presentation and is preferably confirmed with histologic features of a 3-mm punch biopsy. 3 Several other disorders that present with vulvar pruritus and/or similar presentations need to be considered when diagnosing this condition. [clinicaladvisor.com]

We describe an unusual presentation of lichen sclerosus in a postmenarchal, virginal girl. [ncbi.nlm.nih.gov]

• Easy Bruising

  Symptoms may include: Smooth discolored skin patches Blotchy, wrinkled skin patches Itching Soreness or a burning feeling Easy bruising Fragile skin Changes in the tube for urine flow (urethra) Bleeding, blistering or open sores Painful sex When to see [mayoclinic.org]

• Asymptomatic

  For half of the women, it was asymptomatic; the other half suffered from vulvar pruritus or a burning sensation. [academic.oup.com]

  OLS is rare and typically presents as asymptomatic, white, plaque-like lesions. Malignant transformation of preexisting OLS has not been reported. [ncbi.nlm.nih.gov]

• Anemia

  Thyroid dysfunction (12–16%); inflammatory bowel diseases, pernicious anemia, alopecia areata, rheumatoid arthritis, morphea, and scleroderma have been associated with LS in women, whereas in men, conditions such as diabetes mellitus and atopic dermatitis [unboundmedicine.com]

  […] autoimmune-related diseases, 21% had a family history of autoimmune disease, and 42% had autoimmune antibodies. 4 The most common autoimmune diseases associated with lichen sclerosus are autoimmune thyroiditis, alopecia areata, vitiligo, and pernicious anemia [clinicaladvisor.com]

  Support for the latter theory stems from the frequent association of other autoimmune disorders such as alopecia, vitiligo, thyrotoxicosis, hypothyroidism and pernicious anemia in patients with lichen sclerosus. [cancertherapyadvisor.com]

  In the present study, none of our biopsies showed any features of dysplasia or koilocytosis due to frequent association with autoimmune diseases such as alopecia, vitiligo, thyroid, and pernicious anemia; screening tests comprising blood tests for iron [ijdpdd.com]

• Ecchymosis

  Asymptomatic patients Treatment is recommended if patients have features of active disease - eg, ecchymosis, hyperkeratosis or progressive atrophy. [patient.info]

  The typical lesions of LS are white plaques and papules, often with areas of ecchymosis, excoriation, and ulceration. [clinicaltrials.gov]

  Typical lesions of lichen sclerosus are porcelain-white papules and plaques, often seen in conjunction with areas of ecchymosis or purpura. The skin typically appears whitened, thinned and crinkling ('cigarette paper' in appearance). [cancertherapyadvisor.com]

  White hyperkeratosis, atrophic skin, lacerations and ecchymosis typically occur later in the course of disease. [ncbi.nlm.nih.gov]

  These lesions are prone to koebnerization and may express themselves in areas of physical trauma, continuous pressure, and scarring. [5], [8] In genital region, the main features are whitish papules which converge into plaques, often with erythema, ecchymosis [ijdpdd.com]

• Fever

  Relapsing fever borreliosis caused by B hermsii, B turicatae, and B duttonii and EM caused by B spielmanii have been described. 39, 40 The study by van Dam et al 41 suggests that different B burgdorferi genotypes have different pathogenic potentials. [doi.org]

• Abdominal Pain

  However, the use of these agents is limited by significant and potentially harmful side effects including cheilitis, xerosis, teratogenicity, elevated liver enzymes, hypertriglyceridemia, abdominal pain, and alopecia. [ncbi.nlm.nih.gov]

• Pruritus

  Intense and recalcitrant pruritus is a symptomatic hallmark of LS. [liebertpub.com]

  Micheline Moyal-Barracco, Pruritus Vulvae, Pruritus, 10.1007/978-1-84882-322-8_37, (243-252), (2009). Paul D. [doi.org]

  Biopsy of suspected vulvar lichen sclerosus in adults is preferred during the initial visit rather than after empiric therapy because of: the risk for atypia or malignancy; the expansive list of possible differentials associated with vulvar pruritus and [clinicaladvisor.com]

  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]. [ncbi.nlm.nih.gov]

  When properly administered, topical ultra-potent corticosteroids help to resolve the symptoms of pruritus and burning and can prevent further vulvar scarring. [clinicaltrials.gov]

• Blister

  The earliest signs are skin fragility, bruising, and sometimes blistering. Lesions typically cause mild to severe itching. When lichen sclerosus manifests in children, the appearance may be confused with sexual abuse. [msdmanuals.com]

  Some of these lesions evolved with hemorrhagic blisters, which after histopathological examination confirmed the diagnosis of bullous and hemorrhagic lichen sclerosus. [ncbi.nlm.nih.gov]

  Blisters and bleeding may also occur. Lichen sclerosus starts out as small white spots on the skin. These spots are generally shiny and smooth. The spots then grow into larger patches that are thin and crinkled. [web.archive.org]

