Lichen sclerosus

Abstract

Lichen sclerosus is a chronic skin condition with extragenital and/or genital involvement. The disease may develop in women and men of any age, but most frequently affects postmenopausal women. The aetiology of lichen sclerosus remains unknown. Genetic, infectious, environmental, autoimmune, and hormonal factors are presumed to influence its development. In most cases, lesions are located in the anogenital area. It is associated with sexual morbidity and is associated with an increased risk of cancerogenesis. The first-line treatment of lichen sclerosus includes ultrapotent corticosteroid ointments. Topical calcineurin inhibitors are considered second choice treatment. In recalcitrant cases alternative treatment methods (phototherapy and photochemotherapy, oral retinoids, methotrexate, cyclosporine, photodynamic therapy, adipose-derived stem cells and platelet-rich plasma, surgical management) are used. Because of a possible 3-8% risk of malignant transformation of genital lichen sclerosus to squamous cell carcinoma, early diagnosis is required, and long-term follow-up is recommended.streszczenie Liszaj twardzinowy jest przewlekłą chorobą skóry. Występuje w postaci pozagenitalnej i/lub genitalnej. Choroba jest stwierdzana u kobiet i mężczyzn niezależnie od wieku, ale najczęściej dotyczy kobiet w okresie po menopauzie. Etiologia liszaja twardzinowego jest nieznana. Przypuszcza się, że w jego rozwoju pewną rolę odgrywają czynniki genetyczne, zakaźne, środowiskowe, autoimmunologiczne oraz hormonalne. W większości przypadków zmiany są umiejscowione w okolicy anogenitalnej, co wiąże się z zaburzeniem funkcji seksualnych i możliwością kancerogenezy. W terapii pierwszego wyboru u pacjentów z liszajem twardzinowym stosuje się bardzo silnie działające maści z glikokortykosteroidami. Terapia drugiego wyboru opiera się na miejscowych inhibitorach kalcyneuryny. W przypadkach opornych na leczenie stosowane są alternatywne metody terapeutyczne (fototerapia i fotochemioterapia, doustne retinoidy, metotreksat, cyklosporyna, terapia fotodynamiczna, leczenie komórkami macierzystymi pochodzenia tłuszczowego i osoczem bogatopłytkowym, a także zabiegi chirurgiczne). Ze względu na ryzyko złośliwej przemiany postaci genitalnej liszaja twardzinowego w raka płaskonabłonkowego (3-8% przypadków) konieczne jest wczesne rozpoznanie i długoterminowa obserwacja pacjenta.

Extragenital lichen sclerosus: A comprehensive review

“…About 85% of total cases of LS are genital cases, while extragenital form comprises only 15–20% 4 . The extragenital form can occur simultaneously with genital lesions, but in 6% cases of LS, only extragenital form has been described 3,5 . The genital form tends to affect extremes of ages with 2 peaks, one at puberty and another around menopause, while extragenital form usually occurs in middle‐age adults.…”

Photodynamic Therapy in the Treatment of Vulvar Lichen Sclerosus: A Systematic Review of the Literature

“…The worldwide prevalence of lichen sclerosus ranges from 0.1% to 1.67% [ 5 ]. Of note, the exact prevalence of VLS has not been established and is probably underestimated, particularly in young women [ 6 ].…”

“…Several papers report a bimodal onset of disease, that is, in prepubertal children and postmenopausal women [ 2 , 7 ]. However, recent studies suggest that VLS may also affect some other age groups [ 5 ]. In terms of incidence, elderly women predominate (3%), while lichen sclerosus affects about 0.07% of men [ 8 , 9 ].…”

Successfully treated extragenital lichen sclerosus in a 2‐year‐old boy by baricitinib assessed by dermoscopy: A case report

“…The treatment of lichen sclerosus includes topical ultrapotent corticosteroids, topical calcineurin inhibitors, phototherapy and photochemotherapy, photodynamic therapy, oral retinoids, methotrexate, cyclosporine, adipose-derived stem cells and platelet-rich plasma and surgery. 3 Topical ultrapotent corticosteroids, topical calcineurin inhibitors are commonly used for children. We here report a case of extragenital lichen sclerosus in a boy successfully treated by baricitinib, after failure to topical application of calcineurin inhibitor.…”

“…Psoralen and ultraviolet A (PUVA) has been reported effective for the treatment of lichen sclerosus. 3 A case of lichen sclerosus successfully treated by baricitinib plus PUVA was reported, 7 which means there may be a synergistic effect between baricitinib and PUVA. Our case indicates single therapy of oral baricitinib is also effective for lichen sclerosus.…”