Vulvar Lichen Planus (VLP)
Vulvar lichen planus (VLP) is a chronic inflammatory condition of the skin and mucosa, characterized by destruction of basal cells. The cause of this disease remains unknown, although it is potentially related to T cell-mediated immune dysregulation of the skin and mucosa. VLP can present in three forms:
- Erosive Type: The most common form, characterized by erosions at the mucocutaneous junction of the vestibule and vaginal introitus. Some patients may also exhibit oral mucosal involvement.
- Papulosquamous Type: Presents as gray-white, pink, or purple flat-topped papules or plaques.
- Hypertrophic Type: The least common form, which mainly appears as white or pink verrucous or thickened plaques on the labia majora and perineum.
VLP can co-occur with conditions such as AIDS, malignancies, liver cirrhosis, peptic ulcer disease, hepatitis B or C, and ulcerative colitis. The disease primarily affects peri- or postmenopausal women, with almost no cases in younger patients. Common symptoms include pain and burning sensations, though some patients may experience pruritus or remain asymptomatic. Diagnosis is confirmed through histopathological examination. In rare cases, spontaneous remission can occur.
Initial treatment involves the use of high-potency topical corticosteroids (TCs), applied twice daily. As the condition improves, the medication dosage is gradually tapered, and low-dose maintenance therapy may be used for long periods. If first-line treatment fails, second-line therapies may be considered. In advanced stages or when treatment is delayed, complications such as labial or clitoral hood adhesions and vaginal stenosis may develop, requiring surgical intervention. Erosive and hypertrophic types carry a higher risk of malignancy and warrant long-term follow-up for early detection and prompt management.
Behcet’s Disease
Behcet's disease, also known as oculo-oral-genital syndrome or Behcet syndrome, is a chronic, relapsing, systemic disorder. It is marked by recurrent oral ulcers, genital ulcers, ocular inflammation, and other skin lesions, with potential involvement of the cardiovascular system, joints, and even the central nervous system. The etiology remains unclear, although the fundamental pathological change is systemic vasculitis driven by an autoimmune inflammatory response.
Behcet's disease predominantly affects young women aged 20–40 years. It often begins with oral ulcers, followed by genital ulcers, and later ocular involvement. The ulcers range from solitary lesions to multiple ones with well-defined borders, and upon healing, scarring may occur. During the initial outbreaks, localized pain is pronounced. Systemic symptoms such as fever, fatigue, and headache may accompany acute episodes.
Ocular involvement may initially present as conjunctivitis or retinitis but can progress in later stages to hypopyon, optic atrophy, and even blindness. Diagnosis is based on the presence of two major symptoms or their recurrence in combination with other systemic manifestations. A positive skin pathergy test may aid in confirming the diagnosis. Laboratory findings during acute phases may show moderate leukocytosis and elevated erythrocyte sedimentation rate; however, pathological examination of ulcers reveals no specific findings.
Treatment is primarily symptomatic. For severe ulcer pain, sedatives or local anesthetics are used for pain relief. During acute episodes, corticosteroids are employed to facilitate ulcer healing. Long-term low-dose corticosteroids can be used for preventing recurrence.
Vulvar Vitiligo
Vulvar vitiligo is a condition caused by melanocyte destruction. The underlying cause is unclear, but it may be related to autoimmune processes. The disease manifests as distinct, variably sized white macules or patches that are single or multiple with well-defined borders. The affected skin appears smooth, moist, and retains normal elasticity. Vitiligo lesions may also be present on other parts of the body.
Most patients remain asymptomatic, and treatment is generally unnecessary unless associated inflammatory conditions require management.
Secondary Hypopigmentary Disorders
Secondary hypopigmentation may accompany various chronic vulvar conditions, including diabetic vulvitis, vulvovaginal candidiasis, vulvar abrasions, or vulvar condyloma. Many patients experience associated symptoms such as pruritus, burning, or pain. Upon examination, excessive keratinization of the vulvar epidermis is commonly observed, with the keratinized surface appearing white and scaly. Clinically, these conditions are often misdiagnosed as vulvar simplex lichen. Following resolution of the primary disease, the hypopigmented areas typically resolve as well. Applying an oily substance to the desquamated areas may reduce the appearance of whiteness, aiding in differential diagnosis.
Treatment should target the underlying primary condition. Personal hygiene is important, including keeping the vulva clean and dry. Frequent use of soap, cleansers, or medicated solutions on the vulva should be avoided.
Complications and Importance of Early Management
When hypopigmentary disorders, including various lichen-like conditions, are not diagnosed or treated in a timely and standardized manner, complications such as vulvar atrophy, adhesions, scarring, or malignant transformation may occur. These severe complications can profoundly impact a patient’s quality of life. Early diagnosis, prompt treatment, and long-term management are thus critical.