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.
Vulvovaginal-gingival syndromeL43.9
DefinitionThis section has been translated automatically.
EtiopathogenesisThis section has been translated automatically.
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).
ManifestationThis section has been translated automatically.
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).
TherapyThis section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- Carbonari APC et al. (2018) Esophageal lichen planus: An unusual cause of dysphagia in the elderly. Rev Assoc Med Bras 64:214-216.
- Eisen D (1994) The vulvovaginal-gingival syndrome of lichen planus. The clinical characteristics of 22 patients. Arch Dermatol 130:1379-182
- Moutasim KA et al (2008) A case of vulvovaginal gingival lichen planus in association with Good's syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 105:e57-60.
- Panagiotopoulou N et al (2010) Vulvovaginal-gingival syndrome. J Obstet Gynaecol 30:226-30.
- Olszewska M et al. (2016) Vulvovaginal-gingival lichen planus: association with lichen planopilaris and stratified epithelium-specific antinuclear antibodies. Acta Derm Venereol 96:92-96.
- Setterfield JF et al (2006) The vulvovaginal gingival syndrome: a severe subgroup of lichen planus with characteristic clinical features and a novel association with the class II HLA DQB1*0201 allele. J Am Acad Dermatol 55:98-113.
- Storr M et al (2009) Nonprogressive, long-term dysphagia in a patient with esophageal lichen planus. Clin Gastroenterol Hepatol 7:A24.
- Vasudevan B et al (2016) Vulvovaginal-gingival syndrome and esophageal involvement in lichen planus. Indian J Dermatol Venereol Leprol 82:209-211.