Cases reported "Vulvar Diseases"

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1/47. Labial fusion in children: a presenting feature of genital lichen sclerosus?

    Labial fusion is a common condition seen most frequently in infants and young children. While most cases are "physiological," we believe it can occasionally be the presenting feature of genital lichen sclerosus.
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ranking = 1
keywords = lichen sclerosus, sclerosus, lichen
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2/47. Genital lichen sclerosus associated with morphoea or systemic sclerosis: clinical and HLA characteristics.

    Although patients with both morphoea and lichen sclerosus have been reported previously, in the majority of these reports the lichen sclerosus has been extragenital. We report nine patients in whom genital lichen sclerosus coexisted with scleroderma spectrum disorders including seven with morphoea, one with morphoea and lichen planus, and one with systemic sclerosis. The clinical features, associated autoimmune disease, autoantibodies and HLA type are reported. Antibodies to borrelia burgdorferi were not detected in any of the patients. The coexistence of these diseases raises a number of intriguing questions about the relationship between them.
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ranking = 1.402807427738
keywords = lichen sclerosus, sclerosus, lichen
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3/47. Papular acantholytic dyskeratosis of the vulva.

    We describe two patients with unusual asymptomatic, papular lesions on the vulva, clinically resembling lichen planus, the histology of which revealed unexpected findings of suprabasilar clefting, acantholysis and dyskeratotic cells giving rise to corps ronds and grains together with hyperkeratosis and parakeratosis, features originally associated with a diagnosis of Darier's disease. Focal acantholytic dyskeratosis has been described in a wide variety of inflammatory and neoplastic processes including those involving mucous membranes and has been attributed various diagnostic labels. We feel that the findings in our patients are consistent with a diagnosis of papular acantholytic dyskeratosis of the vulva, a rare condition, which was first described in 1984.
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ranking = 0.0028074277380394
keywords = lichen
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4/47. Nail dystrophy due to lichen sclerosus?

    Lichen sclerosus (LS) affects anogenital skin alone in 80% of cases. When extragenital disease occurs, it usually affects the trunk, neck, axillae and wrist flexures. Nail involvement with LS is rare. In contrast, lichen planus (LP) commonly affects extragenital skin. Mucosal lesions occur in 50% of cases, affecting the mouth and genitalia. Nail disease in LP is common, and, if severe, can lead to destruction of the nail bed. LS and LP can coexist. We report two cases of LS with nail involvement. In the Case 1 disease was confined to the nail, and nail biopsy confirmed LS. In the Case 2, the nail changes formed part of the widespread genital and extragenital LS, confirmed histologically. We review existing literature on nail disease in LS and discuss the possible aetiology of the nail changes.
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ranking = 0.85020394856834
keywords = lichen sclerosus, sclerosus, lichen
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5/47. Pseudoepitheliomatous hyperplasia in lichen sclerosus of the vulva.

    Small tentacles or separated nests of squamous cells in the dermis are not uncommonly seen in long-standing vulvar lichen sclerosus (LS) associated with epidermal thickening. We recently encountered a case where separated nests of well-differentiated squamous cells in the dermis were difficult to distinguish from squamous cell carcinoma (SCC). Further biopsies showed similar nests originating from every hair follicle. We postulated a diagnosis of multifocal pseudoepitheliomatous hyperplasia (PEH) to explain this phenomenon. Because we could find no reference to PEH in the setting of LS, we reviewed the biopsies of 92 women with extragenital and vulvar LS with and without carcinoma to determine its frequency and histological appearance. The study population, which excluded the index case, comprised 10 women with extra-anogenital LS, 58 with vulvar LS without carcinoma, and 24 with vulvar LS with carcinoma. The presence of PEH, epidermal thickness, predominant dermal collagen change, degree of inflammation, and presence of fibrin and red blood cells were recorded. The presence or absence of lichen simplex chronicus (LSC), squamous cell hyperplasia (SCH), and differentiated vulvar intraepithelial neoplasia (VIN) were recorded. PEH was identified only in vulvar LS, where it was seen in 7/58 (12.1%) women without carcinoma, 1/24 (8.3%) with carcinoma, and 0/10 (0%) with extra-anogenital LS. Two forms of PEH were seen: predominantly epidermal 7/8 (87.5%) and predominantly follicular 1/8 (12.5%). PEH was associated with increased epidermal thickness, less dermal edema, more dermal inflammation, fresh fibrin, and red blood cell extravasation. In all cases, there was associated LSC, but there was no SCH or differentiated VIN. In conclusion, PEH may explain many of the cases of dermal tentacles and separated squamous nests in vulvar LS with LSC. The association with fresh fibrin and red blood cells suggests that PEH might be a reaction to tissue damage. PEH is distinguished from SCC by its lack of atypia, confinement to the abnormal collagen, and limited growth. The pathologist must be careful about making a diagnosis of PEH in LS with epidermal thickening, looking carefully for basal atypia and other features of differentiated VIN in the overlying epidermis or dermal proliferation. We do not know whether PEH occurs in differentiated VIN and, if it does, how it could be distinguished from SCC.
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ranking = 2.754211428499
keywords = vulvar lichen sclerosus, vulvar lichen, lichen sclerosus, sclerosus, lichen
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6/47. Vulvar lichen planus associated with ulcerative colitis. A case report.

