Inflammatory diseases
Lichen sclerosus
Currently, potent topical corticosteroids provide the best outcomes. Clobetasol propionate 0.05% ointment, applied twice daily for 1-3 months (with the dose gradually tapered) provides short-term relief and long-term control in most patients. Maintenance therapy with 1-2 applications per week may be useful. Once daily application of mometasone furoate 0.1% cream may be an option, but its efficacy compared to ultrapotent topical steroids has not been assessed in double-blind comparison trials.
Long-term sequelae of potent topical corticosteroids (eg, atrophy and thinning of skin and subcutaneous tissues) have not been clinically significant in persons with this disorder. A protective effect from malignant evolution has been suggested but not proved.
Testosterone propionate 2% in petrolatum has been used, but recent studies have shown that it is only slightly more effective than placebo and that it has many adverse effects (eg, clitoral hypertrophy, increased libido, hirsutism, voice alterations).
Encouraging results have been obtained with tretinoin cream 0.025% and systemic acitretin. Close follow-up care is recommended because of the significant risk of developing epithelial cancer.
Treatment with twice daily applications of topical calcineurin inhibitors (pimecrolimus 1% cream and tacrolimus 0.1% ointment) is also promising.
Alternative treatments include intralesional steroid injections and/or cryosurgery, focal ultrasonography, photodynamic therapy, and surgery.
Squamous cell hyperplasia
Treatment of squamous cell hyperplasia is the same as that for lichen sclerosus and is aimed at halting the itch-scratch-itch cycle. General attention to proper hygiene is suggested.
If the skin is moist or macerated, aluminum acetate 5% (Burow) solution applied 3-4 times daily for 30-60 minutes is beneficial.
Systemic antihistamines or tricyclic antidepressants, especially when taken at bedtime, may help. For lichen sclerosus, the treatment of choice is a potent corticosteroid cream. In refractory lesions, intralesional injections of triamcinolone acetonide may be an alternative.
Lichen simplex chronicus (localized neurodermatitis)
Treatment includes removal of irritants and/or allergens (if identified), followed by topical application of mild-to-highโpotency corticosteroids. Do not use high-potency topical steroids for prolonged periods because of adverse effects.
Avoid soaps and cleansing agents other than aqueous cream. Discourage excessive cleaning of the genital area; use of hot water; overheating; and wearing of synthetic, rough, and/or tight clothing.
Review all cases after lichenification has resolved because lichen simplex chronicus may be associated with underlying dermatoses (eg, Paget disease, Bowen disease).
Primary irritant dermatitis
Identification and avoidance of the offending irritants is crucial. Symptomatic relief may be obtained with cool sitz baths and an application of Burow solution.
Corticosteroid ointments may also be used for short-term treatment.
Discourage excessive cleaning with inadequate or aggressive soaps and wearing of tight and/or synthetic fabrics that may cause mechanical irritation and occlusion.
Intertrigo
Gentle cleansing is often enough to elicit considerable improvement or healing.
The use of antiseptic solutions (eg, triclosan, chlorhexidine) and/or absorbent powders may also be helpful. Sparingly applied mild topical corticosteroids (eg, hydrocortisone 1%) may be necessary to promptly relieve local symptoms.
Instruct patients to avoid tight, hot, synthetic clothing and to keep the area cool and dry in order to stop friction and prevent relapses.
Allergic dermatitis
Treatment starts with identification of the offending agent, followed by environmental control.
Oral antihistamines and short-term use of a topical corticosteroid ointment (eg, betamethasone-17-valerate 0.1%, triamcinolone acetonide 0.1%) are usually effective.
Fixed drug eruption: Identification and elimination of the offending agent is the mainstay of treatment.
Atopic dermatitis
In case of acute rash, a sitz bath or compresses with Burow solution once or twice daily are helpful.
Topical steroids, such as betamethasone-17-valerate 0.1% cream, and systemic antihistamines, such as hydroxyzine, are indicated to relieve symptoms.
Antibiotics are recommended in cases of secondary infection.
Advise patients to avoid using irritating detergents, lotions, or perfumed products, and encourage wearing of cotton undergarments.
Seborrheic dermatitis
Treatment includes Burow solution and short-term, low-potency steroid ointments.
Topical antifungals may be helpful because Pityrosporum organisms are thought to play a role in causing the disorder.
Psoriasis
Treatment is aimed at symptom relief, thereby minimizing the scratching and rubbing that stimulate cell turnover.
Instruct patients to avoid chemical or mechanical traumas, including use of irritating detergents and tight systemic clothing, in order to minimize symptoms.
The treatment of choice is calcipotriene ointment, a topical vitamin D-3 preparation that is effective without the risk of skin atrophy.