• Alopecia

  A wide variety of dermatologic associations have been described with CD, including alopecia, dermatitis herpetiformis, and enamel hypoplasia. [ncbi.nlm.nih.gov]

  The exact cause of lichen sclerosus is not known, but it is associated with several autoimmune disorders, including alopecia areata and vitiligo. [cmaj.ca]

  In light of the association between autoimmune disorders and lichen sclerosus, a brief examination for alopecia areata and vitiligo is reasonable. [cancertherapyadvisor.com]

  Thyroid dysfunction (12–16%); inflammatory bowel diseases, pernicious anemia, alopecia areata, rheumatoid arthritis, morphea, and scleroderma have been associated with LS in women, whereas in men, conditions such as diabetes mellitus and atopic dermatitis [unboundmedicine.com]

  […] researchers found that 21.5% had 1 or more autoimmune-related diseases, 21% had a family history of autoimmune disease, and 42% had autoimmune antibodies. 4 The most common autoimmune diseases associated with lichen sclerosus are autoimmune thyroiditis, alopecia [clinicaladvisor.com]

• Papule

  Clinically and histologically, the papules were consistent with cherry angiomas. [ncbi.nlm.nih.gov]

• Localized Scleroderma

  En coup de sabre lesion Lichen sclerosus et atrophicus Linear scleroderma Localized morphea (disorder) Localized scleroderma Morphea Sclerodactyly Scleroderma, linear Scleroderma, localized Clinical Information A chronic disorder, possibly autoimmune [icd9data.com]

  Etiology of lichen sclerosus (LiS) and localized scleroderma (LoS) is uncertain and probably multifactorial. We describe a case of female monozygotic twins who presented co-existence of LiS and LoS. [ncbi.nlm.nih.gov]

  A differential diagnosis should include, but is not limited to: localized scleroderma, cutaneous discoid lupus erythematous, atrophic lichen planus, lichen simplex chronicus, vitiligo, mucous membrane pemphigoid, anal fissures and hemorrhoids, candidiasis [clinicaladvisor.com]

  Orteu, Morphoea (Localized Scleroderma), Rook's Textbook of Dermatology, Ninth Edition, (1-37), (2016). Fiona Lewis, Dermatoses of the Female Genitalia, Rook's Textbook of Dermatology, Ninth Edition, (1-52), (2016). Jennifer L. Bercaw-Pratt, Lori A. [doi.org]

• Confusion

  LICHEN SCLEROSUS ET ATROPHICUS The paper by Montgomery and Hill 1 was a great help in clearing the confusion which existed in the terminology applied to lichen sclerosus et atrophicus. [doi.org]

  Lichen sclerosus et atrophicus and limited systemic scleroderma (acrosclerosis) are inflammatory skin diseases that ultimately evolve into two distinct modes of atrophic scar formation, but which can easily be confused clinically. [ncbi.nlm.nih.gov]

  When lichen sclerosus manifests in children, the appearance may be confused with sexual abuse. With time, the involved tissue becomes atrophic, thinned, hypopigmented (there may be flecks of postinflammatory hyperpigmentation), fissured, and scaly. [msdmanuals.com]

  Some researchers think that lichen sclerosus may be an auto-immune disorder, where your immune system becomes confused and attacks your skin instead of protecting it. [thewomens.org.au]

• Dyspareunia

  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]. [ncbi.nlm.nih.gov]

  The atrophy, strictures, and scarring of the affected genitalia secondary to vulvar lichen sclerosus often result in sexual dysfunction, specifically dyspareunia. 7 These anatomical changes may also lead to decreased lubrication and decreased sensation [clinicaladvisor.com]

  Despite adequate treatment, women with lichen sclerosus continue to experience significant sexual dysfunction (i.e., dyspareunia, decreased orgasm and decreased coital frequency). [cancertherapyadvisor.com]

• Sexual Dysfunction

  […] were treated appropriately, sexual dysfunction improved but did not completely resolve. 6,7 Malignancy. [clinicaladvisor.com]

  "The treatment of vulvar lichen sclerosus and female sexual dysfunction". J Sex Med. vol. 8. 2011. pp. 219-22. (Little is known about the impact of medical therapy and sexual function among women with lichen sclerosus. [cancertherapyadvisor.com]

  Forty-three women (16%) reported sexual dysfunction and 13 (5%) had needed at least one surgical procedure to restore sexual function. Eighty-six (32%) had undergone at least one biopsy. [ncbi.nlm.nih.gov]

  Monitoring for squamous cell carcinoma and sexual dysfunction and providing psychologic support are indicated. Lichen sclerosus can cause anogenital bruising, itching, or blistering early, and atrophy and scarring later. [msdmanuals.com]

• Genital Lesions

  We describe a patient with skin, oral and genital lesions of lichen sclerosus. [ncbi.nlm.nih.gov]