    BACKGROUND: lichen planus (LP) is a common inflammatory dermatosis the diagnosis of which can be very complex when it affects the female genital area exclusively. The condition should be managed by both dermatologists and gynecologists because the association between vulvar LP and other immune-mediated disorders occurs frequently. CASE: Vulvar LP was diagnosed in 37-year-old woman whose past history was significant for ulcerative colitis (UC) since the age of 21. The oral mucosa and skin were completely unaffected, but whitish papules and plaques were seen diffusely on the whole vulvar region, which had become sclerotic and edematous. Microscopic examination of lesional tissue confirmed the diagnosis, showing classic histologic features in spite of the mixed and hypertrophic clinical variant. Oral therapy with cyclosporin at an initial dose of 250 mg/d led to complete remission within 3 months. CONCLUSION: This case is reported on for the rarity of isolated vulvar involvement by LP and for the excellent response to treatment.
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ranking = 0.011229710952158
keywords = lichen
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7/47. Erosive lichen planus of the vulva and vagina.

    BACKGROUND: Erosive lichen planus causes erosion of the vulva and vagina and characteristic oral lesions. dyspareunia is usual, and vaginal stenosis may occur. This report highlights the clinical features and the response to medical therapy. CASES: We report the case histories of three women who presented to the Vulvovaginal Disorders Clinic of the University of iowa with long histories of dyspareunia and advanced vaginal scarring. In each case, the clinical diagnosis of erosive lichen planus was obvious but had not been made previously. All three women have responded well to topical treatment with tacrolimus 0.1% ointment. CONCLUSION: Erosive lichen planus should be suspected in a case of vaginal erosion or narrowing. Surgical management is inappropriate when the mucosa is eroded. Inspection of the mouth may confirm the diagnosis.
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ranking = 0.019651994166276
keywords = lichen
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8/47. tacrolimus ointment for the treatment of vulvar lichen sclerosus.

    The treatment of vulvar lichen sclerosus is generally considered difficult. Ultrapotent corticosteroids represent the most effective topical treatment, but carry the risk of side effects such as skin atrophy. We describe a 71-year-old woman with long-standing vulvar lichen sclerosus refractory to conventional treatment. After 6 consecutive weeks of treatment with tacrolimus ointment 0.1% (Protopic) twice daily, signs and symptoms of lichen sclerosus resolved. To our knowledge, this is the first report of the use of topical tacrolimus, which does not induce skin atrophy, in the treatment of vulvar lichen sclerosus.
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ranking = 13.859828005327
keywords = vulvar lichen sclerosus, vulvar lichen, lichen sclerosus, sclerosus, lichen
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9/47. vulvodynia ... the problem exists here!

    A case of vulvodynia is being reported here. This is a frustrating disorder affecting young women. A 36 years old lady initially being treated non-specifically for vulval pain, dysparunia and vaginal discharge, for several months was investigated in the gynaecology unit of Combined Military Hospital, Rawalpindi. Examination was possible only under anaesthesia and biopsy report confirmed Erosive lichen planus. After dermatological consultation she responded well to steroids. She was counselled about the risk of recurrence and small risk of malignancy.
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ranking = 0.0028074277380394
keywords = lichen
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10/47. Pimecrolimus for the treatment of vulvar lichen sclerosus in a premenarchal girl.

    BACKGROUND: Lichen sclerosus is a chronic cutaneous disorder with a predilection for the vulva. Lichen sclerosus affects more than one in 900 girls. Superpotent corticosteroids like clobetasol propionate are the most effective treatment for vulvar lichen sclerosus. However, recurrence after stopping steroids is very high. As repeated courses of corticosteroids are frequently needed, there are concerns about potential side effects. Therefore, a treatment regimen that does not rely on corticosteroids may be beneficial. As lichen sclerosus is a T-lymphocyte mediated disorder, it has been suggested that pimecrolimus, a topical T-lymphocyte inhibitor, may be safe and effective for the treatment of lichen sclerosus in children. CASE REPORT: A 10-year-old girl with lichen sclerosus was initially treated with clobetasol. Remission was achieved, but 3 months later she had a recurrence. Subsequent treatment with clobetasol led to a breakdown of her peri-anal skin with a superimposed infection. She was then treated with pimecrolimus and remission was achieved. She has had no recurrence of active lichen sclerosus and has less burning with pimecrolimus than with clobetasol. CONCLUSION: Pimecrolimus may be an effective treatment of vulvar lichen sclerosus. Pimecrolimus has been shown to be very safe in the pediatric population for the treatment of mild to moderate eczema, without causing dermal atrophy, tachyphylaxis, striae, rebound flares, or hypothalamic-pituitary axis suppression. As the recurrence rate of active lichen sclerosus in prepubertal girls treated with topical corticosteroids is high, and the majority of prepubertal girls with lichen sclerosus continue to have disease after menarche, a treatment regimen that does not rely on corticosteroids may be beneficial.
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ranking = 13.003217046226
keywords = vulvar lichen sclerosus, vulvar lichen, lichen sclerosus, sclerosus, lichen
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