Lichen sclerosus is a lymphocyte-mediated chronic inflammatory dermatitis that most commonly involves the anogenital area. Treatment options include topical corticosteroids, tacrolismus, carbon dioxide laser ablation and finally vulvectomy as surgical approach. The objective of this study is to report a refractory anogenital LS subject and describe the use of V-Y advancement flap for the reconstruction of the perineal defect after vulvectomy.
Case: A persistent LS case who was treated succesfully with surgery is reported and literature is reviewed. The patient had well tolerated the surgical procedure with satisfactory cosmetic results.
Conclusion:
A multidisciplinary team work would be ideal in the management of persistent LS cases. Surgical treatment of LS should be reserved as a last resort for patients who were refractory to other medical options and in the case of a surgical attempt recontruction of the vulva should be well known.
Key words: Lichen sclerosus, Vulvectomy, Vulvar reconstruction.
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Introduction:
Lichen sclerosus (LS) is a benign, chronic skin disease that most commonly occures in the anogenital epithelium (1). It affects both gender and children especially women in postmenopausal ages. Although the exact aetiology is still unknown, there is a strong association between LS and autoimmune disorders such as; alopecia areata, vitiligo, thyroid disorders and diabetes mellitus (2). Besides the autoimmune diseases in association with HLA class II antigens, hormonal factors, infectious causes, and genetic influence have also been associated with LS (1).
Classic LS is characterized by marked inflammation, epithelial thinning and distinctive dermal changes with an appearance of fine "cigarette paper"-like wrinkling accompanied by symptoms of pruritus, dyspareunia and dysuria (3,4). A considerable number of patients (33%) are asymptomatic but have the clinical signs of LS on physical examination. The diagnosis of LS is based upon the presence of characteristic clinical manifestations, ideally with histological confirmation obtained from a vulvar punch biopsy. A delay in the diagnosis may arise, due to the patients' embarrassment and/or reluctance of the physician to fully evaluate the symptoms as well as unfamiliarity with the disease (5).
The optimal management of LS could be achieved with a multidisciplinary approach. Surgical treatment is preferred in the case of a post-inflammatory sequelae, phimosis or in a malignant state. Although the presence of vulvar scars or the characteristics of the defect will limit our surgical preferance there are many surgical alternatives proposed for the perineal reconstruction, including local muscle and fasciocutaneous skin flaps (6,7,8). We are describing a persistant lichen sclerosis case who was treated surgically and recontructed with V-Y advancement vulvar flaps.
Case report:
A 49 year old Caucasian woman married for 30 years G3, P2, A1 suffering from vulvadynia because of persistent vulvar LS for 16 years, admitted to our clinic. She has been receiving medication with carbamezepine for restless leg syndrome for six years. She had two caesarean sections and three lumbar disc hernia operations in which an internal fixator was placed to the lumbar vertebras.
She used to have local hydrocortisone, estrogen and testosterone therapies since she received her initial diagnosis. She has had vulvar alcohol injection once and vulvar betametasone, lidocain injections and phototherapy several times. Her history revealed a vulvar denervation operation to the pudental nerve with the diagnosis of vulvadynia. Moreover she had three times simple vulvectomy and vulvar reconstruction operations. These treatment modalities failed to alleviate her symptoms, and sclerosing changes of vulvar area which extends to posterior, involving the perineum and anus.
The remarkable findings in her pelvic examination were the hyperkerotic lichen plaques spread to anus, vaginal orifis and periclitoral area (Figure 1a). We observed the vaginal orifis as narrow shaped and labia majora - minora were absent. Hyperkeratotic cicatrices were noticable on her mons pubis due to her previous surgical operations and lichen plaques. In her speculum examination, vagina and cervix were normal in shape and uterus - bilateral ovaries were normal by transvaginal ultrasound and no malignant cells were reported in her pap smear test. Histopathological diagnosis of the subject was confirmed with two previously performed sequential vulvar biopsies that revealed characteristic epidermal atrophy and dermal hyalinization (homogenization of the collagen in the upper dermis).
Our patient had persistent and exacerbating symptoms and we decided to perform simple vulvectomy and V-Y advancement flap for the reconstruction of vulvar defect (Figure 1a) and obtained informed consent accordingly. Briefly; urinary catheterization was needed to prevent urethral injury and to provide enough wound hygiene after surgery. After vulvectomy (Figure 1b), we performed V shape incisions bilaterally extending to medial portion of the thigh without any complication (Figure 1c). The incisions were deeply performed until the superficial genital fascia to move the flaps freely and meticulous hemostasis was done. The mobility of the cutaneous flaps are tested. Following the release of the flap, this excess block of subcutaneous tissue is folded into the defect. The lateral transverse defect which was formed when the island flap moved medially is closed horizontally, thus giving the final scar a horizontal "Y" shape. We aproximated the subcutaneous tissue with 2/0 polyglactin 910 [Ethicon, Edinburgh, UK]. The skin was closed by interrupted 3/0 polyglactin 910 [Ethicon, Edinburgh, UK] sutures (Figure 1d). We repaired the circular defect above the vaginal orifis with free rotation flap. No drains were placed. Postoperative closure of vulvar defect was seen perfect in shape. She was hospitalized for the following two days (Figure 2a) and uretral catheterization was removed on her second day. All the vital signs of the patient were usual without any wound infection or hematoma in the closure. In the early postoperative period, the healing of vulva was uneventful and completed after three weeks (Figure 2b).
