Vulvar syringoma showing progesterone receptor positivity

Abstract

Case reportA 27 year old woman was seen in our clinic for pruritus vulvae. Physical examination revealed multiple 3-5 mm sized symmetrical skin coloured papules on both labia majora (Fig. 1). The lesions had been present for three years and were pruritic. There were no similar lesions elsewhere in the body. There was no family history of similar lesions. An incisional biopsy was performed under local anaesthesia and the histopathological diagnosis was syringoma (Fig. 2).She was admitted and the lesions on both labia majora were excised totally under local anaesthesia. The skin was closed by subcutaneous mattress sutures with 4/0 prolene. Sutures were removed on the seventh postoperative day. One year later, no scar was seen on the incision site and there was no pruritus.The specimen showing multiple syringoma was further investigated for oestrogen and progesterone receptor positivity by immunohistochemistry, which revealed 70% nuclear progesterone positivity within the eccrine glands of syringoma (Fig. 3). Similarly normal eccrine glands in the deeper dermis showed nuclear progesterone expression (arrow, Fig. 3). There was also some nonspecific cytoplasmic progesterone staining within the lesion.

Syringoma: A Clinicopathologic and Immunohistologic Study and Results of Treatment

“…Wallace and Smoller13 reported that eight of nine syringoma cases showed strong nuclear and cytoplasmic progesterone receptor positivity, supporting the hypothesis of hormonal influence on syringoma. Yorganci et al14 reported a vulvar syringoma showing progesterone receptor positivity. Timpanidis et al15 also reported an eruptive clear cell syringoma associated with diabetes in which immunohistochemical staining showed negativity for ER and strong positivity for PR.…”

Vulvar Nonclear Cell Syringoma Associated with Pruritus and Diabetes Mellitus

“…Patients with vulvar syringoma may complain of severe itching [4–6]. Increased pruritus during menstruation and detection of ER and PR in some patients suggested that hormonal factors play role in the development of vulvar syringomas [4, 7, 8]. Pruritus was frequent in the largest series reported by Huang et al; however, investigators did not detect positivity for ER and PR [5].…”

Rare sweat gland tumors of vulva: Report of two cases

“…In a case report by Yorganci et al ., immunohistochemistry of vulvar syringoma was shown to display progesterone positivity. [5] Vulvar syringomas with vulvar prutitus has been reported in a 9 year old child. [6] Venerophobia may sometimes be associated with genital lesions.…”