Vulvar pruritus is a common complaint among young girls and women presenting to primary care physicians, gynecologists, and dermatologists. Female genital itch is especially disruptive because of its interference with sexual function and intimacy. Causes of vulvar itch are vast and may be inflammatory, environmental, neoplastic, or infectious, often with several causes coexisting simultaneously. Diagnosis may be difficult because of the unique anatomy and inherent properties of genital and perianal skin. Treatment is aimed at eliminating outside irritants, restoring epidermal barrier function, and suppressing inflammation.

Lichen sclerosus (LS) is a chronic inflammatory skin disease of unknown origin predominantly affecting the anogenital area that causes pruritus and pain and is associated with an increased risk of malignancy. In some cases, LS vanishes after application of imiquimod, raising the question whether human papillomavirus (HPV) may have an etiopathogenic role in anogenital LS. The databases MEDLINE and Embase were systematically searched using the PRISMA guidelines. Twenty-seven papers were included that reported the prevalence of HPV in LS and in LS associated with neoplasia. HPV was identified in 0-80% (median 22%) of all LS cases. The prevalence of HPV was higher among male patients with LS (median 29%) than among female patients (median 8%). HPV16 was the most prevalent genotype, but the distribution of genotypes indicates that even low-risk HPV can cause LS. The diverging detection rates are probably due to small sample sizes in the reviewed papers and different detection methods. Factors possibly underestimating the prevalence of HPV are a selective search for high-risk HPV, DNA destruction in fixed tissue, focally residing HPV, and possibly a clearing of HPV before the time of biopsy. Seventy-five percent of sexually active people acquire HPV during their lifetime, thus HPV alone is not a cause of LS. Genetic and immunological host factors and viral factors other than type are likely to contribute. Future studies should include patients with a short duration of symptoms, and biopsies should be multiple and fresh.

Lichen sclerosus (LS) is a benign, chronic, inflammatory skin disease with a predilection for the anogenital region in women. Although males can also be affected, the ratio of female to male incidence has been reported to be as high as 6-10:1 and possesses a bimodal age distribution of pre-pubertal girls and postmenopausal women [1, 2]. Affected skin usually demonstrates polygonal papules that coalesce into porcelain white plaques and can be associated with edema, telangiectasias, and comedo-like plug formation [3]. Lichen sclerosus can be debilitating for some patients causing significant pruritus, pain, dysuria, and dyspareunia [4]. Rarely, lichen sclerosus appears in various extragenital areas, although most cases are relatively asymptomatic [3]. Even more uncommonly, as displayed in this case report of a 69-year-old woman, LS can present extragenitally with a bullous or hemorrhagic appearance [5].

Itch management can be particularly complicated in some small areas like the scalp or the anogenital region for many reasons: the frequently poor diagnosis of the causes of itch in these areas, the dense innervation of these areas, and the symbolic value of these areas for the human psyche. The diagnosis of itchy scalp is easier than that of anogenital pruritus. Clinical examination and a careful inventory of all diseases of the patient and of the local environment are necessary. Localized treatments are frequently used at both sites, whereas specific pharmaceutical formulations are necessary for the pilose or the mucous environment. Nonetheless, systemic treatments or psychological interventions can be very useful.

Anogenital pruritus is defined as intense itching, acute or chronic, affecting the anal, perianal, perineal and genital skin, which is a dominant problem in the course of various cutaneous and systemic conditions. It is one of the common, extremely annoying symptom for which patients attend the Dermatology Outpatient Department (OPD). Anogenital skin is highly sensitive to soaps, perfumes, clothing and superficial trauma and it is more prone for itchy dermatoses as a result of warmth, friction, lack of aeration, sweating and occlusive inner garments. Anogenital pruritus is associated with a wide spectrum of diseases which includes localized infections, infestations, inflammatory dermatoses, allergic and irritant conditions, anorectal diseases, systemic causes, nutritional disorders, psychological and when the cause cannot be found out it is often termed idiopathic. Patients are highly reluctant in consulting the physician for anogenital itch in the early stage, they usually present at a later stage with either atypical manifestations or depigmentation and lichenification, secondary to constant scratching. They often resort to over the counter topical agents, particularly combination products which contain topical steroids. The irrational use of such products results in complications like skin atrophy, striae, incognito etc. A proper clinical history, clinical examination, investigations like scrapping for fungus and itch mite, skin biopsy, patch test and relevant blood investigations to rule out systemic conditions should be carried out, when needed, to arrive at an accurate diagnosis, before treating the patient.

Extramammary Paget's disease is a marginated plaque resembling Paget's disease but occurring in anogenital area, axilla or most commonly on the vulva. A 62-year-old postmenopausal woman presented with extremely pruritic plaque on the perineal skin which progressed gradually over 3 years and did not respond to any topical/systemic steroids, antibiotics, and antifungals. Examination revealed 7 × 8 cm. hypertrophic, verrucous plaque with erosions, and crusts at places. There was no evidence of visceral malignancy. Biopsy showed Paget's cells which were positive for Periodic Acid Schiff and alcian blue stain. Surgical excision was done considering the premalignant potential.

Dermatological complications in Turner syndrome are infrequent but occasionally cause significant morbidity. Lichen sclerosus (LS) is a chronic inflammatory mucocutaneous affection characterized by pruritus in the anogenital area. It is yet not clear its pathophysiology but it's linked with genetic factors and autoimmunity. This is a case report of a girl with Turner syndrome with growth hormone treatment that started with vulvar pruritus and was diagnosed as lichen sclerosus.