Vulvar Nonclear Cell Syringoma Associated with Pruritus and Diabetes Mellitus

Abstract

Background. Syringoma is a benign eccrine sweat gland tumor, characterized by multiple skin colored or slightly yellowish papules. Vulvar syringoma without extragenital involvement is an extremely rare variant of syringoma. Mail Observations. Herein, we report a 40-year-old diabetic female patient who presented with only lichenified plaques on the vulvar region without any extragenital lesions. Diagnosis of vulvar syringoma was made depending on the characteristic double lined cystic enlargements and comma-like tails found in histopathological examination. Immunohistochemical stains for estrogen and progesterone receptors were negative. Conclusion. Vulvar syringoma may present with no obvious papules on lichenified plaques.

Syringoma, hormone receptors and associated endocrinopathies. Are they clinically relevant?

“…Later on, Wallace and Smoller showed nuclear and cytoplasmic staining with ER in 1/9, and with PR in 8/9 of syringomas [3]. Since then in some studies, ER and/or PR were detected in syringomas, but some other studies did not get the same result as in our case [1,[4][5][6][7][8][9][10][11] (Tab. I).…”

“…The severity of pruritus and the size of the lesions may increase during menstruation, pregnancy and use of oral contraceptives. Based on these findings, it has been suggested that the growth of the syringomas including VS are at least partially under control of hormones [1][2][3][4]. The first authors pointed to this issue are Swanson et al, who investigated immunoreactivity for ER protein in sweat gland tumors [2].…”

“…Endocrinopathies associated with non-clear cell syringomas are scant and listed in Table 2 [4,[12][13][14]. It is not known whether the treatment of associated disorder affects the regression or clearance of syringomas in the patients with DM or other endocrinopathies.…”

“…The etiopathogenesis of syringomas is not clear yet, but hormonal influences have been suspected [1]. There are some reported cases of syringomas manifesting lesional estrogen receptor (ER) and progesterone receptor (PR) positivity [1][2][3][4]. Herein, we present a case of vulvar syringoma (VS) and in this context, we review the endocrinological abnormalities associated with syringomas, reported up to date, and discuss the clinical importance of lesional ER and PR positivity.…”