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Lichen Sclerosus

Lichen Sclerosis et Atrophicus


Presentation

  • The typical presentation of CD generally includes gastrointestinal symptoms, but more individuals are presenting with extraintestinal manifestations.[ncbi.nlm.nih.gov]
  • 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]
  • Morphea and lichen sclerosus et atrophicus (LSA) have similar clinical presentations.[ncbi.nlm.nih.gov]
  • We describe an unusual presentation of lichen sclerosus in a postmenarchal, virginal girl.[ncbi.nlm.nih.gov]
  • We think that our case might represent a unique variant of interstitial MF presenting with LS-like lesions. The diagnostic challenge arising from this uncommon presentation is discussed together with review of the literature.[ncbi.nlm.nih.gov]
Epilepsy
  • We report a case of Lichen Sclerosus in a 73-year-old man who had been treated for epilepsy with carbamazepine.[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]
  • In this report we describe three girls with CD who presented with hypopigmented skin lesions and pruritus in the perivaginal and perianal areas, consistent with the diagnosis of lichen sclerosus (LS).[ncbi.nlm.nih.gov]
  • 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]
Blister
  • 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]
  • The most common side effects of laser assisted hair removal are pain, erythema, edema, hypopigmentation, hyperpigmentation, blistering, crusting, erosions, purpura, folliculitis, and scar formation ( 1 ).[ncbi.nlm.nih.gov]
  • 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]
  • Skin fragility may lead to breaking of some small blood vessels in the skin which appear as tiny blood blisters. Occasionally, blisters and small cracks called fissures can be seen.[skinsupport.org.uk]
  • Blisters. What causes it? What causes lichen sclerosus? You can’t give lichen sclerosus to someone else. No one knows what exactly causes the disease. Some possible causes include: An overactive immune system. Hormone problems.[niams.nih.gov]
Papule
  • Clinically and histologically, the papules were consistent with cherry angiomas.[ncbi.nlm.nih.gov]
  • Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis, characterized by shiny, atrophic, hypochromic papules with a predilection for the genital and perineal skin.[ncbi.nlm.nih.gov]
  • The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color.[ncbi.nlm.nih.gov]
  • Both forms feature cutaneous involvement typified by erythematous papules with scar-like centers, and the classic histologic picture is described as wedge-shaped dermal necrosis overlying thrombotic vasculopathy.[ncbi.nlm.nih.gov]
  • Physical examination revealed the presence of numerous 2-4 mm, mostly perifollicular, hypopigmented, slightly atrophic papules and plaques. Many of the lesions had a central keratotic plug.[ncbi.nlm.nih.gov]
Koebner Phenomenon
  • It has been reported in post-traumatic areas as a result of Koebner phenomenon. LSA responds poorly to treatment making it a therapeutic challenge (2). Laser therapy has been described for LSA with variable results (3,4).[ncbi.nlm.nih.gov]
  • The Koebner phenomenon, which occurs in damaged skin secondary to inflammation, also produces LS. So skin injury has been suggested as a possible trigger of LS in genetically predisposed people.[f1000research.com]
  • Bilateral Koebner phenomenon in lichen sclerosus et atrophicus. Int J Dermatol 1987; 26: 117-8. Gross P. Generalized lichen sclerosus et atrophicus associated with band-like scleroderma (Koebner phenomenon and lichen sclerosus).[cirp.org]
  • phenomenon.[ 15 ] Oxidative stress may also be responsible for sclerosis, autoimmunity, and carcinogenesis in case of LS.[ 16 ] Cell kinetics An elastase-type enzyme produced by vulvar fibroblasts leads to the destruction of connective tissue in patients[ncbi.nlm.nih.gov]
Hemorrhagic Bullae
  • We present a 63-year-old woman with extensive extragenital, ivory-colored, atrophic plaques on her trunk and extremities and disseminated hemorrhagic bullae.[ncbi.nlm.nih.gov]
  • The most common variation occurs when the inflammation is intense enough to cause separation of a large area of epidermis, creating blisters, or large, occasionally hemorrhagic, bullae.[ncbi.nlm.nih.gov]
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]
  • Common symptoms are irritation, soreness, dyspareunia, dysuria, and urinary or fecal incontinence.[ncbi.nlm.nih.gov]
  • Despite adequate treatment, women with lichen sclerosus continue to experience significant sexual dysfunction (i.e., dyspareunia, decreased orgasm and decreased coital frequency).[cancertherapyadvisor.com]
Genital Lesions
  • We describe a patient with skin, oral and genital lesions of lichen sclerosus.[ncbi.nlm.nih.gov]
  • The patient was referred for dermatologic evaluation, which identified no skin or genital lesions, and no treatment was employed. After 6 years, no significant changes in clinical features were observed.[ncbi.nlm.nih.gov]
  • Genital lesions were commonly seen on the vulva and prepuce [Figure 2] .[ijdpdd.com]
  • Lucio Andreassi and Roberta Bilenchi, Non-infectious inflammatory genital lesions, Clinics in Dermatology, 32, 2, (307), (2014).[doi.org]
  • Objective To observe the clinical effects of short-term application of ultrapotent topical corticosteroid on symptomatic genital lesions of lichen sclerosus in pediatric patients.[doi.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]
  • Common symptoms are irritation, soreness, dyspareunia, dysuria, and urinary or fecal incontinence.[ncbi.nlm.nih.gov]
  • Pain, soreness, and dysuria may be a consequence of erosions or fissures.[doi.org]
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]
  • Surgical treatment of therapy refractory pruritus vulvae in lichen sclerosus. Med Welt 1982; 33: 1323–6 PubMed Google Scholar 187. Paniel BJ, Truc JB, Robichez B, et al. Vulvoperineoplasty [in French].[doi.org]
  • David Nunns, Pruritus vulvae, Current Obstetrics & Gynaecology, 12, 4, (231), (2002). K.J. Rolfe, J.C. Crow, W.M.N. Reid, E. Benjamin, A.B. MacLean and C.W.[doi.org]

