In 2002, the British Association of Dermatologists produced the first guidelines for the management of lichen sclerosus (LS). These guidelines have been updated twice since then, once in 2010 and again recently in 2018 (published in this issue of the BJD). They aim to offer evidence–based recommendations for diagnosis and management of LS in both adult and paediatric patients. The authors offer a comprehensive study of genital LS in particular, a disease with an estimated prevalence in adult females of up to 3% and 0·07% in males.
We still know very little regarding the aetiology and pathophysiology of LS and thus have not been able to utilize targeted therapies or advocate effective prevention. In the clinical setting, the main challenge in managing LS remains the physician's ability to decide confidently on the appropriate steroid potency and duration of use that would result in effective symptom control and halt disease progression, but not increase the risk of potential side–effects. This uncertainty is usually heightened when treating LS in children. Determining the need for maintenance therapy and the frequency and length of follow–up is also another difficulty we face, given that over one–half of female patients discharged from specialized U.K. vulval clinics are then not followed up appropriately by their primary–care providers.
In comparison with previous guidelines, the 2018 guidelines offer a more expanded examination of LS aetiology, possibly reflecting the increase in academic interest and research geared towards achieving a better understanding of LS pathophysiology. Studies have illustrated an increased incidence of tissue–specific antibodies and associations with other autoimmune diseases, especially thyroid disease in female,[5–7] but not in male,[8,9] patients. An association has also been noted between male LS and increased body mass index, coronary artery disease, diabetes mellitus and smoking. The fact that LS is rare in men circumcised at birth, and that LS is documented in cases of urostomy, ileostomy, hypospadias and hypospadias repair, suggests that irritation from urinary occlusion may play a central role in LS development.[1,9]
Clear algorithms for management and follow–up are included in the guidelines. In adult female patients, after the diagnosis of anogenital LS is made, an initial 3–month induction regimen is recommended. Clobetasol propionate (CP) 0·05% ointment should be applied once a day for a month, on alternative days for the next month, and then twice weekly for the third month, in combination with a soap substitute and a barrier preparation. This recommendation is based on randomized control trials that found CP 0·05% to be more effective than topical tacrolimus 0·1%, testosterone 2% in petrolatum and ultraviolet A1 home–based phototherapy, and equally efficacious to mometasone furoate 0·1%. Two follow–up visits, at 3 months and 6 months, are suggested. After 3 months, about 70% of adult female patients will achieve remission.[1,15] Treatment failure may result from poor compliance or coexistent vulvodynia, and a biopsy might be required at this point to confirm the diagnosis. The topical steroid should be continued in a regular regimen once or twice per week for ongoing active LS disease.
Female patients must be followed up every 6–12 months until there is symptom control, good sexual function and no further alteration in architecture. At this time, the patient can be discharged back to their general practitioner with annual checks and clear instructions on self–monitoring. The guidelines state that topical steroids are to be used as needed thereafter, recommending a practical approach and suggesting that the treatment be tapered to maintain symptom control and resolution of skin thickening and ecchymosis, but not pallor, as pallor is known not always to resolve completely. Female children should be referred to specialized vulval services, and the same adult treatment algorithm using CP 0·05% appears to be effective and safe to follow.
The algorithm for male patients is somewhat different. After making the diagnosis of genital LS, CP 0·05% ointment must be commenced once daily for 1–3 months with an emollient soap substitute and barrier. There are no randomized controlled trials for male LS management, but a large retrospective series demonstrated that 50% of male patients with LS responded to CP. A follow–up assessment of response must be done at 3 months. If remission is achieved, then a follow–up is planned for after 6 months, followed by discharging the patient back to his general practitioner with emphasis on self–examination. A repeat course of topical treatment for 1–3 months is recommended in those who relapse.
Treatment failure in adult and paediatric male patients can result from tight phimosis, which requires circumcision. Obesity and burying of the penis can also result in treatment failure; this necessitates weight reduction and in some cases bariatric surgery and reconstructive penile surgery. Patients might have residual active disease even after circumcision and might then require a repeat course of a potent topical steroid. It is therefore wise to inform all male patients of this possible risk before they consent to undergoing a therapeutic circumcision.
Long–term follow–up in a specialist clinic is reserved for patients with troublesome symptoms, atypical disease, previous cancer or any type of diagnosed or pathologically uncertain intraepithelial neoplasia. Persistent erosions, ulcers, and and fixed erythematous areas must be urgently referred for tissue sampling to exclude intraepithelial neoplasia or invasive squamous cell carcinoma.
The 2018 guidelines offer recommendations applicable not only in secondary–care settings but also in primary care. This guidance helps physicians address 'steroid phobia' by clearly advising the application of half a fingertip unit of CP in female patients and about one fingertip unit of CP in male patients, such that a 30–g tube lasts 3 months. Moreover, it offers physicians a reliable benchmark when deciding on matters such as treatment and follow–up protocols. The aim of our treatment is not only maintaining disease control and preventing scarring and malignancy, but also maintaining adequate urinary and sexual function, as this has a significant impact on a patient's quality of life. The psychosexual factor deserves our attention during every clinical consultation, given that it more often than not can impact compliance and overall treatment success. The authors advise giving the patient the opportunity to express their concerns about sexual function; one might even suggest routinely completing questionnaires such as the Dermatology Life Quality Index or the Female Sexual Function Index, as this might optimize the quality of care we offer patients with this chronic condition.
In conclusion, there remains much for us to learn about LS. There is a lack of high–quality evidence, with only a few published randomized controlled trials to date. Moving forward, this should encourage us to pursue more targeted research questions. The James Lind Alliance Lichen Sclerosus Priority Setting Partnership, which is under way, will hopefully guide future scientific work towards better understanding of the aetiology, treatment and prevention of LS and elucidating how similar or potentially dissimilar the condition can be in male and female patients.
The British Journal of Dermatology. 2018;178(4):823-824. © 2018 Blackwell Publishing