Lichen sclerosus is a lymphocyte-mediated chronic inflammatory dermatitis that most normally involves the anogenital country. Treatment options include topical corticoids, tacrolismus, C dioxide optical maser extirpation and eventually vulvectomy as surgical attack. The aim of this survey is to describe a stubborn anogenital LS topic and depict the usage of V-Y promotion flap for the Reconstruction of the perineal defect after vulvectomy.
Case: A relentless LS instance who was treated succesfully with surgery is reported and literature is reviewed. The patient had good tolerated the surgical process with satisfactory decorative consequences.
A multidisciplinary squad work would be ideal in the direction of relentless LS instances. Surgical intervention of LS should be reserved as a last resort for patients who were furnace lining to other medical options and in the instance of a surgical effort recontruction of the vulva should be good known.
Cardinal words: Lichen sclerosus, Vulvectomy, Vulvar Reconstruction.
Lichen sclerosus ( LS ) is a benign, chronic tegument disease that most normally occures in the anogenital epithelial tissue ( 1 ) . It affects both gender and kids particularly adult females in postmenopausal ages. Although the exact aetiology is still unknown, there is a strong association between LS and autoimmune upsets such as ; alopecia areata, vitiligo, thyroid upsets and diabetes mellitus ( 2 ) . Besides the autoimmune diseases in association with HLA category II antigens, hormonal factors, infective causes, and familial influence have besides been associated with LS ( 1 ) .
Authoritative LS is characterized by pronounced redness, epithelial cutting and typical cuticular alterations with an visual aspect of all right “ coffin nail paper ” -like pursing accompanied by symptoms of pruritus, dyspareunia and dysuria ( 3,4 ) . A considerable figure of patients ( 33 % ) are symptomless but have the clinical marks of LS on physical scrutiny. The diagnosing of LS is based upon the presence of characteristic clinical manifestations, ideally with histological verification obtained from a vulvar clout biopsy. A hold in the diagnosing may originate, due to the patients ‘ embarrassment and/or reluctance of the doctor to to the full measure the symptoms every bit good as strangeness with the disease ( 5 ) .
The optimum direction of LS could be achieved with a multidisciplinary attack. Surgical intervention is preferred in the instance of a post-inflammatory sequelae, phimosis or in a malignant province. Although the presence of vulval cicatrixs or the features of the defect will restrict our surgical preferance there are many surgical options proposed for the perineal Reconstruction, including local musculus and fasciocutaneous tegument flaps ( 6,7,8 ) . We are depicting a persistant lichen induration instance who was treated surgically and recontructed with V-Y promotion vulvar flaps.
A 49 twelvemonth old Caucasian adult female married for 30 old ages G3, P2, A1 enduring from vulvadynia because of relentless vulvar LS for 16 old ages, admitted to our clinic. She has been having medicine with carbamezepine for ungratified leg syndrome for six old ages. She had two cesarean subdivisions and three lumbar phonograph record hernia operations in which an internal fixator was placed to the lumbar vertebras.
She used to hold local cortisol, estrogen and testosterone therapies since she received her initial diagnosing. She has had vulvar alcohol injection one time and vulvar betametasone, lidocain injections and phototherapy several times. Her history revealed a vulval denervation operation to the pudental nervus with the diagnosing of vulvadynia. Furthermore she had three times simple vulvectomy and vulval Reconstruction operations. These intervention modes failed to relieve her symptoms, and sclerosing alterations of vulvar country which extends to posterior, affecting the perineum and anus.
The singular findings in her pelvic scrutiny were the hyperkerotic lichen plaques spread to anus, vaginal orifis and periclitoral country ( Figure 1a ) . We observed the vaginal orifis as narrow shaped and labia majora – minora were absent. Hyperkeratotic scars were noticable on her mons pubis due to her old surgical operations and lichen plaques. In her speculum scrutiny, vagina and neck were normal in form and uterus – bilateral ovaries were normal by transvaginal ultrasound and no malignant cells were reported in her pablum smear trial. Histopathological diagnosing of the topic was confirmed with two antecedently performed consecutive vulvar biopsies that revealed characteristic cuticular wasting and cuticular hyalinisation ( homogenisation of the collagen in the upper corium ) .
Our patient had relentless and worsening symptoms and we decided to execute simple vulvectomy and V-Y promotion flap for the Reconstruction of vulvar defect ( Figure 1a ) and obtained informed consent consequently. Briefly ; urinary catheterisation was needed to forestall urethral hurt and to supply plenty wound hygiene after surgery. After vulvectomy ( Figure 1b ) , we performed V form scratchs bilaterally widening to median part of the thigh without any complication ( Figure 1c ) . The scratchs were profoundly performed until the superficial genital facia to travel the flaps freely and punctilious haemostasis was done. The mobility of the cutaneal flaps are tested. Following the release of the flap, this extra block of hypodermic tissue is folded into the defect. The sidelong transverse defect which was formed when the island flap moved medially is closed horizontally, therefore giving the concluding cicatrix a horizontal “ Y ” form. We aproximated the hypodermic tissue with 2/0 polyglactin 910 [ Ethicon, Edinburgh, UK ] . The tegument was closed by interrupted 3/0 polyglactin 910 [ Ethicon, Edinburgh, UK ] suturas ( Figure 1d ) . We repaired the round defect above the vaginal orifis with free rotary motion flap. No drains were placed. Postoperative closing of vulvar defect was seen perfect in form. She was hospitalized for the undermentioned two yearss ( Figure 2a ) and uretral catheterisation was removed on her 2nd twenty-four hours. All the critical marks of the patient were usual without any wound infection or haematoma in the closing. In the early postoperative period, the healing of vulva was uneventful and completed after three hebdomads ( Figure 2b ) .