Discussion:
A variety of names and descriptions have been used for the disease that is currently named lichen sclerosus. In 1887, Hallopeau was the first to describe the histological features of the disease (9). LS occurs at all ages but not in neonates and is rare in the first year of life. The disease has a bimodal peak incidence in prepubertal girls and menopausal women. In a study the prevalence of lichen sclerosus in childhood was found to be 1:900. The majority of patients consists of women aged between 50-70 years, while 5-15% of the LS subjects were children.
Lifelong surveillance of all women with LS is considered essential because of the risk of malignant progression, (10). Although women with vulvar LS are at increased risk of developing invasive squamous cell cancer (SCC) of the vulva (4), the estimated risk is to be less than 5% (11). No evidence exists that regular check-ups reduce the risk of malignant progression. Nevertheless, patients with vulvar LS should be examined at least yearly and localized, persistent, nonresolving lesions should be biopsied (4). In contrast to vulvar LS extragenital LS lesions are not associated with an increased risk of malignancy (9,12).
There is not a universally accepted management strategy for women with LS. It usually provides symptomatic relief, without necessarily correcting the underlying disorder. The treatment of LS consists of education and support, behavioral modification to maintain good perineal hygiene and avoidance of any local irritants, medication and surgery. Various conservative therapeutic options such as, topical testosterone, topical progesterone, topical corticosteroids, estrogens, retinoic acid, retinoids, vitamin A, chloroquine and a short course of systemic corticosteroids are all accepted as treatment options (13).
All published reports on the management of LS indicate moderate to strong topical steroids as the treatment option of choice (12,14,15). Mild to moderate potency topical corticosteroids are also commonly used for treatment of adult vulvar LS (3). Lately, super potent topical steroids were found to be an effective treatment for this problem with both short- (16) and long-term (17) efficacy. Topical steroid therapy is not without complications, including the possibility of atrophy, contact sensitization, skin changes, and secondary infection.
Although there are no well studied therapies in women who failed to respond to corticosteroids, progesterone, testosterone, retinoids, topical immune system modulating agents (tacrolismus, pimecrolimus) and cyclosporine has been used in some randomized trials (4). Small randomized trials showed that progesterone and testosterone creams are less effective than corticosteroids (clobetasol) with more side effects (18). Treatment with an oral retinoid (acitretin) was found to be effective in one randomized trial (19). Successful treatments with tacrolimus oinment was reported (20) but often discontinued due to burns on application sites. As presented in our case refractory cases have been treated with intradermal alcohol injections with variable results (21). Another concern must be the physicological problems that may appear (i.e. narcotic abuse) in persistent LS subjects due to chronic vulvar pain. The American College of Obstetricians and Gynaecologists suggests annual examinations for patients whose LS is well controlled and more frequent visits for those with poorly controlled disease (15).
Surgery does not have a key role in the treatment strategy of LS and could be considered as a last resort to treat complications secondary to the LS (22,23). Surgical intervention in LS should not be aimed at removing the disease but at resolving complications of the disease: to release a buried clitoris, to separate fused labia, or to widen a narrowed introitus in case of complaints about clitoral symptoms (pain or sexual clitoral dysfunction) (22). Its use is limited since cicatrices and contractures may appear after surgery. However, if surgery is the preferred treatment modality, it is important to know how to reconstruct the vulva. Patients wish to continue their sexual life as soon as possible. V-Y advancement flap is an effective method for reconstruction of the perineal region. This technique will provide better blood supply and nerve sensation and will allow the expanding of the vaginal orifis.
The rationale behind the surgical therapy is primarily to treat those patients who did not or respond poorly to medical treatment and secondly to prevent 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 attempts and systemic medications for 16 years. All treatment modalities were discussed with the family and the final decision of the patient was surgery due to her exhaustion and better cosmetic expectation.
Consequently, surgical intervention must be deferred until LS has been controlled with medication or should be reserved for managing postinflammatory sequelae. If an excisional procedure (i.e. vulvectomy) is the preferred treatment modality, reconstruction of the vulvar defect with V-Y advancement flap seems to be an applicable and highly effective surgical technique with its good cosmetic results and rapid healing after surgery.
Legends
Figure 1: Pre and intraoperative pictures of vulvar lichen sclerosus patient. 1a; hyperkerotic lichen plaques spread to anus, vaginal orifis and periclitoral area, 1b; vulvectomy was performed, 1c; V shape incisions bilaterally extending to medial portion of the thigh, 1d. closure of the skin, giving the final scar a horizontal "Y" shape.
Figure 2: Postoperative pictures of vulvar lichen sclerosus patient. 2a; post operative second day, 2b; completed vulvar healing three weeks later.
