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Gynaecology Case Reports Meffert JJ, Davis BM, Grimwood RE. 1995. Lichen sclerosus. Journal of the American Academy of Dermatology 32:393–416. Oyama N, Chan I, Neill SM et al. 2003. Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet 362:118–123.
Simple vulval trichilemmal cyst E. N. Tola1, B. Elezoglu2 & F. D. Dellal3 Departments of 1Obstetrics and Gynecology, 2Pathology and 3Endocrinology and Metabolism, Agri Government Hospital, Agri, Turkey DOI: 10.3109/01443615.2012.755502 Correspondence: F. D. Dellal, Department of Endocrinology and Metabolism, Agri Government Hospital, Agri, Turkey. E-mail:
[email protected]
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Introduction
Figure 1. Vulval lichen sclerosus with uterine prolapse, demonstrating classical changes of lichen sclerosus affecting the exposed surface.
Trichilemmal cysts (TCs) are uncommon benign cutaneous lesions that originate from follicular outer root sheath epithelium. They are localised in the dermis or subcutaneous tissue. Two pathological subtypes have been defined: proliferating trichilemmal cysts (PTCs) and simple TC. Both of these generally occur on the scalp of older women. Other locations are forehead, chest, elbow, back, ankle, abdomen, and rarely, vulva. Moreover, a vulval localisation for PTCs is extremely rare. Simple TCs are benign lesions; however, PTCs can occasionally prove to be locally aggressive, with malignant transformation rarely defined. Therefore, clinical and pathological discrimination of simple TCs and PTC is essential. Here, we report on a case of simple vulval TC with atypical localisation and unusual age of the presentation.
Case report Lichen sclerosus may have clinical overlap with lichen planus. The presence of oral LP is not enough to confirm vulval lichen planus, as vulval lichen sclerosus may be associated with oral lichen planus (Marren et al. 1994). Additionally, in areas of lichenoid change, it can be difficult to distinguish these entities histologically. The presence of vaginal involvement is a diagnostic discriminator and indicator of lichen planus. There is only one case report of lichen sclerosus affecting the vagina (Longinotti et al. 2005) and it is possible that this may have been due to lichen sclerosus/lichen planus overlap. However, the development of lichen sclerosus on vaginal tissue seen in our case, is likely to result from the keratinisation of the prolapsed epithelium, that is not seen when the normal anatomy is preserved. The affected skin demonstrates pathognomic ecchymosis and the non-prolapsed mucosa remains unaffected. The clinical presentation of lichen sclerosus may be modified by environmental factors, and tissue metaplasia in response to trauma may allow its development at sites where it is not characteristic. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Bump RC, Mattiasson A, Bo K et al. 1996. The standardisation of terminology of female pelvic organ prolapse and pelvic floor dysfunction. American Journal of Obstetrics and Gynecology 175:10–17. Haylen BT, de Ridder D, Freeman RM et al. 2010. An International Urogynecological Association (IUGA)/International Continence Society (ICS) joint report on the terminology for female pelvic floor dysfunction. Neurourology and Urodynamics 29:4–20. Longinotti M, Schieffer YM, Kaufman RH. 2005. Lichen sclerosus involving the vagina. Obstetrics and Gynecology 106:1217–1219. Marren P, Millard P, Chia Y et al. 1994. Mucosal lichen sclerosus/lichen planus overlap syndromes. British Journal of Dermatology 131:118–123. Marren P, Yell J, Charnock FM et al. 1995. The association between lichen sclerosus and the antigens of the HLA system. British Journal of Dermatology 132:197–203.
A 24-year-old woman (G2P2) presented with a vulval mass on the left major labium. She reported the mass to be present for about 6 years and was slowly increasing in size. Her general and obstetric history was otherwise unremarkable. On physical examination, a firm, mobile, pedunculated, but non-ulcerated mass measuring 4 ⫻ 4 ⫻ 5 cm covered by normal overlying vulval skin, was observed. Inguinal lymph nodes were not palpable. The initial differential diagnosis included a vulval lipoma. The patient consented on the surgical removal, and subsequently, the mass was completely excised under general anaesthesia. The resected specimen was fixed in 10% formalin for histopathological examination. Microscopic findings The wall of the cyst had squamous cells, lying on the thick basal membrane; the granular layer was lacking. The central parts of the cyst material included amorphous keratinous material. No atypical nuclei or pleomorphism was observed in the epithelial cells (Figure 1).
Discussion Trichilemmal cysts are uncommon skin lesions, originating from follicular outer root sheath epithelium. An autosomal dominant inheritance has been defined (Naki et al. 2009). Although simple TCs are always benign lesions, PTCs are generally benign, but sometimes can be locally aggressive with malignant transformation and metastases (Folpe et al. 2003). Simple TCs are probably the precursor lesions of PTCs. Therefore, PTCs are believed to originate from ordinary simple TCs following incidents such as minor trauma, subsequent inflammation and chemical irritation from the cyst content (Avinoach et al. 1989). Trichilemmal cysts usually grow slowly. Interval to presentation was reported to range between 4 and 50 years (Sau et al. 1995). In our case, this period was 6 years. Approximately 90% of trichilemmal cysts occur on the scalp, especially on the occipital area, where hair follicles are abundant. The remaining 10% occur on other sites of the body such as the back, forehead, chest, abdominal skin, nose, ankle, elbow and buttocks (Brownstein and Arluk 1981; Dabska 1971; Falleti et al. 2009). It is logical that the scalp would be, by far, the most common localisation, possibly secondary to traumatic and/or inflammatory
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Figure 1. (A) The lining is composed of stratified squamous epithelium that lacks granular cell layer and merges imperceptibly with homogenous eosinophilic keratin (H&E, ⫻ 100). (B) Peripheral layers demonstrate a palisading arrangement, whereas cells close to the cyst cavity are swollen and filled with pale cytoplasm. Absence of intercellular bridge between the epithelial cells lining the cyst wall (H&E, ⫻ 400).
