Lichen Sclerosus of the Male Genitalia -- The Current Level of Knowledge.

Introduction and Objectives. Although discovered in the late 19th century by the American physician Robert F Weir, lichen sclerosus is still a condition not fully understood. Initially considered a simple skin disorder, it mainly affects the genital regions of both men and women, producing white hypomelanotic lesions, that if left untreated tend to evolve into more serious health related problems. In this paper we conducted a thorough review of the literature in order to assess the current level of knowledge of lichen sclerosus of the male genitalia, to identify its etiology, clinical presentation and diagnostic methods, and establish the main treatment options. Materials and Methods. So far a total of five etiologic factors have been considered for lichen sclerosus: autoimmunity, genetics, hormonal infiuence, repeated microtrauma and infections. As only empirical observations sustain each of them, and none alone can explain or predict lichen sclerosus, it is assumed that this condition has a multifactorial origin, and the precise appearance mechanism has yet to be determined. Regarding the lichen sclerosus of the male genitalia, four distinct clinical stages of presentation have been described, based on the extent of the disease and the complications it produces. During the first stage the white hypomelanotic lesions can be observed at the level of the prepuce, that gets scarred and brittle, and can lead to phimosis. For the second stage the lichen lesions also affect the glans of the penis, that can fuse with the phimotic prepuce. The third stage marks the involvement of the urethra, patients exhibiting stenoses and strictures, of various degrees of severity. And the fourth and most dangerous stage represents the malignant transformation of the involved tissues, resulting in squamous cell carcinoma of the penis or urethra, verrucous carcinoma of the penis, and erythroplasia of Queyrat. While definitive diagnosis is obtained after a histopathological examination of a removed specimen, the clinical presentation can often be enough to identify lichen sclerosus. Imaging like ultrasound, computed tomography or magnetic resonance is also required to correctly assess the extent of the complications. Results. Various treatment options have been employed for lichen sclerosus thus far, ranging from local ointments and creams to surgical excision and correction in the complicated cases. Some, like the revulsives and X-rays, retain only historic value, having been already abandoned. Out of the topical treatments used nowadays, meant to alleviate the symptoms and reduce the lichen affected areas, the most common are corticosteroids, calcineurin inhibitors, hormonal creams and retinoids. The same effects have been obtained with some forms of phototherapy. But when lichen sclerosus evolves beyond simple skin lesions, surgery is required in order to correct the acquired complications. If phimosis is associated, a complete circumcision of the involved tissue is needed. For the second stage, if the prepuce is fused to the glans, a restoration of lost obscured coronal sulcus procedure achieves the best results. In the third stage, when urethral strictures develop, various forms of urethroplasties are necessary to restore normal micturition, and even perineal urethrostomies are considered in cases of panurethral strictures. The fourth and most severe stage of lichen sclerosus, marked by the malignant transformation, imposes an oncologic treatment catered to the extent of the disease, from a simple excision of a prepuce or penile tumor, to complete emasculation and ilio-inguinal lymph node dissection, or even palliative chemotherapy in metastatic cases. Conclusions. Although known for almost 150 years, lichen sclerosus is still not a fully understood disease. Being considered a simple skin disorder for a long time, it's been overlooked by both patients and physicians, and its true incidence is underestimated. Only recently, after all the evolutive complications of lichen sclerosus have been identified, more effort has been invested in the diagnosis and treatment of this condition. While uncomplicated lichen sclerosus can be managed by topic therapies, advanced forms require complex surgical interventions and experienced physicians in order to achieve the best results. But further research is still needed to clarify the etiology and evolution risks. [ABSTRACT FROM AUTHOR]