LICHEN SCLEROSUS: THE DISEASE
www.ls-bxo.org
www.lichensclerosus.org

History

The lichen sclerosus (LS) disease was first clinically described by Hallopeau in 1887 and given the name of lichen plan atrophique. Darier in 1892 reported the histological features of LS and named it lichen plan sclérux. Friedrich in 1976 proposed the removal of “et atrophicus” from the term lichen sclerosus et atrophicus, resulting in the current term lichen sclerosus. Stuhmer in 1928 fully described the LS involving male genitalia and named it balanitis ( chronic inflammation of the glans penis) xerotica (abnormally dry appearance of the lesions) obliterans (association of occasional endoarteritis) (BXO). For many years LS was reported in the urological literature as BXO. In 1995 the American Academy of Dermatology recommended that the term LS be used in future reports.

Epidemiology in male

LS has been reported in male patients of all ages. The exact prevalence of LS is unknown because male patients suffering from LS may present to various specialists including urologists, dermatologists and paediatricians and methods of diagnosis and treatment may differ. In 1971, the prevalence of LS was estimated to range from 1 to 300 to 1 of 1.000 of all patients referred to a community based dermatology department. A study of 1.178 boys with phimosis found that 40% had evidence of LS, with the higest incidence in boys 9 to 11 year old.

Symptoms and clinical presentation

LS cause destructive scarring that can lead to urinary and sexual problems and a decrease of quality of life. Symptoms are pruritus and soreness, difficulty in retracting the foreskin and a poor urinary stream. Examination shows typical flat, atrophic, ivory to white colored papules that coalesce in plaques of varying sizes, commonly with a non-retractile prepuce and meatal stenosis.

LS and urethral stricture

Controversies exist about the incidence of LS involving the anterior urethra in adult men. The dermatological literature fails in recognizing the involvement of the anterior urethra in male patients with genital LS. On the contrary, urological literature began to emphasize the urethral involvement in  BXO in 1970. In 1971, Bainbridge et al. reviewing the natural history and histological sections of 17 cases of BXO, first emphasizedthe diagnostic histological feature of the disease in urethral tissues. In 1978, Mallo et al. reported 5 cases of BXO, emphasizing  urethral involvement with histological findings from the foreskin, meatus and penile urethra. In 1979, Herschorn et al. described a case of biopsy-proven BXO that involved not only the usual areas, but the anterior urethra, as well. In 1979, Khezri, reviewing a series of 20 patients with histologically proven BXO, suggested that urethral involvement is limited to the squamous epithelium of the external urinary meatus and fossa navicularis, and that there is no evidence to indicate that associated strictures to the urethra were also due to BXO. In 1998, in a report on 114 patients undergoing anterior urethroplasty for non-traumatic conditions, 28 (24.5%) cases of urethral strictures due to LS were identified, and histological assessment of the urethra uniformly showed LS characteristics. In 1999, Barbagli et al. reported that, in a series of 106 patients who underwent urethroplasty for anterior urethral strictures, 31 (29%) received a specific pathological diagnosis of LS, which involved the meatus in 19%, navicularis urethra in 16%, penile urethra in 3% and the entire anterior urethra in 52% of the cases. A high incidence of LS is reported in patients with failed hypospadias repair. However, the relationship between LS and anterior urethral strictures still remains an open and controversial issue and further investigative studies are mandatory.

Etiology

The etiology of LS remains unknown, but a variety of causes have been proposed:

  1. Autoimmune theory and genetic factors

  2. Infection

  3. Hormonal influence

  4. Local factors

Therapy

The goal for treatment of LS are to alleviate symptomps, to prevent cancer, to solve urinary obstruction.
Medical treatment of includes steroids, hormones, systemic therapy.
Surgical management includes circumcision, meatoplasty, urethroplasty.