaterally symmetrical firm skin colored papules which are commonly seen in periorbital areas and are cosmetically a concern. Even though periorbital is the most common location, rare forms have been described. Generalised eruptive syringomas is a rare form. Here, we report a patient with sudden eruptive syringomas that included the whole body posing a difficulty in the diagnosis.

Introduction

Syringomas are common, benign, adnexal tu- mours that are derived from the intraepider- mal portion of the sweat ducts. The word syringoma is derived from the Greek word

‘syrinx’ that means a tube [1]. These are more commonly seen in females and periorbital area is the most common site involved. Fried- man and Butler described four types of syrin- gomas- localised, generalised, familial and those associated with Down Syndrome [2].

Syringomas present as asymptomatic, mul- tiple, firm, skin colored papules that are b ilaterally symmetrical in distribution [3]. Va- rious treatment modalities have been tried that may include chemical cautery, electro- cautery, cryotherapy, excision, dermabr asion, topical and oral retinoids and CO2 la- sers, with variable results [4]. Diagnosis is cli- nical and biopsy is only required in case of atypical presentation. Such an atypical pre- sentation with generalised eruptive syringo-

mas in an adult female patient is being pre- sented.

Case Report

A 23 year-old female presented to the skin OPD with 15 years old history of multiple papules over whole body. The lesions were asymptomatic. These initially developed over face and then gradually spread over to rest of the body in one year. After that the lesions remained stable. There was no fa- mily history or any history of similar complains in the past. Mucocutaneous examination revealed bi- laterally symmetrical, multiple, firm, skin colored and flat topped papules with varying sizes and well defined irregular margins which were present over face, trunk, bilateral upper and lower limbs (Fi- gures 1a and b) (Figure 2). Examination of scalp, nails and mucous membranes was found to be normal. There were no associated cutaneous or systemic diseases. Based on history and clinical examination, a differential diagnosis of verruca plana, epidermodysplasia verruciformis and erup- Page 1 of 3

(page number not for citation purposes)

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tive syringomas and generalised lichen planus was kept. After taking informed consent, skin punch biopsy was taken from lesion on arm which repor- ted an epithelial neoplasm involving upper reticu- lar dermis and made up of solid epithelial islands and ductal structures which were lined with two or three layers of cuboidal cells. These cells pre- sent in solid cords showed attempt towards ductal differentiation. Stroma was made up of thickened collagen bundles with impression of syringoma. All other routine laboratory investigations were nor- mal. Based on clinical and histopathology findings, a final diagnosis of generalised eruptive syringo- mas was made and patient was treated with isot- retinoin 20mg daily. The degree of response to treatment could not be seen as the patient did not come for follow up.

Discussion

Syringomas are common benign adnexal tu- mours. These arise from intraepidermal por- tion of eccrine sweat glands. They usually arise during puberty and are more common in females [5,6] Periorbital location is the most common, but other forms may also be seen.

Ours was one such unusual case with gene- ralised eruptive syringomas in an adult fe- male. Syringomas may resolve spontaneously or may require treatment which includes che- mical cautery, electrocautery, cryotherapy, dermabrasion, excision, topical and oral reti- noids and CO2 laser. The results have been variable. The only concern is that the lesions are cosmetically concerning for the patient.

Our patient was started on isotretinoin 20 mg daily keeping in mind the generalised nature of the disease.

To conclude we can say that syringomas are benign but they can be cosmetically unappea- ling and a cause of concern for the patient and when the presentation is rare, they pose a di- agnostic challenge for the doctor. Treatment is usually unsatisfactory and should aim at co- unselling the patient regarding benign nature of the disease.

References

1. Korekawa A, Nakajima K, Nishikawa Y, Matsuzaki Y, Nakano H, Sawamura D. Late-onset, eruptive syrin- gomas in an elderly man: correlation with carbama- zepine. Acta Derm Venereol 2012; 92: 87-88. PMID:

21953350

2. Friedman SJ, Butler DF. Syringoma presenting as milia. J Am Acad Dermatol 1987; 16: 310-314. PMID:

3819065

J Turk Acad Dermatol 2018; 12(2): 18122c3. http://www.jtad.org/2018/2/jtad18122c3.pdf

Page 2 of 3

(page number not for citation purposes) Figures 1a and b. Showing presence of multiple papular lesions over trunk and arms (with inset showing close-up

of the abdominal lesions)

Figure 2. Showing typical epithelial neoplasm in reticu- lar dermis made up of solid epithelial islands with ductal structures and typical tadpole appearance of the

ducts.

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3. Jamalipour M, Heidarpour M, Rajabi P. Generalised eruptive syringomas. Indian J Dermatol 2009; 54 :65-67. PMID: 20049275

4. Teixeira M, Ferreira M, Machado S, Alves R, Selores M. Eruptive syringomas. Dermatol Online J 2005;

11: 34. PMID: 16409930

5. Patrizi A, Neri I, Marzaduri S, Varotti E, Passarini B.

Syringoma: A review of twenty-nine cases. Acta Derm Venereol 1998; 78: 460-462. PMID: 9833049 6. Soler-Carrillo J, Estrach T, Mascaro JM. Eruptive

syringoma: 27 new cases and review of literature. J Eur Acad Dermatol Venereol 2001; 15: 242-246.

PMID: 11683289

Page 3 of 3

(page number not for citation purposes) J Turk Acad Dermatol 2018; 12(2): 18122c3. http://www.jtad.org/2018/2/jtad18122c3.pdf

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