A assortment of names and descriptions have been used for the disease that is presently named lichen sclerosus. In 1887, Hallopeau was the first to depict the histological characteristics of the disease ( 9 ) . LS occurs at all ages but non in newborns and is rare in the first twelvemonth of life. The disease has a bimodal extremum incidence in prepubescent misss and menopausal adult females. In a survey the prevalence of lichen sclerosus in childhood was found to be 1:900. The bulk of patients consists of adult females aged between 50-70 old ages, while 5-15 % of the LS topics were kids.
Lifelong surveillance of all adult females with LS is considered indispensable because of the hazard of malignant patterned advance, ( 10 ) . Although adult females with vulval LS are at increased hazard of developing invasive squamous cell malignant neoplastic disease ( SCC ) of the vulva ( 4 ) , the estimated hazard is to be less than 5 % ( 11 ) . No grounds exists that regular check-ups cut down the hazard of malignant patterned advance. Nevertheless, patients with vulval LS should be examined at least annually and localized, relentless, nonresolving lesions should be biopsied ( 4 ) . In contrast to vulvar LS extragenital LS lesions are non associated with an increased hazard of malignance ( 9,12 ) .
There is non a universally recognized direction scheme for adult females with LS. It normally provides diagnostic alleviation, without needfully rectifying the implicit in upset. The intervention of LS consists of instruction and support, behavioural alteration to keep good perineal hygiene and turning away of any local thorns, medicine and surgery. Assorted conservative curative options such as, topical testosterone, topical Lipo-Lutin, topical corticoids, estrogens, retinoic acid, retinoids, vitamin A, chloroquine and a short class of systemic corticoids are wholly accepted as intervention options ( 13 ) .
All published studies on the direction of LS indicate centrist to strong topical steroids as the intervention option of pick ( 12,14,15 ) . Mild to chair authority topical corticoids are besides normally used for intervention of grownup vulvar LS ( 3 ) . Recently, ace potent topical steroids were found to be an effectual intervention for this job with both short- ( 16 ) and long-run ( 17 ) efficaciousness. Topical steroid therapy is non without complications, including the possibility of wasting, contact sensitisation, tegument alterations, and secondary infection.
Although there are no good studied therapies in adult females who failed to react to corticoids, Lipo-Lutin, testosterone, retinoids, topical immune system modulating agents ( tacrolismus, pimecrolimus ) and cyclosporine has been used in some randomised tests ( 4 ) . Small randomized tests showed that Lipo-Lutin and testosterone picks are less effectual than corticoids ( clobetasol ) with more side effects ( 18 ) . Treatment with an unwritten retinoid ( acitretin ) was found to be effectual in one randomised test ( 19 ) . Successful interventions with tacrolimus oinment was reported ( 20 ) but frequently discontinued due to Burnss on application sites. As presented in our instance furnace lining instances have been treated with intradermic intoxicant injections with variable consequences ( 21 ) . Another concern must be the physicological jobs that may look ( i.e. narcotic maltreatment ) in relentless LS topics due to chronic vulvar hurting. The American College of Obstetricians and Gynaecologists suggests one-year scrutinies for patients whose LS is good controlled and more frequent visits for those with ill controlled disease ( 15 ) .
Surgery does non hold a cardinal function in the intervention scheme of LS and could be considered as a last resort to handle complications secondary to the LS ( 22,23 ) . Surgical intercession in LS should non be aimed at taking the disease but at deciding complications of the disease: to let go of a inhumed button, to divide fused labia, or to widen a narrowed introitus in instance of ailments about clitoric symptoms ( hurting or sexual clitoric disfunction ) ( 22 ) . Its usage is limited since scars and contractures may look after surgery. However, if surgery is the preferable intervention mode, it is of import to cognize how to retrace the vulva. Patients wish to go on their sexual life every bit shortly as possible. V-Y promotion flap is an effectual method for Reconstruction of the perineal part. This technique will supply better blood supply and nervus esthesis and will let the expanding of the vaginal orifis.
The principle behind the surgical therapy is chiefly to handle those patients who did non or react ill to medical intervention and secondly to forestall the development of invasive carcinoma of the vulva ( 23 ) . Our patient had a long medical history about LS in which she had received several local therapies, phototherapy, surgical efforts and systemic medicines for 16 old ages. All intervention modes were discussed with the household and the concluding determination of the patient was surgery due to her exhaustion and better decorative outlook.
Consequently, surgical intercession must be deferred until LS has been controlled with medicine or should be reserved for pull offing postinflammatory sequelae. If an excisional process ( i.e. vulvectomy ) is the preferable intervention mode, Reconstruction of the vulvar defect with V-Y promotion flap seems to be an applicable and extremely effectual surgical technique with its good decorative consequences and rapid healing after surgery.
Figure 1: Pre and intraoperative images of vulvar lichen sclerosus patient. 1a ; hyperkerotic lichen plaques spread to anus, vaginal orifis and periclitoral country, 1b ; vulvectomy was performed, 1c ; V form scratchs bilaterally widening to median part of the thigh, 1d. closing of the tegument, giving the concluding cicatrix a horizontal “ Y ” form.
Figure 2: Postoperative images of vulvar lichen sclerosus patient. 2a ; station operative 2nd twenty-four hours, 2b ; completed vulvar mending three hebdomads subsequently.