Lichen sclerosus is a chronic, inflammatory, mucocutaneous disorder of genital and
extragenital skin. It was first described by Hallopeau in 1881. In 1976, the International
Society for the Study of Vulvovaginal Disease concluded that the terminology LS should be
adopted for men and women. Both sexes are affected, but it is 6 to 10 times more prevalent in
women than in men. LS can occur at any age, but the incidence increases with age.
The aetiology of LS remains unknown thought infectious, autoimmune, and chronic irritation
was investigated for the develop of LS. The autoimmune pathogenesis seems most likely. An
increased incidence of autoantibodies to the extracellular matrix protein and autoantibodies
to BP180 antigen in LS are reported .Other potential markers include CD4+ T-cells clones,
which are found in overabundance in LS patients's tissue. and possibly promote fibroblast
cell proliferation.
Risk factor to progression of LS was occlusion and exposure of urine. The susceptible
epithelium may play a central role in the pathogenesis of LS. The skin may have an
isotraumatopic response, to urine, feces, and other non-specific liquid irritants in occluded
spaces and may play an important role in the etiology of LS in both men and women.
The Köbner phenomenon describes the occurrence of disease- specific lesions on normal
appearing skin after trauma and it is described in LS. Mechanical factors like friction due
to tight clothing, occlusion, surgical trauma, radiotherapy and scars are thought to play an
important role in triggering and maintaining LS .
Some patients with lichen sclerosus do not have any symptoms, while most patients experience
intense itching, discomfort and/or erosions/ulcers. LS is a debilitating disease, causing
itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual
dysfunction in women and men. Vulvar itching, bleeding, or pain, pain during sex, skin
bruising and tearing , blisters, easy bleeding from minor rubbing of the skin, trouble
urinating or pain with urination, painful erections (in men). Lichen sclerosus typically has
a remitting relapsing course that is complicated by permanent scarring of the affected areas.
Biopsy is worthwhile both to confirm the diagnosis and to exclude malignant change. It has
been suggested that the expression of selected cellular markers (such as p53, survivin,
telomerase, Ki-67, and cyclin D1) can help distinguish between indolent LS and LS with true
malignant potential.
Topical and systemic treatments have been addressed but with few results. Surgery represent
the solution for genital and urethral involvement. Depending on extension of genital and
urethral involvement, surgical repair can range from a minimally invasive treatment to a more
extensive reconstruction but still today surgery represents an open debate.