Penile lichen sclerosus: An enigmatic and challenging disease

In this retrospective study, the authors present their data from 365 patients with lichen sclerosus (LS). The authors describe their diagnostic and therapeutic approach, the patient's short-term outcome and provide an excellent literature review of this disease.

LS appears to be primarily an autoimmune phenomenon with a known genetic component.[1] Literature data suggest that LS may not be a purely dermatologic condition and that metabolic and life-style factors such as elevated body mass index, diabetes mellitus, coronary artery disease and smoking may have a contributing role in the development and chronicity of LS.[2]

LS progressively affects the prepuce, glans and meatus. Authors of the study observed that these locations were also the most commonly affected (63%). The frenulum often becomes contracted and circumferential involvement of the preputial aperture leads to a progressive fibrous phimosis. The surface of the glans and inner prepuce may ulcerate.

In Western countries today, the most common cause for inflammatory urethral strictures is LS. The virtual increase of this dermatologic-urologic pathologic entity has probably resulted from its relatively recent identification and classification as a cause of urethral disease. However, the incidence of urethral involvement in male patients with genital LS and the percentage of LS strictures of the total number of strictures remain unknown. Percentage of urethral strictures in authors study was relatively high approaching 37%. Urethral involvement starts at the meatus and may include every site of the anterior urethra. The blood supply to the penis and urethra is at its most tenuous point distal from the body and the development of microvascular disease in conjunction with LS may explain the anatomically distal to proximal progression of this condition in urethral stricture formation. Meatal stenosis leads to high-pressure voiding and inflammation of the periurethral glands, with potential progressive panurethral involvement.[3] Thus, cystourethroscopy may be advised in cases of potential urethral involvement to exclude other pathology and also to identify the extent of any urethral involvement with lichen sclerosus.[4]

The European Association of Urology guidelines identify LS as a strong risk factor for penile squamous cell carcinoma (pSCC).[5] The rate of malignancy among patients with LS is estimated to be 2.3-8.4%, whereas histologically-confirmed LS/Balanitis xerotica obliteran is identified in 28-44% of patients with pSCC.[6]

Circumcision was done in 237 patients while 33 patients underwent meatotomy along with circumcision. A total of 30 patients who were already circumcised, were undergone only meatotomy. All the cases of LS induced stricture were treated with buccal mucosal graft either in one stage or in two stages. A trial of steroids may alleviate the need for surgery in selected cases, although if phimosis is an issue then a circumcision is generally required. Severe lichen sclerosus may require extensive surgery involving either glans resurfacing with grafting or complete degloving with or without split skin grafting depending on any previous circumcision.[7]

Intervention on a meatal or penile stricture in the early stages of the disease, instead of useless dilation, could be useful in stopping the large diffusion of urethral involvement. Extensive or complex surgery may be required which should be limited to centers with experience. Follow-up of patients with lichen sclerosus is important as the disease may recur. Interestingly lichen sclerosus may recur in the scar line of any previous surgery due to the Koebner phenomenon, where recurrent lichen sclerosus appears despite “clear margins” on the original excision. Regular follow-up of circumcised patients with persistent or chronically active LS and a low threshold for biopsy of suspicious lesions is advisable.

In conclusion LS is completely cured if diagnosed and treated early by medical and surgical means. Dermatologists and urologists should have in mind all the diagnostic and therapeutic aspects of the disease and its complications, particularly penis cancer.