Extracellular matrix protein 1 autoantibodies in male genital lichen sclerosus

Edmonds, E. V. J.; Oyama, Noritaka; Chan, Ien; Francis, N.; McGrath, John A.; Bunker, C.B.

doi:10.1111/j.1365-2133.2011.10326.x

Cited by 54 publications

(50 citation statements)

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“…And what of autoimmunity? Epiphenomenon; chronic itiflammation exposes epitopes that elicit autoreactivity in those genetically predisposed and this may compound the infiammatory process (11,12). Occam's razor (http://en.wikipedia.org/wiki/Occam's razor) favours the urinary contact hypothesis.…”

Section: Discussionmentioning

confidence: 96%

Urinary Voiding Symptomatology (Micro-incontinence) in Male Genital Lichen Sclerosus

Bunker¹,

Patel²,

Shim³

2013

Acta Derm Venerol

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Male genital lichen sclerosus (MGLSc) is responsible for male dyspareunia, urological morbidity and squamous carcinoma of the penis. The aetiology is essentially unknown but an autoimmune mechanism is most favoured. The first author of this paper (CBB) has argued that chronic, occluded, exposure of susceptible epithelium to urine is perniciously central to the pathogenesis (1-3). MGLSc never occurs in men who were circumcised at birth; it is associated with trauma, instrumentation, genital jewelry (piercing), and gross anatomical abnormalities (e.g. fi-ank hypospadias); it recurs in grafts, and it rarely causes perianal disease: in striking contrast with women, the male perineum is rarely chronically exposed to urinary irritation (3). Sub-preputial wetness has been associated with foreskin length and balanitis (4, 5), however GLSc has not been linked to napkin or diaper dermatitis in children although there has been a report associating it with incontinence in the elderly (6).Symptomatology associated with the leaking or dribbling of small amotmts of urine (micro-incontinence) in men may not be readily volunteered or elicited. However, it has become apparent to CBB over many years of interviewing men with GLSc that such symptomatology is frequently present. METHODSTo attempt to quantify the presence of this symptomatology in MGLSc 3 approaches to the clinical records of cases were employed.Firstly, we scrutinised the Male Genital Dermatoses Clinic (MGDC) at one of our institutions. The work load of this clinic has been described (7) as has the spécifie experience of MGLSc (3). For the last four years, each new case presenting to the weekly MGDC has been assessed by the attending physician using a routine, standard, structured form to record symptoms and signs. The patient is asked specifically about his urinary voiding patterns and habits; explicit questions are asked about post-mieturition micro-incontinence (i.e. leaking or dribbling of small quantities of urine from the urinary meatus). Over a 12 month period, all those patients, uncircumeised at presentation, diagnosed with imequivocal MGLSc (diagnosed either by punch biopsy or post-circumcision preputial specimen histologieal analysis) were identified at follow-up. A similar number of patients with an unequivocal alternative diagnosis was identified. The initial clerking forms of these MGLSc and non-MGLSc cases were then inspected to determine their presenting symptomatology.The second approach was to review the initial clerking forms of all new cases of male genital skin disease seen in 4 consecutive MGDCs and correlate the responses to the questions about voiding with the working clinical diagnosis in each case.Finally, we retrospectively reviewed the records and/or clinic letters of all the patients diagnosed clinically with MGLSc in the general dermatology clinics done by one of us in another institution over a one-year period. RESULTSIn the first study (from Spring 2010 to Spring 2011) 17 patients (mean age±SD 45.9 ±14.4 years) were identified with hist...

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Section: Discussionmentioning

confidence: 96%

Urinary Voiding Symptomatology (Micro-incontinence) in Male Genital Lichen Sclerosus

Bunker¹,

Patel²,

Shim³

2013

Acta Derm Venerol

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“…The most common autoimmune diseases were thyroiditis (12%), alopecia areata (9%), vitiligo (6%) and pernicious anaemia (2%) [4,5,6,7,8]. IgG1 autoantibodies directed against the glycoprotein of an extracellular matrix (ECM1) were found in the serum of about 70% of women with LS; however, even in the males, the concentration of anti-ECM1 antibodies was higher than in the controls in some recent clinical records reported in literature [9,10]. There are, however, conflicting opinions concerning the true pathogenetic mechanism, since it would seem that the trigger is actually the local chronic irritation and that the auto-antibodies develop only later.…”

Section: Introductionmentioning

confidence: 99%

Genital Lichen Sclerosus in Male Patients: A New Treatment with Polydeoxyribonucleotide

Zucchi¹,

Cai²,

Cavallini³

et al. 2016

Urol Int

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Lichen sclerosus (LS) is an inflammatory and chronic disease that causes itching, pain, dysuria, urinary retention, dyspareunia and sexual dysfunction, in both men and women. The first line pharmacological treatment is based on the use of topical steroids, which have proved their efficacy in 60-70% of cases but with a high rate of relapses in time (50-80% of the patients of both sexes). The purpose of our non-randomised prospective pilot study was to evaluate the efficacy and tolerability of a new loco regional therapy with polydeoxyribonucleotides (PDRN) in the treatment of male genital LS. PDRN is an healing and anti-dystrophic drug with anti-inflammatory effects, through the reduction of cytokine. Twenty one male patients suffering from genital LS were recruited. All the patients were submitted to treatment using loco-regional intradermal injections with PDRN. Dermatology Life Quality Index (DLQI), International Index of Erectile Function (IIEF-5) and PGI-I questionnaires were administered at baseline and at the end of treatment in order to evaluate the results of this treatment. The statistical evaluation of the data obtained with the DLQI questionnaire showed a marked improvement of the overall conditions in terms of quality of life, with an average change of scores from 15 to 4 (p < 0.0001). PGI-I questionnaire showed that 80% of the patients treated considered their post-treatment conditions as ‘improved'. There was no significant change in terms of sexual function according to the IIEF questionnaire (p = 0.189). The results obtained show the excellent tolerability and the therapeutic efficacy of PDRN, with clear improvement of the local symptoms and of the quality of life.

