Vulvovaginal-gingival syndrome L43.9

Vulvovaginal-gingival syndrome, also called "vulvovaginal-gingival lichen planus", in short VVG-LP, is a distinct clinical subtype of lichen planus and consists of the following triad: vulvar, vaginal and gingival lichen planus lesions. In most patients (75%), the scalp is also affected (lichen planus follicularis). Due to the complicating late sequelae (vulvar and vaginal fibrosis, alopecia), the affected patient population requires special medical care.

An autoimmunological genesis can be assumed. In about 30% of the patients with a "vulvovagino-gingival syndrome" autoimmunological "concomitant diseases" such as diabetes mellitus, Hashimoto thyroiditis (15%), vitiligo (5%), alopecia areata (4%), celiac disease, pernicious anemia, Sjögren's syndrome, idiopathic thrombocytopenic purpura are found.

A genetic predisposition has been discussed in this group of patients: there are relationships with the HLA phenotype DQB1 (Setterfield JF et al. (2006).

The mean duration of the disease is > 10 years (3-31 years).

In a larger collective of lichen planus patients, this constellation was present in exactly 10% of cases. In 75% of these patients, the scalp was also affected (Olszewska M et al. (2016).

In addition to local therapeutic measures (see below Lichen planus mucosae), the initial weight-adapted use of azathioprine (e.g. Imurek) 1.5-2.0 mg/kg bw/day p.o. is recommended, if necessary in combination with a glucocorticoid in an initial medium dosage (e.g. prednisolone 0.5 mg/kg bw/day p.o.). Subsequently symptom-adapted lower dosage (5.0-7.5 mg/day p.o.).

Alternative: Ciclosporin A (1.0-5.0 mg/kg bw/day).

Alternative: MTX 15mg s.c./week, if necessary switch to oral therapy and lower MTX dosage (2.5mg/oral/week) in a symptom-adapted manner.

It is foreseeable that this therapy must be followed over a longer period (>6 -12 months). The patient must be instructed regarding the necessary 4-weekly laboratory checks. Clinical monitoring with regard to a mycotic overgrowth(yeast diagnostics) is important.

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