  Genital lesions were commonly seen on the vulva and prepuce [Figure 2]. [ijdpdd.com]

  Alberto Rosenblatt, Homero Gustavo de Campos Guidi and Walter Belda, Premalignant Lesions of the Penis, Male Genital Lesions, 10.1007/978-3-642-29017-6_16, (375-401), (2012). [doi.org]

  Genital lesions in women. In: Taylor and Kelly's Dermatology for Skin of Color. 2nd ed. McGraw Hill; 2016. https://accessmedicine.mhmedical.com. Accessed July 25, 2022. Cooper SM, et al. [mayoclinic.org]

• Dysuria

  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]. [ncbi.nlm.nih.gov]

  Pruritus, chronic discomfort, dysuria, and pain with defecation were common chief complaints in our patients. [doi.org]

  If there is meatal scarring, poor urinary stream or dysuria. Signs: White patches on the glans or prepuce. Haemorrhagic vesicles or purpura. Rarely, blisters or ulcers. [patient.info]

• Pruritus Vulvae

  Micheline Moyal-Barracco, Pruritus Vulvae, Pruritus, 10.1007/978-1-84882-322-8_37, (243-252), (2009). Paul D. [doi.org]

  Lichen sclerosus can be extremely itchy and sore ( pruritus vulvae ). Sometimes bruises, blood blisters and ulcers appear after scratching, or from minimal friction (eg tight clothing, sitting down). Urine can sting and irritate. [dermnetnz.org]

On further workup, there was no evidence of disseminated disease involving other organs. [ncbi.nlm.nih.gov]

• Chlamydia

  Chlamydia trachomatis infection in women with lichen sclerosus vulvae and vulvar cancer. Neuro Endocrinol Lett. 2009. 30(5):671-4. [Medline]. Borghi A, Corazza M, Minghetti S, Bianchini E, Virgili A. [emedicine.medscape.com]

No significant effect of treatment duration on time to relapse was found. [doi.org]

This study will consist of a two-week screening period and a 12-week treatment period. [clinicaltrials.gov]

Prognosis Prognosis is good for more acute genital cases of lichen sclerosus (LS), especially for those in the pediatric age group, in whom it may resolve spontaneously. [emedicine.medscape.com]

Etiology and Pathophysiology The etiology of LS is unknown. Some studies have shown a link between autoimmune diseases and LS in women, whereas this association in men has not been analyzed to the same extent. [unboundmedicine.com]

Lichen sclerosus et atrophicus is a chronic disease of unknown etiology characterized by atrophic and sclerotic plaques in both genital and extragenital regions. [ncbi.nlm.nih.gov]

Meijer, Penile cancer: epidemiology, pathogenesis and prevention, World Journal of Urology, 10.1007/s00345-008-0302-z, 27, 2, (141-150), (2008). [doi.org]

Epidemiology Incidence LS is a rare entity. Occurs at all ages and in both genders, but a higher incidence is present in postmenopausal women. The male-to-female ratio varies between 1:3 and 1:10. [unboundmedicine.com]

Material and methods We report 20 patients undergoing circumcision due to histologically confirmed lichen sclerosus, in whom we analysed demographic, clinical and analytical characteristics to complete an epidemiological profile of patients with this [elsevier.es]

A review of this case and the literature describes the clinical features, pathophysiology, and treatment options for this extragenital bullous variant in an effort to raise awareness of this rare clinical presentation. [ncbi.nlm.nih.gov]

Etiology and Pathophysiology The etiology of LS is unknown. Some studies have shown a link between autoimmune diseases and LS in women, whereas this association in men has not been analyzed to the same extent. [unboundmedicine.com]

Spiess, Understanding the Pathophysiology of Penile Cancer and Its Preneoplastic Lesions, Penile Cancer, 10.1007/978-1-4939-6679-0_1, (1-11), (2016). [doi.org]

Complications include pain, sexual and/or urinary dysfunction, reduced quality of life, and an increased risk of squamous cell carcinoma ( Pathophysiology Inflammation and altered fibroblast function in the papillary dermis lead to fibrosis of the upper [emedicine.medscape.com]

General Prevention No clear preventive methods are reported in the current guideline; however, early aggressive treatment may prevent disease progression. Commonly Associated Conditions 20–30% of women with LS also have autoimmune diseases. [unboundmedicine.com]

The only predictor we have identified is a lack of consistent preventive treatment. [doi.org]

While treatment can prevent further changes in genital tissue, any scarring that has already occurred is not reversible without surgery. [drexelmedicine.org]

Every other month or greater, injection intervals were required to prevent relapse in all patients. [liebertpub.com]

Strong topical steroid medication is prescribed to control inflammation, help smooth out the roughened skin, and prevent or halt scarring. However it cannot reverse loss of skin architecture. [mshc.org.au]