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Download Vulvar Lichen Sclerosus - MD Anderson Cancer Center
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Vulvar Lichen Sclerosus Deepthi A James MSN, RN, FNP-C Definition & Cause Lichen sclerosus(LS) is a benign, chronic condition with marked inflammation and epithelial thinning, that creates patchy white skin, that’s thinner than normal. The cause is unknown, but the most popular theory is LS is caused by auto-immune dysfunction. Lichen Sclerosus Lichen sclerosus is a remitting and relapsing condition and there is no cure. Lichen sclerosus may affect skin on any part of the body, but mostly involves skin of the vulva, perineum and skin around the anus. Lichen sclerosus Earlier known as Lichen sclerosus et atrophicus. “et atrophicus” was dropped as areas of thickening and hyperplasia often occur. In 15 to 20 percent of patients LS can affect skin surface such as neck, breasts, shoulders, wrists, thighs, and rarely inside the mouth. Incidence Vulvar LS occur at any age but tends to have two peaks of onset: pre-pubertal girls and postmenopausal women . The true prevalence is not known; estimates range from 1 in 30 older adult women to 1 in 59 women in a general gynecology practice to 1 in 300 to 1000 patients referred to dermatologists. Etiology Unknown. Genetic factors- familial aggregations of LS among fathers and daughters, mothers and daughters, sisters and twins are reported Etiology Local factors- skin graft placed at vulva developed LS and skin with LS from the vulva became normal when grafted to thigh, suggesting local vulvar factors facilitate disease expression. Immunologic abnormality- Immune system disorders are more common among patients with LS, suggests autoimmune mechanisms may be involved in the etiology . Etiology Hormonal factors- The highest incidence of LS in women is observed during low-estrogen physiologic states, such as the pre pubertal child and the postmenopausal woman, suggesting a hormonal influence on the pathogenesis of the disease. Infection: Infectious agents such as Borrelia burgadoferi- acid-fast bacteria, human papillomavirus [HPV]) have been hypothesized to induce LS, but no clear relationship. Symptoms of LS Vulvar pruritus ( hall mark of disease) Dyspareunia Difficult orgasm Dysuria Other Anal forms of sexual dysfunction discomfort Risk factors for Vulvar Cancer Two pathways vulvar dystrophy (eg, lichen sclerosus), human papillomavirus (HPV) infection, vulvar or cervical intraepithelial neoplasia Signs of LS White plaques are usually seen. Hemorrhagic, purpuric, hyperkeratotic bullous, eroded and ulcerated. Lesions affect labia majora and extend over the perineum and around anus. Extension to Fissures genital folds or buttocks seen at posterior fourchette, interlabial folds or around the clitoris. Signs of LS Scratching results in excoriation and thickening of the epidermis lining. As disease progress distortion of vulvar architecture occurs. Distinction between labia majora and minora is lost and clitoris gets buried under the fused prepuce. Shrinkage of the introitus and perineum causes fissuring upon insertion of vaginal speculum. Lichen sclerosus lichen sclerosus showing the characteristic ‘figure-of-eight’ distribution: typical primary lesions are the ivory-white, flat, polygonal papules, giving lichen sclerosus the name ‘white-spotdisease’ in the past. Am J Clin Dermatol. 2013 February;14(1):27-47. Lichen sclerosus A 27-year-old woman with lichen sclerosus affecting the vulva and perineum: perineal pallor, sclerosis, and sagittal fissure Am J Clin Dermatol. 2013 February;14(1):27-47. Diagnosis Based on clinical manifestations Ideally with histologic confirmation Biopsy- A 4 mm vulvar punch biopsy is sufficient. Lab tests- related to auto immune disease. Differential diagnosis Lichen planus Lichen simplex chronicus Endogenous and exogenous dermatitis Vitiligo Mucous membrane pemphigoid Differential Diagnosis Anal fissures and hemorrhoids Candidiasis Psoriasis Estrogen deficiency Associated malignancy/vulvar squamous cell carcinoma Medical Management Topical corticosteroids are the mainstay of therapy Clobetasol propionate, has long been considered the standard of care for vulvar LS. Treatment Regimen Initial treatment- is application of corticosteroid for 6 to 12 weeks. Once-nightly regimens & tapering regimens can successfully treat LS Tapering regimen is application once at night x 4 weeks, then every other night x 4weeks, then twice weekly x 4weeks. Ointment is preferred over cream, as cream formulations are more irritating. Administration The ointment is applied and spread as a film over the affected area. Use of 1 Fingertip Unit (FTU) per application; 1 FTU is the amount of ointment expressed from a tube with a 5 mm nozzle, applied from the distal skin crease of the index finger to the tip (approximately 0.5 g). In the clinic, using a large mirror or using clinical photographs, teach patients exactly where to apply the ointment. A 30 g tube of ointment is typically enough to last for the initial treatment period. Maintenance Treatment Topical corticosteroid ointment - two to three nights per week . Reevaluate after 12 weeks- to ensure disease is adequately controlled. If symptoms recur during or after tapering, the frequency of treatment is increased. Twice-weekly Mometasone furoate 0.1% ointment, for long-term maintenance. Patient Education Patient education is a key component in management of vulvar LS. Discuss -individual situation, tactful coverage of architectural changes and distortion of vulva. Discuss the chronicity of vulvar LS, expectations of frequent recurrences and remissions. Patient Education Vulvar hygiene, importance of cessation of scratching, minimize factors that may exacerbate symptoms. Discuss self-examination of the vulvar area with a mirror and fingertips on a monthly basis. and should return for evaluation if they detect thickened areas of skin or sores that do not heal. Discussion of sexual function. Reassure the disease is not contagious. A referral to a sexual counsellor to address any sexuality concerns.