Low-potency corticosteroid ointments may be used but are seldom effective as monotherapy.
Advise patients to avoid tar preparations, which are irritating to the vulvar skin.
In severe cases, systemic antipsoriatic agents (eg, cyclosporin, acitretin, methotrexate) may be used.
Reiter disease
Methotrexate is the treatment of choice.
Systemic retinoids or cyclosporine and topical corticosteroids may provide some relief.
Lichen planus
The first-line treatment consists of topical high-potency corticosteroid ointments.
Intralesional corticosteroid injections may also be used. Short courses of systemic corticosteroids may be needed for severe symptoms or flares of the disease.
Systemic administration of cyclosporine may be curative in steroid-resistant cases. Encouraging results have been reported in the erosive form with tacrolimus 0.1% ointment, topical cyclosporine, or systemic retinoids (eg, etretinate, acitretin).
Lupus erythematosus
A team consisting of a dermatologist, rheumatologist, and internal medicine expert is needed for successful management in cases of systemic involvement.
Chronic disease may be addressed with current pharmacological treatments for discoid lupus, including high-potency steroids (eg, halobetasol, clobetasol), antimalarials (eg, chloroquine, hydroxychloroquine), and intralesional triamcinolone.
Darier disease
Tazarotene gel is a new-generation topical retinoid that provides a useful and safe alternative for patients with mild disease in whom the use of systemic retinoids or other drugs is contraindicated.
Consider systemic treatment with oral retinoids or high-dose vitamin A for severe debilitating disease.
Daily washing with triclosan solution and use of absorbent powders are recommended.
Aphthosis
Treatment is symptomatic and mainly consists of topical fluorinated corticosteroid ointments.
Recommend gentle cleansing, sitz baths, and avoidance of irritants.
Behรงet disease
Topical superpotent steroids twice a day can be prescribed for the control of local symptoms.
Extremely painful ulcers may be treated with an injection of intralesional triamcinolone.
Systemic treatment consists of prednisone, dapsone, azathioprine, methotrexate, cyclosporin, or colchicine.
Pyoderma gangrenosum
No specific treatment addresses pyoderma gangrenosum.
Sulfonamides, corticosteroids, or immunosuppressive agents, including cyclosporine, have been the most commonly used systemic agents.
Make a thorough search for the presence of associated disorders.
Crohn disease
Treatment depends on the extent of involvement of the perineal area and the associated bowel disease.
A multidisciplinary approach that includes gynecologists, dermatologists, and gastroenterologists is needed to successfully manage the condition.
Infliximab followed by maintenance therapy with methotrexate may represent an option for pyostomatitis vegetans associated with Crohn disease.
Hidradenitis suppurativa
Gentle cleansing with triclosan solution once or twice daily is recommended.
Multiple therapies, including topical and systemic antibiotics, oral contraceptives, corticosteroids, and leuprolide acetate, have been used with limited success.
The pathophysiology is similar to that of acne vulgaris; thus, treatment with oral isotretinoin has been beneficial in selected cases.
Abscess and sinus tract formation often require surgical drainage; therefore, surgery remains a mainstay in the treatment of this disorder, and wide excision of the involved areas may be necessary.
Fox-Fordyce disease
Treatment measures, including topical corticosteroids, phototherapy, and oral contraceptives, are generally of limited help.
In some cases, surgical excision may be attempted.
Plasma cell vulvitis
Treatment with topical steroids may be helpful in symptomatic patients.
In one case, excellent results were reported after 3 months of topical cyclosporine application.
Vulvar vestibulitis
Treatment is difficult and often proves to be unsatisfactory. Notably, spontaneous resolution has been reported in up to 50% of cases.
Patients with symptoms only during intercourse may benefit from topical anesthetics such as lidocaine 5% ointment. Topical steroids are often used, but they appear to be of limited benefit.
Some patients have responded to intralesional interferon alfa.
Systemic treatment with tricyclic antidepressants or gabapentin may sometimes be helpful.
Consider surgery (vestibulectomy) for selected patients who do not have significant vaginismus and who do not respond to conservative treatments after 1 year.
Blistering diseases
Familial benign chronic pemphigus (Hailey-Hailey disease)
The use of anti-inflammatory agents (eg, topical corticosteroids, dapsone, thalidomide) or immunomodulatory agents (eg, methotrexate, topical or systemic cyclosporine) has been tried with some success.
In most cases, topical steroids are helpful for providing symptomatic relief with lesions of limited extension.
Advise patients to minimize mechanical stress to the skin (eg, avoiding tight clothes), especially in warm and humid environments, which tends to trigger the disease.
