Presentation
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]
A 14-year-old girl presented with a 1-year history of a pruritic, bullous lesion on her posterior neck. A biopsy revealed bullous lichen sclerosus. [ncbi.nlm.nih.gov]
Entire Body System
- Shivering
Mallory Shiver, Catherine Papasakelariou, Jameel Ahmad Brown, Marla Wirges and Jay Kincannon, Extragenital Bullous Lichen Sclerosus in a Pediatric Patient: A Case Report and Literature Review, Pediatric Dermatology, 31, 3, (383-385), (2012). A. [doi.org]
- 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]
- Family History of Diabetes Mellitus
A family history of diabetes mellitus, vitiligo, alopecia areata, or thyroid disease and the presence of other autoimmune diseases were identified as risk factors for male LS [ 18 ]. [doi.org]
Skin
- Pruritus
Micheline Moyal-Barracco, Pruritus Vulvae, Pruritus, 10.1007/978-1-84882-322-8_37, (243-252), (2009). Paul D. [doi.org]
Intense and recalcitrant pruritus is a symptomatic hallmark of LS. [liebertpub.com]
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]
- 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]
- 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]
Neurologic
- Stroke
Clitoral adhesions are released with delicate knife strokes. [ncbi.nlm.nih.gov]
Urogenital
- 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]
- 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]
- 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]
- Oligomenorrhea
Premature ovarian failure should be considered in a woman with hot flashes and oligomenorrhea or amenorrhea before age 45. Estrogen deficiency can lead to a thinned epidermis, labial adhesion, and dyspareunia. [ncbi.nlm.nih.gov]
Workup
On further workup, there was no evidence of disseminated disease involving other organs. [ncbi.nlm.nih.gov]
Serum
- Hypertriglyceridemia
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]
Treatment
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 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
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]
Etiology of the disease The exact etiology of lichen sclerosus has not been ascertained; however, evidence points to an increased likelihood of an autoimmune and genetic component. 4 In a study of 350 women with lichen sclerosus, researchers found that [clinicaladvisor.com]
Epidemiology
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]
In 1971, Progesterone used in LS, Wallace defines clinical factors and epidemiology of LS for all later reports. In 1976, Friedrich defines LS as a dystrophic, not atrophic condition; "et atrophicus" dropped. [en.wikipedia.org]
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]
The only predictor we have identified is a lack of consistent preventive treatment. [doi.org]
It can prevent women (or men) from having sexual intercourse, due to pain or scarring, and can affect urination. [healthywomen.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]