CLINICAL REPORT
365
ActaDV ActaDV Advances in dermatology and venereology Acta Dermato-Venereologica
Clinical Features , Complications and Autoimmunity in Male Lichen Sclerosus
Despina KANTERE 1 , Gunilla ALVERGREN 2 , Martin GILLSTEDT 1 , Fani PUJOL-CALDERÓN 3 and Petra TUNBÄCK 1
1
Department of Dermatovenereology , Sahlgrenska University Hospital , Göteborg , 2 Department of Dermatovenereology , Södra Älvsborgs Hospital , Borås , 3 Department of Rheumatology and Inflammation Research ( current affiliation Department of Psychiatry and Neurochemistry ), University of Gothenburg , Göteborg , Sweden
Lichen sclerosus is a chronic inflammatory disease associated with substantial morbidity . Knowledge of the aetiology and progression of lichen sclerosus is therefore needed . In this cross-sectional study , 100 male patients diagnosed with lichen sclerosus were interviewed and examined . Since there is a possible link between lichen sclerosus and autoimmunity , blood tests were analysed for thyroid disease , antinuclear antibodies and antibodies to extracellular matrix protein 1 , but autoimmunity was found to be infrequent . In 72 participants active genital lichen sclerosis was observed and complications were common ; 27 patients had preputial constriction and 12 meatal engagement . In total , 13 patients needed a referral to the Department of Urology , including one patient with suspected penile cancer . In conclusion , despite available treatment with ultra-potent steroids and circumcision , lichen sclerosus in males is frequently complicated by phimosis and meatal stenosis . However , the disease can also go into remission , as seen in 27 % of our patients .
Key words : lichen sclerosus ; circumcision ; penile cancer ; autoimmunity .
Accepted Sep 19 , 2016 ; Epub ahead of print Sep 27 , 2016 Acta Derm Venereol 2017 ; 97 : 365 – 369 .
Corr : Petra Tunbäck , Department of Dermatovenereology , Sahlgrenska University Hospital , SE-413 45 Göteborg , Sweden . E-mail : petra . tunback @ derm . gu . se
Lichen sclerosus ( LS ) is a chronic disease and spontaneous remissions are considered rare . However , adequate treatment can usually control the disease and limit the risk of scarring , dyspareunia , meatal stenosis and malignant evolution . First-line treatment is topical ultra-potent clobetasol propionate , but if the effect is inadequate the next choice in males is circumcision ( 1 ). Also , local treatment with tacrolimus can be an alternative ( 1 ). The disease is more common in females than males ( 3 – 10 : 1 ), but can also be seen in children and middleaged men ( age range 30 – 50 years ) ( 2 ). In males , LS is considered to be a disease of the uncircumcised individual , although it can also persist after circumcision ( 3 ).
The aetiology of LS is unclear . Studies of females with LS have demonstrated an association with autoantibodies and autoimmune disease ( 4 – 6 ). These reports support the hypothesis of autoimmunity as a pathogenesis of LS in females , but this has not been universally confirmed ( 7 ). In men , the link to autoimmunity has not been investigated to the same extent , and instead a main cause is considered to be the occlusive effect of the prepuce . It is also suggested that exposure of the sensitive epithelium of glans and prepuce to urine is a pathomechanism of LS in males ( 8 ). Circumcision removes the occlusive effect of the foreskin and reduces the koebnerization and consequences of micro-incontinence after micturition . These observations suggest a sex difference in the aetiological background of LS .
The primary aims of this cross-sectional study of 100 male patients with LS were to investigate the clinical signs and complications of LS in males , the effect of circumcision , and the link between autoimmunity and LS .
MATERIAL AND METHODS
The study was approved by the ethics committee of the Medical Faculty of the University of Göteborg , Sweden .
Study participants
In our earlier study of LS , a retrospective analysis was performed of records from 771 male patients diagnosed with LS during the period 1997 – 2007 ( 9 ). In order to be included typical clinical criteria for LS had to be fulfilled ( 1 ). Of the 771 patients , 632 ( 82 %) agreed to visit and , amongst them , 100 patients were randomly selected for an appointment at the Departments of Dermatovenereology in Göteborg ( 75 patients ) and Borås ( 25 patients ) during 2012 . The study participants had been diagnosed with typical LS on clinical grounds , by a dermatovenereologist , between the years 1997 and 2007 . In 39 patients ( 39 %) the diagnosis was also verified with a biopsy . If the clinical picture was typical , a biopsy was not considered essential , according to international guidelines for management of LS ( 1 ). Biopsies were always performed when the clinical picture was not typical , if dysplasia was suspected and in cases not responding to treatment .
In order to classify the disease as active , the patient would present with either atrophy , ecchymoses , erythema , induration or preputial constriction , in addition to hypopigmentation . The disease was considered inactive if none of the above-mentioned signs of active LS had been present for at least one year . A detailed medical history and dermatological assessment were carried out at the visit . Data on phimosis , meatal stenosis , signs of extragenital LS , and the activity of the genital disease were collected , as was information on circumcision and the presence of autoimmune disease in patients and first-degree relatives .
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2537 Acta Derm Venereol 2017 ; 97 : 365 – 369