Genital Lichen Sclerosus – Has Th ere Been any Progress in Treatment?

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Abstract

Lichen sclerosus (LS) is a chronic inflammatory dermatosis localized mainly in the anogenital region, accompanied by itching, atrophy and sclerosis. Progressive destructive scarring in genital lichen sclerosus (GLS) may result in burying of the clitoris in females and phimosis in males. Affected persons have an increased risk of genital cancers. It is often unrecognized in everyday clinical practice due to undiagnosed squamous cell carcinoma at the site of lesions. Remissions are rare and the estimated remission rate is only 16%. GLS is a lifelong, incurable condition, but signifi cant improvement can be achieved. Numerous therapeutic modalities have been used in GLS; unfortunately, the number of controlled studies is small and the results are mostly related to the management of symptoms, not the progression of the disease and destructive scarring. A systemic meta analysis of seven randomized controlled trials on local therapy of GLS was performed. It included a total of 249 patients treated with six topical agents: clobetasol propionate, mometasone furoate, testosterone, dihydrotestosterone, progesterone and pimecrolimus. Topical corticosteroids, clobetasol propionate 0.05% (highly potent) and mometasone furoate 0.05% (potent), showed to be significantly more efficient compared to placebo. Pimecrolimus 1% cream and clobetasol propionate 0.05% showed similar efficacy. Both agents have proven effective in the treatment of GLS: there was no statistically significant difference in relieving symptoms of pruritus and burning/pain. Tacrolimus 0.1% ointment also proved to be effective in the treatment of GLS. Topical androgens and progesterone did not show significant efficacy. Topical tretinoin and calcipotriol have been used with limited success, but they may induce irritation, so they are rarely used in the treatment of GLS. Other therapeutic options for GLS include ultraviolet A1 (UVA-1) phototherapy, methotrexate, retinoids, cyclosporine, stanozolol, hydroxychloroquine, calcitriol, laser and photodynamic therapy, but the number of patients is small to allow for conclusive assessment. Surgery is not a standard therapeutic option for GLS.

In conclusion, treatment of GLS should be carried out in two phases: introduction of remission and maintenance of remission; topical therapy should include highly potent corticosteroids once daily during three months, followed by twice per week, or twice daily during 4 to 6 weeks, and then twice per week. There are different opinions regarding maintenance therapy: application of super potent or potent topical corticosteroids; these patients need long-term, several-year follow-up, although there is no agreement what parameters should be assessed; treatment efficacy is often reduced to monitoring GLS symptoms.

Sažetak

Lichen sclerosus (LS) je hronična upalna dermatoza, lokalizovana uglavnom u anogenitalnoj regiji, praćena svrabom, atrofi jom i sklerozom. Progresivno destruktivno ožiljavanje u genitanom lihenu sklerozus (GLS) može dovesti do prekrivanja klitorisa kod žena i fi moze kod muškaraca. Postoji povećan rizik za genitalni kancer. U praksi se dešavaju previdi zbog nedijagnostikovanja spinocelularng karcinoma na mestu lezija. Remisije su retke, kod lečenih do 16%. Lečenje GLS je dugotrajno, nema izlečenja, ali je moguće postići značajno poboljšanje. Brojna terapijska sredstva primijenjuju su za lečenje GLS, nažalost, broj kontrolisanih studija je mali a dobijeni rezultati se odnose na kontrolu simptoma, ali ne i na progresiju bolesti i pojavu ožiljavanja. Urađena je metaanaliza na sedam randomizovanih kontrolnih studija koje su se odnosile na lokalnu terapiju GLS. Analiza je obuhvatila ukupno 249 lečenih pacijenata i šest lokalnih preparata: klobetazol propionat, mometazon furoat, testosteron, dihidrotestosteron, progesteron i pimekrolimus. Topijski kortikosteroidi, klobetazol propionat 0,05% (jako potentan) i mometazon furoat 0,05% (potentan), u odnosu na placebo pokazali su se značajno efi kasnijim. Pimekrolimus 1% krem u komparaciji sa klobetazol propionatom 0,05%, pokazao je sličnu efi kasnost. Oba preparata su se pokazala efi kasnim u lečenju GLS: nije utvrđena statistički značajna razlika u njihovoj efi kasnosti kada su kupiranje pruritusa i pečenja/ bola u pitanju. Efi kasnim u lečenju GLS pokazao se i takrolimus u obliku 0,1% masti. Topijski androgeni i progesteron nisu ispoljili značajnu efi kasnost. Topijski tretinoin i kalcipotriol mogu dati poboljšanje, ali i iritaciju, pa se retko primenjuju u lečenju GLS. Druga terapijska sredstva koja se primenjuju u GLS su fototerapija (UVA-1 rays), metotreksat, retinoidi, ciklosporin, stanazolol, hidroksihlorokvin, kalcitriol, laseri i fotodinamička terapija, ali je broj lečenih ovim preparatima mali da bi se donosili temeljni zaključci. Hirurška intervencija kao primarni oblik lečenja GLS nije indikovana.

Zaključak. Lečenje GLS treba sprovoditi kroz dve faze, uvođenjem u remisiju i održavanjem postignutog efekta; u lokalnoj terapiji treba koristiti jako potentne kortikosteroide jedanput dnevno tokom tri meseca, zatim dvaput nedjeljno, ili dvaput dnevno 4 do 6 nedelja, a potom dvaput nedeljno. Da li u održavanju postignutog učinka treba primenjivati superpotentne ili potentne topijske kortikosteroide mišljenja su različita; potrebno je dugotrajno praćenje ovih bolesnika, više godina, mada ne postoji saglasnost koje parametre treba procenjivati; najčešće se procena efi kasnosti leka svodi na praćenje simptoma.

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Serbian Journal of Dermatology and Venereology

The Journal of Serbian Association of Dermatovenereologists (SAD)

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