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“…Bu hastalıklar açısından ailesel ve kişisel otoimmun hastalık öyküsü LS için risk faktörü kabul edilir (1,5,6). LS'nin otoimmun hastalık olduğunu düşündüren bir diğer bulgu, genital LS'li hastalarda dolaşımda ekstraselüler matriks protein (EMP) -1'e karşı Ig G yapısında antikor varlığının gösterilmesi olmuştur (1,7). Ek olarak, LS'de bazal membran proteinlerine karşı antikor üretimi ve T hücre reaktivitesi saptanmıştır (5).…”

Section: Etyopatogenezunclassified

“…Yine de, genital LS'de bu antikorların varlığı kimi yazarlar tarafından epifenomen olarak değerlendirilmektedir. Duyarlı epitelin idrar ile hasarlanması sonucu açığa çıkan antijenik özel-likte yapıların antikor üretimine neden olduğu düşünülmüştür (7,8). EMP-1 hem kadın hem de erkek genital LS'de anlamlı düzeyde yüksek saptanmış, ancak hastalığı başlatan bir faktör olarak bütünüyle kabul görmemiştir.…”

Section: Etyopatogenezunclassified

Liken Sklerozus

Ergin¹

2012

Turk J Dermatol

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Öğrenme HedefleriLiken sklerozusun etyopatogenezinde suçlanan faktörlerin belirlenmesi, klinik ve histopatolojik özelliklerinin tanınması, ayırıcı tanısına giren diğer dermatozların dışlanması, dermatozun ve tedavi sırasında ortaya çıkabilecek komplikasyonlarının ayırd edilebilmesi ve algoritmik tedavi programının oluşturulmasıdır. TanımLiken sklerozus (LS); porselen beyazı maküller ile karakterize, progresif, sklerozan inflamatuvar bir dermatozdur (1). Tarihçeİlk kez 1887 yılında, Hallopeau tarafından, liken planusun atrofik formu (lichen plan atrophique) şeklinde rapor edilen hastalığı, AbstractLichen sclerosus is a progressive, inflammatory dermatosis characterized with porcelain white sclerotic plaques most commonly in the anogenital area of postmenopausal women. Children and men can also be affected. Extragenital involvement may occur. Even though the etiology is unknown; autoimmune, genetic, hormonal and infectious factors are suggested. Lichen sclerosus is associated with autoimmune diseases such as thyroid disease, alopecia areata, vitiligo, diabetes and pernicious anemia. An increased incidence of autoantibodies to the extracellular matrix protein 1 is shown in sera of patients with lichen sclerosus. Familial occurence and an association with HLA DQ7 supports the presence of genetic factors in its etiology. However, the role of estrogen and androgens could not be demonstrated as etiologic factors. Clinical similarity with acrodermatitis chronica atrophicans led researches to be held for the detection of Borrelia burgdorferi antibodies ended with conflicting results. Disease may be triggered with trauma in the presence of genetic predisposition. Pruritus is the main symptom. Pain, burning and dysesthesia may occur. Lichen sclerosus may be confused with sexual abuse in girls. Furthermore, it is more severe in the presence of sexual abuse. If not treated it leads to permanent anatomical disorders in the anogenital region. It is one of the most common cause of acquired phimosis in boys. Meatal stenosis and urinary obstruction may develop. Erectile dysfunction and dyspareunia due to introital stenosis may cause psychosexual problems in men and women. Perianal involvement causes constipation in women and girls. Long-standing anogenital lesions have the risk of developing squamous cell carcinoma. Verrucous carcinoma rarely occurs. Extragenital symptoms do not have similar risks. Ultra-potent topical corticosteroids are used as the first line therapy. In the case of unresponsiveness to corticosteroids the alternative approaches are topical immunmodulators, UVA1, calcipotriol, retinoids, and photodynamic therapy. In the presence of neoplasia and other complications surgical interventions are considered. Due to mainly preputial involvement, circumcision is an alternative procedure to topical corticosteroids in men. Often it may be curative. Lichen sclerosus is a disease with serious complications that needs life-long follow-up. Patients should be trained and encouraged for effective participation to the treatment...

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Extracellular matrix protein 1 autoantibodies in male genital lichen sclerosus

Cited by 54 publications

References 12 publications

Urinary Voiding Symptomatology (Micro-incontinence) in Male Genital Lichen Sclerosus

Urinary Voiding Symptomatology (Micro-incontinence) in Male Genital Lichen Sclerosus

Genital Lichen Sclerosus in Male Patients: A New Treatment with Polydeoxyribonucleotide

Liken Sklerozus

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