Workup

  • On further workup, there was no evidence of disseminated disease involving other organs.[ncbi.nlm.nih.gov]
  • Workup revealed a high-grade stricture extending from the meatus to the site of the previous anastomosis at the penoscrotal junction.[ncbi.nlm.nih.gov]
  • According to Naswa and Marfatia, it cannot replace confirmation of VLS by histopathology, so biopsy is still mandatory in doubtful cases.[ 25 ] An autoimmune workup such as antinuclear antibodies, Vitamin B12 level, thyroid profile, and Borrelia antibodies[ncbi.nlm.nih.gov]

Treatment

  • No significant effect of treatment duration on time to relapse was found.[doi.org]
  • We initiated 0.05% betamethasone ointment for treatment of the lesions.[doi.org]
  • This study will consist of a two-week screening period and a 12-week treatment period.[clinicaltrials.gov]
  • Remission was achieved quickly, after the fourth session, with excellent treatment tolerance. ECP is now recognized as an effective treatment for erosive lichen planus, graft-versus-host disease (GVHD), and scleroderma.[ncbi.nlm.nih.gov]
  • In this case, the treatment was surgical.[ncbi.nlm.nih.gov]

Prognosis

  • Donata Grimm, Katharina Prieske, Sabrina Mathey, Sascha Kuerti, Eike Burandt, Barbara Schmalfeldt and Linn Woelber, Superficially invasive stage IA vulvar squamous cell carcinoma—therapy and prognosis, International Journal of Gynecologic Cancer, 10.1136[doi.org]
  • Early diagnosis and treatment play a substantial role in a patient’s prognosis and result in a decreased risk of malignancy and scarring.[clinicaladvisor.com]
  • 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

  • It has been postulated that there is a common etiologic agent, which may involve autoimmunity, response to trauma, or infection. The link between Borrelia infection and both morphea and LSA has been widely studied but remains controversial.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • 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 (LSA) is an inflammatory disease that affects the genitals, which was first described by Hallopeau in 1887 and is of unknown etiology.[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
  • Meijer, Penile cancer: epidemiology, pathogenesis and prevention, World Journal of Urology, 10.1007/s00345-008-0302-z, 27, 2, (141-150), (2008).[doi.org]
  • Teng, Pediatric Lichen Sclerosus: A Review of the Epidemiology and Treatment Options, Pediatric Dermatology, 32, 5, (593-599), (2015). G. Kirtschig, K. Becker, A. Günthert, D. Jasaitiene, S. Cooper, C.‐C. Chi, A. Kreuter, K.K. Rall, W. Aberer, S.[doi.org]
  • They present evidence‐based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.[doi.org]
  • Kizer WS, Prarie T, Morey AF (2003) Balanitis xerotica obliterans: epidemiologic distribution in an equal access health care system. South Med J 96:9–11 CrossRef PubMed Google Scholar 11.[link.springer.com]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]
  • 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]
  • Accreditation Infection Prevention and Control The Office for Mental Health and Wellbeing Vision and Priority Workshops Frequently Asked Questions LifeSpan - Integrated Suicide Prevention Primary navigation Page last updated on: 7 Jan 2019[health.act.gov.au]
  • The only predictor we have identified is a lack of consistent preventive treatment.[doi.org]

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