Morphology XXX Blackwell Oxford, International IJD © var_onlineissn 0011-9059 2008 The UK Publishing International Journal Ltd of Dermatology Society of Dermatology

Vulvar pruritus caused by syringoma of the vulva Vulvar pruritus Kavala MORPHOLOGY et al. caused by syringoma of the vulva

Mukaddes Kavala, MD, Burçe Can, MD, Ilkin Zindanci, MD, Emek Kocatürk, MD, Zafer Türkoglu, MD, Nesimi Büyükbabani, MD, and Melek Koç, MD

From the Department of Dermatology, Göztepe Training and Research Hospital, Istanbul, Turkey and Department of Pathology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey Correspondence Burçe Can, MD Güvenç Sok. Beyaz Evler No: 23 Da: 2 Göztepe, Istanbul Turkey E-mail: [email protected]

Case Report A 20-year-old woman presented with a 1.5-year history of pruritic papules on her vulva. There was no past history of eczema or contact sensitization. Cutaneous examination revealed multiple, 1–3 mm sized, skin-colored papules, distributed bilaterally over the labia majora (Fig. 1). No other similar lesions were detected on the rest of the body. Histological examination of the lesion showed a tumoral lesion containing ductal structures formed by epithelial cells some of which were clear cytoplasm embedded in fibrotic stroma (Fig. 2). This appearance was diagnostic of a syringoma. Discussion Syringomas are tumors of ecrine sweat glands that appear as multiple, firm or soft, skin-colored to transparent papular lesions. They frequently involve the face, neck and chest, and occasionally the genital region. The lesions develop generally during adolescence or adult life and are seen more frequent in females.1–3 Sporadically seen syringomas may rarely be familial.4 There is no association with systemic diseases but coexistence with Down syndrome has been reported.5 Vulvar syringomas are considered to be rare clinical variant and symmetrically distributed on the labia majoras. Vulvar syringomas which are usually asymptomatic and noticed during routine gynecologic examination may cause severe pruritus occasionally in the genital region. One of the reported cases with pruritus had a periodic pruritus which tended to exacerbate during menstruation.2,6,7 In our case the pruritus had a constant pattern. Syringomas are rarely seen as a solitary lesion. A 5 mm sized syringoma on the vulva has been © 2008 The International Society of Dermatology

Figure 1 Multiple skin-colored papules over labia majora

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cream and oral antihistaminic therapy was started which improved the pruritus but not the syringomas. Cryotherapy of the lesions was advised, but the patient refused the treatment. Patients who have vulvar pruritus and typical localized syringomas on the face should be searched for vulvar involvement. References

Figure 2 Tumoral lesion containing ductal structures formed by

epithelial cells, some of which are clear cytoplasm embedded in fibrotic stroma (H&E, ×100)

reported in the literature.8 Also, in our case the pruritic lesions on the vulva were localized bilaterally. In the differential diagnosis of vulvar syringoma Fox-Fordyce disease, epidermal cysts, senile angiomas, lichen simplex chronicus, and condyloma acuminata should be considered.9 Histopathologic examination include normal epidermis, and dilated cystic sweat ducts, the walls of which are lined usually by two rows of epithelial cells embedded in a fibrous stroma in the dermis.7 Treatment of syringoma is usually not necessary unless there are cosmetic issues. The treatment modalities for vulvar syringomas include excision, electro-desiccation, laser, and cryotherapy.9,10 Since our patient had intense pruritus, mild potent corticosteroid

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1 Di Lernia V, Bisighini G. Localised vulval syringomas. Pediatr Dermatol 1996; 13: 801. 2 Gerdsen R, Wenzel J, Uerlich M, et al. Periodic genital pruritus caused by syringoma of the vulva. Acta Obstet Gynecol Scand 2002; 81: 369–370 . 3 Brown SM, Freeman RG. Syringoma limited to the vulva. Arch Dermatol 1971; 104: 331. 4 Baden HP. Hereditary syringoma (letter). Arch Dermatol 1977; 113: 1133. 5 Urban CD, Cannon JR, Cole RD. Eruptive syringomas in Down’s syndrome. Arch Dermatol 1981; 117: 374–375. 6 Carneiro SJC, Gardner HL, Knox JM. Syringomas. Three cases with vulvar involvement. Obstet Gynecol 1972; 39: 95–99. 7 Turan C, Ugur M, Kutluay L, et al. Vulvar syringoma exacerbated during pregnancy. Eur J obstet Gynecol reprpd Biol 1996; 64: 141–142. 8 Blasdale C, McLelland J. Solitary giant vulval syringoma. Br J Dermatol 1999; 141: 374–375. 9 Isaacson D, Turner ML. Localized vulvar syringomas. J Am Acad Dermatol 1979; 1: 352–356. 10 Karam P, Benedetto AV. Syringomas: new approach to an old technique. Int J Dermatol 1996; 35: 219–220.

© 2008 The International Society of Dermatology