Lichen planus van de vulva - huidziekte en behandeling

CLINICAL FEATURES

Vulval/vulvovaginal lichen planus

Vulval/vulvovaginal LP most commonly affects women in their late 50s or early 60s.[24] The affected group is mostly postmenopausal, and the disease has not been reported before puberty.[25]

The clinical forms of LP on the female genitalia are mainly erosive, papulosquamous, and rarely hypertrophic.[26] The presentation varies from subtle fine white interlacing linear papules/plaque in its mildest form to severe erosive disease leading to scarring. The classical cutaneous LP lesions are uncommon in the genital region. The hair-bearing areas (mons pubis and inner thighs) are uncommon sites for LP. On the labia majora, lesions may appear dusky red or reddish-brown instead of the typical violaceous colour of cutaneous LP. Occasionally, the hyperkeratotic variant exists as firm white papules/plaques with irregular borders and thickened irregular surface [ Figure 1 ].

Violaceous-whitish plaque with interspersed erosions

Erosive disease is the most significant and distressing form of Vulval LP. This most commonly involves the posterior vestibule and often extends anteriorly to the labia minora [ Figure 2 ].[25] Dusky erythema or erosions, surrounded by a typical white lacy border, are visualized in some cases interspersed with whitish areas. Severe long-standing erosive disease can cause loss of normal architecture with fusion of the labia minora, loss of interlabial sulcii, and burying of the clitoris. The erosive form is often associated with inflammatory vaginitis[25] which produces symptoms of vaginal discharge, pain, and burning during micturition. A per-vaginal examination may be extremely painful, and bleeding on manipulation/instrumentation can occur. Vaginal lesions have been variably reported to be present in 20%–58% cases of Vulval LP.[27,28] Adhesions often develop in long-standing disease andnarrowing/shortening or even total obliteration of the vagina is often the final outcome [ Figure 3 ].

Introduction

Erosive lichen planus (ELP) is a variant of lichen planus which involves chronic and painful ulceration of the skin and mucosal surfaces. ELP is thought to be the result of autoimmune damage of the basal cell layer, which is mediated by activated CD8 T lymphocytes.[1] Occasionally, ELP may present in conjunction with other clinical forms of lichen planus or may be induced by drug exposure. Clinical findings include painful, persistent ulcers, primarily of the mouth and genitals.[2] Complications of ELP include secondary infection, development of squamous cell carcinoma, and scarring.

Lichen planus (LP) is thought to be a T-cell mediated auto-immune inflammatory disorder, which destroys basal epithelial cells.[1] CD8 T cells are considered primary offending cells based on biopsy specimens. It is believed that the presentation of exogenous antigens, such as viruses, metals, or drugs, may lead to CD8 activation and destruction of keratinocytes.[3] LP has also been associated with other auto-immune diseases, including vitiligo, auto-immune thyroid disease, and alopecia areata.[4]

PATHOGENESIS

T-helper and T-cytotoxic lymphocytes, natural killer cells, and dendritic cells are the main inflammatory cells involved. Activated cytotoxic T-cell infiltrate into the epithelium, leading to the apoptosis of basal keratinocytes.[14]

Antibasement membrane zone antibodies chiefly targeting BP180 have been reported to be present in sera of about 61% of patients with erosive vulval LP, suggesting that autoimmune mechanisms may be important in its pathogenesis.[15] It is, however, hypothesized though that these antibodies may have a transient or amplifying role in the pathogenesis but are unlikely to be primarily pathogenic.[16] Similar findings have been reported in LSeA as well.[17] Cooper et al. demonstrated circulating autoantibodies in 40% women with erosive vulval LP compared with 20% of controls (P < 0.001). Antinuclear (25%) and antithyroid (19%) antibodies were the most frequent antibodies detected.[16] Further, about 31% patients reported a first-degree relatives with one or more autoimmune disorders.

An association of OLP with hepatitis C virus (HCV) infection has been reported from some geographical locations. The reported prevalence of HCV antibodies in various studies varies from 0% to 62% with a high prevalence in Italian (29%) and Japanese (62%) patients and a very low prevalence reported from the USA and Northern European nations.[9,18,19,20] However, the two studies done so far to explore the association of vulval LP with HCV (both from the UK) have been negative.[21,22] More data from other regions may present a different picture though.

Is there a cure for lichen planus?

There’s no cure for lichen planus, but medications that treat the symptoms can be helpful, and some may even be able to target a possible underlying cause. Medications often prescribed include:

There are a few things you can try at home to complement your prescription treatments. The American Academy of Dermatology recommends these methods for skin lichen planus:

• soak in an oatmeal bath
• avoid scratching
• apply cool compresses to a rash
• use OTC anti-itch creams

You might want to try different home treatments and self-care strategies for lichen planus on other parts of the body as well:

Oral lichen planus

• avoid eating acidic, spicy, or sharp foods
• avoid alcohol and mouthwash that contains alcohol
• use a prescribed lidocaine solution (that’s swished in the mouth) for pain relief
• eat soft foods if your mouth is sore
• keep up with regular dental visits

Genital lichen planus

• avoid soap and wash with plain water
• use a skin softener, such as petroleum jelly before and after urination
• apply an ice pack wrapped in a towel to soothe itching and swelling
• avoid tight clothing

Talk with your doctor before adding OTC products to your treatment plan. This way you’ll be certain that nothing you might take will interact with the prescription medications you’re taking.

Lichen planus can be difficult to treat if it develops on your vagina or vulva. This can lead to pain, scarring, and discomfort during sex.

The condition can also cause discoloration of the skin, wherever it appears on the body.

Developing lichen planus can also increase your risk of squamous cell carcinoma. This is particularly the case if you have the erosive form of lichen planus. The increase in risk is small, but you should talk with your doctor for routine skin cancer examinations.

Skin pigmentation

After the lichen planus rash has cleared up, the affected area of skin may be a different colour to what it was originally. There may be a brown or grey mark, which can sometimes last for months. This is known as post-inflammatory hyperpigmentation, and tends to be more noticeable in people with darker skin.

Erosive lichen planus is a long-lasting (chronic) form of lichen planus that causes painful ulcers to develop, as well as burning and discomfort in the genital areas of both male and females.

Occasionally, in around 2% of cases, long-term cases of erosive lichen planus can develop into certain types of cancer, such as:

You should also visit your dentist regularly to ensure that your teeth and gums remain healthy, and that any ulcers or patches that form in your mouth are examined and treated promptly. Depending on individual circumstances, dental check-ups for adults are recommended anywhere between every 3 to 24 months.

Read more about treating lichen planus, including self-help tips for managing all types of the condition.