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