Eruptive syringoma: a case report

INTRODUCTION

Syringoma, derived from the Greek word syrinx literally means a pipe or a tube. Syringomas are a group of benign adnexal neoplasm which tend to undergo ductal (acrosyringeal) differentiation. Though adult women are most commonly affected, it can occur in both the sexes.¹ Although occasionally solitary, the lesions are usually multiple and may be present in a great number. Clinically lesions are small, firm, smooth, skin coloured or slightly yellowish, 1-2 mm papules and tend to occur in the periorbital area, particularly around the lower eyelid.¹ In eruptive syringomas, a rare variant first described by Jacquet and Darier in 1887, the lesions occurred in successive crops on the anterior chest, neck, upper abdomen, axillae and the periumbilical region at puberty or during childhood. They may remain stationary throughout the life or may disappear spontaneously. There is also a familial variant. There are only few reports of its widespread distribution as eruptive syringoma in the literature.^2-6 We, therefore, report this rare variant in an adult female.

CASE REPORT

A 30 years old female presented with multiple asymptomatic, skin coloured lesions of 10 years duration with increasing cosmetic disfigurement. Lesions started erupting over face including periorbital region spreading to neck, upper chest as well as thighs. Patient was not on any medication. Family history was unremarkable. Examination revealed multiple, discrete, well defined, skin coloured papules over face, neck, chest and thighs of sizes varying from 1 to 4 mm (Figure 1A and B). Clinically, a differential diagnosis of epidermodysplasia verruciformis, papular granuloma annulare, eruptive syringoma, papular sarcoidosis, steatocystoma multiplex were considered.

Histopathological examination revealed normal epidermis. Dermis showed collection of cystic ducts and epithelial cords with comma like tails. No evidence of granuloma or malignancy was seen (Figure 2 A and B). The histopathological findings were consistent with the clinical diagnosis of eruptive syringoma.

DISCUSSION

Syringomas are benign adnexal tumours originating from intraepidermal portion of eccrine sweat ducts. The initial description of syringoma was given by Kaposi; later, Jacquet and Darier described the eruptive form.^1,2 Since its description, few cases have been reported.³ Syringoma has been classified into four variants: Localized, lesions associated with Down's syndrome, generalized eruptive syringoma and a familial autosomal dominant form.^1,6 Clinically, lesions are small, firm, smooth, skin-coloured or slightly yellowish, 1-5 mm papules tending to occur in the peri-orbital area, particularly around the lower eyelid. They may occur in other sites like thighs, axillae, abdomen and vulva. They have a tendency to develop during puberty or in the third or fourth decade.^1,4 In cases of eruptive syringomas there are successive crops of numerous disseminated papules, with a predilection to occur over the anterior trunk, that is, on anterior aspect of the neck, chest, trunk, axilla, and inner aspect of upper arm and around axilla.^3,4 A positive association between eruptive syringoma and heat stimuli has also been found.⁵ There have been reports of unilateral, nevoid, bathing trunk, and generalized distribution of syringoma.⁶

Estrogen and progesterone receptors have been detected within syringoma with histochemical study. This finding may explain why syringomas are more common in females and peak incidence during puberty.⁷ On histopathology of syringoma, epidermis is unremarkable. The dermis shows multiple ducts and solid epithelial nests, cords, tubules embedded in a sclerotic stroma in the upper and middle parts. 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 the appearance of tadpole.⁸ Cells of epithelial proliferation have pale, eosinophilic cytoplasm and rounded monomorphic nucleus. Histochemical studies have shown that all eccrine types of enzymes and glycogen are present in the tumor cells of syringoma. Eccrine-specific monoclonal antibody positively stains syringoma lesions. Hence, although formerly thought to be of mixed origin, now syringoma is considered to be a benign appendageal tumor of intra-epidermal eccrine sweat duct.⁶ Clinical diagnosis of syringomas is often difficult due to a similar presentation of widespread papular eruptions such as epidermodysplasia verruciformis, papular granuloma annulare, papular sarcoidosis, lichen planus, flat warts, papular mucinosis, xanthoma disseminatum, steatocystoma multiplex, acneiform eruptions, and mastocytosis. Histopathology of the lesions often confirms the diagnosis with its definite classical findings. Our patients sought medical advice because of cosmetic concern. Multiple treatment options are currently available including surgical excision, dermabrasion, and electrodessication with curettage, laser resurfacing, chemical peeling, cryotherapy, fulguration, oral and topical retinoids, and 1% topical atropine.¹¹ But none of them are satisfactory, and recurrence is common because these tumours are situated deep in the dermis and are numerous in numbers.^9,10 We advised the patient CO² fractional laser. The prognosis of the disease of possible recurrence was explained to the patient upon treatment. Syringomas occur more frequently in females on the face in a localized manner, but it is important to consider this as a differential diagnosis of any eruptive papular lesions occurring throughout the body. The specific histopathological features would clinch a final diagnosis and would be the most important diagnostic tool.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

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