aetiology. TCs have rarely been defined to be located on the vulva (Buchler et al. 1978; Falleti et al. 2009; Naki et al. 2009). Therefore, our case describes an extremely unusual location of this lesion. TCs are more frequently seen in women older than 50 years (i.e. 50–80 years). This may imply hormonal influences in the development and perhaps growth of these tumours (Chang et al. 2006). However, our patient was 24 years old, indicating that hormonal milieu might not be the foremost determining factor in the aetiology of TCs. Squamoid islands (foci of squamous differentiation), presence of trichilemmal type of keratinisation, presence of calcified foci, keratinised cells without any granular layer (Fernandez-Figueras et al. 2001), and absence of premalignant epidermal lesion such as actinic keratosis are characteristic for TCs. Moreover, an eosinophilic hyaline membrane surrounding the tumour lobules and prominent glycogen-rich or clear cells in some areas can be encountered both in simple and proliferative TCs. Presence of atypical nuclei, pleomorphism, mitotic activity and dyskeratotic cells distinguishes proliferative from simple TCs. The histopathological findings of our case included trichilemmal-type keratinisation and squamous epithelium that lacks granular cell layer without atypical nuclei or pleomorphism in epithelial cells, in line with the diagnosis of simple TC. In simple TCs, surgical excision is curative; however, long-term follow-up can be necessary to prevent possible recurrences. Closer surveillance may also be necessary following PTC removal, due to its malignant potential. In our case of simple TC, complete surgical excision proved to be an adequate strategy. In conclusion, the differential diagnosis of a vulval mass in a young woman should include TCs. Although simple and proliferating, TCs present with similar clinical characteristics but they can be differentiated from each other by histopathological examination. Therefore, excision of any vulval mass, irrespective of age, may be justified. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Avinoach I, Zirkin HJ, Glezerman M. 1989. Proliferating trichilemmal tumor of the vulva. Case report and review of the literature. International Journal of Gynecological Pathology 8:163–168. Brownstein MH, Arluk DJ. 1981. Proliferating trichilemmal cyst: a simulant of squamous cell carcinoma. Cancer 48:1207–1214. Buchler DA, Sun F, Chuprevich T. 1978. A pilar tumor of the vulva. Gynecologic Oncology 6:479–486. Chang SJ, Sims J, Murtagh FR et al. 2006. Proliferating trichilemmal cysts of the scalp on CT. American Journal of Neuroradiology 27:712–714. Dabska M. 1971. Giant hair matrix tumor. Cancer 28:701–706. Falleti J, Cuccuru A, Mignogna C. 2009. Proliferating trichilemmal cyst of the vulva. Clinical and Experimental Dermatology 34:459–460. Fernandez-Figueras MT, Casalots A, Puig L et al. 2001. Proliferating trichilemmal tumour: p53 immunoreactivity in association with p27Kip1 over-expression indicates a low-grade carcinoma profile. Histopathology 38:454–457. Folpe AL, Reisenauer AK, Mentzel T et al. 2003. Proliferating trichilemmal tumors: clinicopathologic evaluation is a guide to biologic behavior. Journal of Cutaneous Pathology 30:492–498.
Naki MM, Api O, Ergen B et al. 2009. Vulvar Simple Trichilemmal Cyst: Case Report. Turkish Gynecology and Oncology 2:45–48. Sau P, Graham JH, Helwig EB. 1995. Proliferating epithelial cysts. Clinicopathological analysis of 96 cases. Journal of Cutaneous Pathology 22:394–406.
Cervical schistosomiasis: An increasing presentation in the developed world H. Maraj1, K. Chin1 & D. Bhattacharjee2 Department of 1Obstetrics and Gynaecology, Staffordshire General Hospital, Stafford and 2Medical Microbiology, University Hospital of North Staffordshire, Stoke on Trent, UK DOI: 10.3109/01443615.2012.758692 Correspondence: H. Maraj, Department of Obstetrics and Gynaecology, Staffordshire General Hospital, Weston Road, Stafford, ST16 3SA, UK
Introduction Schistosomiasis is a human disease caused by infection from one of the species of parasitic trematodes of the genus Schistosoma. Worldwide, approximately 200 million people are infected mainly in developing countries with over 70% of cases from sub-Saharan Africa. Female genital schistosomiasis affects 9–13 million women worldwide, mainly in areas where S. haematobium is endemic (Carey et al. 2001). The organs involved are cervix, vulva, ovary, clitoris and fallopian tube. Cases in the developed world are rare and mainly confined to the immigrant population and those who travel to endemic areas.
Case report A 30-year-old woman presented with a report of moderate dyskaryosis on her cervical smear. She was born in Zimbabwe and had moved to the UK at age 23. She had a past history of heavy menstrual bleeding. On speculum examination, the cervix appeared normal but acetowhite changes were noted on colposcopy. Cervical punch biopsies showed koilocytosis and cervical intraepithelial neoplasia (CIN) I. She subsequently had a large loop excision of the transformation zone, which confirmed CIN I with focal CIN II. There was also schistosomiasis infestation with a severe active chronic inflammatory infiltrate. She was treated with Praziquantel and her subsequent cervical smear was negative.
Discussion Increasing migrant populations and travel to endemic areas mean that there should be increased vigilance to the possible diagnosis of female genital schistosomiasis. Schistosomes modulate the human immune system and survive in the blood for years (Hoffmann and Dunne 2003). Adult schistosomes produce hundreds of eggs per day. Clinical manifestations of chronic disease result from host immune responses to schistosome eggs.
