Guidelines Summary
The British Association of Dermatologists has issued updated guidelines on the management of lichen sclerosus in males and females. [41]
Studies have illustrated an increased incidence of tissue‐specific antibodies and associations with other autoimmune diseases, especially thyroid disease in female, but not in male, patients.
Female treatment guidelines
In adult female patients, after the diagnosis of anogenital lichen sclerosus is made, an initial 3‐month induction regimen is recommended. Clobetasol propionate 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 clobetasol propionate 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 achieve remission. 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 lichen sclerosus 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 her 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 clobetasol propionate 0.05% appears to be effective and safe to follow.
Male treatment guidelines
An association has also been noted between male lichen sclerosus and increased body mass index, coronary artery disease, diabetes mellitus, and smoking. The fact that lichen sclerosus is rare in men circumcised at birth, and that lichen sclerosus is documented in cases of urostomy, ileostomy, hypospadias, and hypospadias repair, suggests that irritation from urinary occlusion may play a central role in lichen sclerosus development.
The algorithm for male patients is somewhat different. After making the diagnosis of genital lichen sclerosus, clobetasol propionate 0.05% ointment must be commenced once daily for 1-3 months with an emollient soap substitute and barrier preparation. There are no randomized controlled trials for male lichen sclerosus management, but a large retrospective series demonstrated that 50% of male patients with lichen sclerosus responded to clobetasol propionate. A follow‐up assessment of response must be done at 3 months. If remission is achieved, then a follow‐up is planned for 6 months, followed by discharging the patient back to his general practitioner with an 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 pediatric 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 fixed erythematous areas must be urgently referred for tissue sampling to exclude intraepithelial neoplasia or invasive squamous cell carcinoma.
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Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.
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Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.
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Typical lichen sclerosus histology demonstrating homogenized edematous papillary (upper) dermis and effaced epidermis.
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More advanced vulvar lichen sclerosus; eroded areas need to be carefully examined and a biopsy sample should be taken to exclude coexistent squamous cell carcinoma. Courtesy of Wilford Hall Medical Center Dermatology slide files.
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Male genital lichen sclerosus may present with a sclerotic ring at the edge of the prepuce or anywhere on the glans itself. Advanced disease at the urethral os may lead to urinary obstruction. Courtesy of Wilford Hall Medical Center Dermatology Slide files.
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Late lichen sclerosus may show less edema in the upper dermis and more sclerosis throughout the dermis. Involvement of the lower dermis or fat may occur in lichen sclerosus/scleroderma overlap presentations.