Bacterial, yeast, or viral infections are also considered trigger factors, and treatment with antibiotics (eg, tetracycline, erythromycin, mupirocin) or antifungals (eg, nystatin) often yields good results.
Bullous pemphigoid: Topical superpotent steroids or systemic treatment with prednisone or prednisolone, dapsone, tetracycline, or cyclophosphamide may be used.
Cicatricial pemphigoid: Treatment is the same as that for bullous pemphigoid.
Pemphigus vulgaris
Before corticosteroids became available, the mortality rate associated with this disease was high because of fluid loss and superinfection.
Besides systemic treatment with corticosteroids and immunosuppressants, topical treatment with corticosteroids may be used for lesions limited to the vulva, but relapses are frequent.
Erythema multiforme (minor/major)
Identification and treatment of any underlying cause is essential.
Mild cases are self-limited and require no treatment.
Systemic corticosteroid treatment is controversial.
Epidermolysis bullosa
No effective treatment is currently available.
Avoidance of trauma is of utmost importance in order to prevent scarring.
Pigmentary changes
Acanthosis nigricans
No specific treatment is available.
If malignancy is suggested, a thorough investigation is indicated.
Some patients may improve with weight loss and a subsequent change in hormonal status.
Lentigo, lentiginosis, and benign vulvar melanosis: No treatment is needed.
Melanocytic nevus: No treatment is needed.
Postinflammatory hyperpigmentation: No specific treatment is available.
Postinflammatory hypopigmentation: No treatment is needed.
Vitiligo
Vulvar lesions are difficult to treat and are often resistant to therapy.
Treatment with topical steroids may be attempted.
In general, therapies currently used in the management of extensive vitiligo are not indicated for lesions limited to the vulva.
Benign tumors, hamartomas, and cysts
Mucous cysts: Treatment is accomplished by surgical excision.
Bartholin cyst and Skene duct cyst
No treatment is needed unless abscesses form. In this case, incision and drainage followed by oral antibiotic administration is required.
Perform marsupialization for persistent or recurrent cysts. It should not be used to treat a gland abscess.
Excision is recommended in postmenopausal women because of a higher risk of Bartholin gland carcinoma in persons in this age group.
Epidermal inclusion cyst
Incision and drainage may be required.
If infection is recurrent, the cyst can be excised with the aid of local anesthesia once acute inflammation subsides.
Seborrheic keratosis: These lesions are benign and do not require specific treatment; however, some patients request removal for cosmetic reasons.
Acrochordon: If the polyp is causing symptoms, it can be easily removed.
Fibroma, fibromyoma, and dermatofibroma: Remove these tumors for diagnostic purposes in order to exclude a rare leiomyosarcoma or sarcoma.
Lipoma: As with lipomas that occur elsewhere, these tumors do not usually require surgical excision unless they become painful or cosmetically unacceptable to the patient.
Hidradenoma: Hidradenomas are easily removed by simple excision.
Hemangioma
Because of the well-established tendency toward spontaneous regression, no therapeutic measures are required unless complications occur.
Corticosteroids and interferon, both systemic and intralesional, have been used in severe cases.
Lymphangioma
Treatment includes surgical excision, laser therapy, electrocoagulation, superficial cryotherapy, or sclerosing therapy.
Local recurrence after treatment is common.
Angiokeratoma
Treatment options include excision and cryotherapy.
No treatment is needed for asymptomatic lesions.
Pyogenic granuloma: Excision and laser destruction are the most effective ablative procedures.
Endometriosis
Individual cutaneous lesions can be excised.
With widespread disease, consider pharmacological suppression of endometrial function (danazol).
Heterotopic sebaceous glands and sebaceous gland hyperplasia
No treatment is required except a discussion with the patient regarding the benign nature of the lesions.
Excise or ignore sebaceus gland hyperplasia. Very small lesions can be treated with cryotherapy.
Papillomatosis (papillary vulvar hirsutism)
No treatment is needed.
Reassure anxious patients that pearly papules are not a disease but, rather, a normal anatomic structure.
Congenital malformations
Ambiguous external genitalia: Plastic surgery and/or hormonal treatments may be attempted following identification of the underlying disorder.
Congenital labial hypertrophy
No treatment is needed if the patient has no symptoms.
Surgical reduction may be considered when the condition is problematic.
Labial adhesions
A topical conjugated estrogen cream applied in a thin layer twice a day for 2-3 weeks, along with gentle traction, often leads to separation.
Long-term application of a bland ointment after a daily warm water soak and gentle traction is recommended to reduce the risk of recurrence.
Manual separation with the patient under some form of anesthesia may be necessary.
Atrophy of the vulva
This condition may be treated with emollients, topical estrogens, or both.