Generalized Eruptive Syringoma

Abstract

Eruptive syringoma is a very rare variant of syringoma. It is a benign adnexal tumor of the intraepidermal portion of eccrine sweat ducts. Here we report a 32-year-old female presented with classical asymptomatic eruptive syringomas involving her face and extremities.

What was known?

Though syringoma is fairly common condition but its eruptive variant is rare, which present around the puberty. It usually occur after inflammatory condition and present with itchy multiple shiny papules.

Introduction

The word syringoma is derived from Greek language syrinx meaning tube. Syringomas are benign adnexal tumors of eccrine origin. It shows differentiation of intra-epidermal portion of eccrine duct and having four major variants as proposed by Friedman and Butler:[1] Localized, generalized including eruptive and multiple, familial and associated with Down syndrome. The usual site of predilection is periorbital and neck. While other sites like axillae, abdomen and extremities are also involved. Other rare forms like plaque type, linear, vulvar, penile, chondroid, scalp, acral, solitary over ankle were also reported in the literature.[2,3,4]

Case Report

A 32-year-old female presented in outpatient department with multiple skin to brown colored papules all over the face and upper extremity since last 1 month [Figure 1a and b]. The lesion developed spontaneously over forehead and gradually spread involving remaining face and the upper extremity. Since that time there was no change in the lesion. There was no similar history in the family. No significant medical or surgical history was present. She denied any use of medication prior to development of lesion. We kept differential diagnosis as trichoepitheliomas, hyperplasia of sebaceous glands, eruptive xanthomas and urticaria pigmentosa. Darrier sign was negative. General examination was normal including laboratory and hematological investigations.

Figure 1.

(a) Multiple, bilaterally symmetrical, flat topped, skin to brown colored papules all over the face and upper extremity (b) Multiple, flat topped skin to brown colored papules over the face and upper extremity

On cutaneous examination multiple, symmetrical skin colored to brownish shiny papules of diameter 2-4 mm was present all over the face and upper extremity. The remaining physical examination was normal without any history of photosensitivity. Mucosal surface was not involved. Systemic examination was unremarkable. Skin biopsy of the facial lesion showed a normal epidermis with benign proliferation of multiple eccrine ducts lined by two rows of epithelial cells embedded in fibrotic stroma of the upper dermis and some of which having tadpole-like appearance and having an amorphous material in the ductal lumina [Figure 2]. So on the basis of clinicohistopathological correlation, the diagnosis of eruptive syringoma was made.

Figure 2.

Multiple eccrine ducts embedded in fibrotic stroma of dermis with tadpole-like appearance (H and E, ×40)

Discussion

Eruptive syringoma is a rare, uncommon clinically distinct variant of syringoma. It was first described in 1887 by Jacquet and Darier. It usually presents before or during puberty affecting about 0.6% of the general population. It occurs more frequently in females as compared to males. The lesions are usually bilaterally symmetrical that occurs in successive crops with flat topped, angulated, skin to brownish shiny papules having both follicular and non-follicular involvement. It is usually associated with pruritus which was absent in our case. It usually spares palms, soles and mucosal surfaces.[5] Eruptive syringoma may be associated with Down's syndrome, milium, elevation of carcinoembryonic antigen, diabetes, psychiatric disorders, Ehler Danlos syndrome, prurigo nodularis, melanocytic nevi and sarcoidosis. It may rarely simulate urticaria pigmentosa.[6,7]

The pathophysiology for development of eruptive syringoma is still not fully understood. But the proposed and accepted theory involves previous cutaneous inflammatory process leading to hyperplastic reactive process in eccrine duct due to inflammation because many cases of eruptive syringomas were reported after cutaneous inflammatory process like contact dermatitis, shaving, laser hair removal, alopecia areata, radiation dermatitis etc., but some authors believes that it is a neoplastic process which is less acceptable theory for development of lesion.[8,9]

Treatment

Eruptive syringomas often creates significant cosmetic concerns for patients and may be unacceptable in the society. It may go under spontaneous regression over the period of time but t he course is largely unpredictable Therefore treatment of syringoma is mainly cosmetic and not standardized which includes excision, dermabrasion, cryo-therapy, electrodesiccation; chemical peels especially trichloroacetic acid, oral and topical retinoids, topical atropine, carbon dioxide laser, pulsed dye laser and anti-glandular eccrine monoclonal antibodies. But the risk of scarring especially over face remains with surgical and chemical modalities of treatment. But none is prevent risk of recurrence with variable result.[10,11]

What is new?

We describe a case of generalised eruptive syringoma having late onset of presentation and was totally asymptomatic without any prior inflammatory disease.