Benign Vulvar Dermatoses

So back in December 2019 (episode 66), we talked about vulvovaginal itching and gave a broad differential for workup of this very common symptom. Today, we thought we might focus on a few of the commonly tested and supremely confusing vulvar dermatoses, based on the new ACOG PB 224.

A quick refresher on diagnosis and workup: 

• History and physical are supreme in helping you establish acute vs. chronic and narrowing down most likely suspects.

• Labs beyond a wet mount are usually not needed, unless suspecting immunocompromise or needing a genital culture for persistent vaginitis symptoms.

• Biopsies in general are reserved for ruling out possible premalignant or malignant areas.

Lichen Simplex Chronicus (LSC)

• Chronic, nonscarring, inflammatory condition characterized by intense itch-scratch.

  • Most commonly reported symptom is chronic or intermittent intense itch, most commonly in the evening or night.

• Very common - accounting for up to a third of vulvar clinic visits, and often is a secondary condition of other “itchy” vulvar disease like contact dermatitis. 

• Most commonly encountered in middle aged women or elderly, and in women with a history of environmental allergies, asthma, or childhood eczema.

• On exam, appearance is often erythematous, scaling, and/or lichenified (thickened/leathery) plaques, with variable degrees of excoriation due to the intense itching associated with the condition. 

  • The skin with longstanding disease is often described as “bark-like” it’s so thick! 

• Consideration can be given to identifying other diseases that may be contributing to itching, such as candidiasis, but biopsy is not generally indicated.

• Treatment is multipronged:

  • Education on stopping the itch-scratch cycle.

  • Removal of offending/worsening factors, such as contact dermatitis, excessive heat/moisture, or treatment of infection.

  • Medium or high-potency topical corticosteroid, applied once or twice daily. If started on steroid, should be seen again within 4 weeks to assess response and adjust course. 

  • Oral anti-pruritic medications.

Lichen Sclerosus

• Chronic, scarring disorder that is bimodal in age distribution -- affects most commonly the anogential skin of prepubertal girls and postmenopausal women. 

• Often asymptomatic and goes unrecognized by many clinicians.

• If symptomatic, most common presenting symptoms include itching/irritation/burning, dyspareunia, and tearing.

• On exam, the skin often has the classic “cigarette paper” appearance - thin, whitened, and crinkled. 

  • Extensive involvement from the superior vulva to the perianal tissue may create an “hourglass shape” of involvement which is classic for this disease.

  • Due to the scarring nature of the condition, the introitus may be narrowed, there may be phimosis (inability to retract skin around) of the clitoral hood, and presence of fissures. 

• Biopsy is generally warranted in postmenopausal patients - patients with LS have increased risk of vulvar squamous cell carcinoma, ranging from 2-5%. 

  • Biopsy should be undertaken in areas which appear to be high risk -- i.e., if presence of any ulcers/erosions, or hyperpigmented or hyperkeratotic areas.

• Treatment of LS is important in order to halt and prevent further scarring. Initially, a high potency corticosteroid should be used, generally clobetasol propionate 0.05% or mometasone furoate 0.1%. 

  • Dosing / application schedule is not well studied, but the PB recommends nightly for 4 weeks, every other night for 4 weeks, and then twice weekly for 4 weeks.

  • Maintenance therapy is generally needed until puberty in girls, or lifelong in older patients, and should be the most infrequent dosing that maintains resolution. 

  • For disease not responding to steroids, it is most important to ensure the diagnosis is correct! You don’t want to treat VIN/ vulvar SCC with steroids. 

    • Intralesional steroid injections or topical calcineurin inhibitors such as tacrolimus can be used for particularly resistant disease, but consider vulvar specialist referral before you are doing these.

Lichen Planus

• Multisystem scarring dermatosis affecting the skin, oral mucosa, and vulvovaginal area, likely as a consequence of a dysfunctional cell-mediated immune system. 

  • Autoimmune disorders have been seen in up to a third of patients with LP.

• Rare, with incidence in general population less than 1%. 

• Most common in perimenopausal and menopausal women. 

• Common presenting symptoms are dyspareunia, burning, soreness, itching, and vaginal discharge.

• Diagnosis is complicated as there are multiple potential presentations; However, over 70% with vulvovaginal disease will also have oral involvement, so if suspected an oral exam should also be performed. 

  • Classic (papulosquamous): white, reticular, lacy, fernlike striae.

    • Dusty pink, poorly demarcated papules may also be present.

    • Occasionally with extensive involvement can “white out” vulvar skin and make picture confusing versus lichen sclerosus.

  • Erosive deep, painful, and erythematous lesions appear in posterior vestibule extending to labia minora. Architecture is often distorted and vaginal epithelium may be completely denuded. Lesions are extremely friable. 

  • Hypertrophic is the least common presentation, though with white, thick, warty plaques. 

• Wet mount performed for diagnosis will often demonstrate an abundance of immune cells in addition to parabasal and basal epithelium, as well as increased pH (5-6)

• Biopsy may be indicated based on the presentation, and may be helpful for distinguishing from rarer diagnosis like bullous pemphigoid / pemphigus vulgaris (ask your derm friends).

• Treatment is based on expert opinion but is high-potency topical corticosteroids, generally twice daily and tapered back over time. 

  • For erosive disease, also should be treated with intravaginal steroid therapy, such as hydrocortisone suppositories, and followed by vulvar specialist. 

  • Often will also need dilator therapy along with steroids due to scarring nature of disease. 

A quick word on topical corticosteroids

• So medium and high-potency corticosteroids feature heavily in the treatment of benign vulvar dermatoses… so review PB 224 for a list of them! 

  • Ointments are the preferred treatment compared to creams, lotions, or gels, as the ointments have the least additional additives that can make skin more sensitive and also allow for highest effective dose to penetrate the skin. 

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ACOG PB 224