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  • Title: 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.
    Author: Setterfield JF, Neill S, Shirlaw PJ, Theron J, Vaughan R, Escudier M, Challacombe SJ, Black MM.
    Journal: J Am Acad Dermatol; 2006 Jul; 55(1):98-113. PubMed ID: 16781300.
    Abstract:
    BACKGROUND: The vulvovaginal gingival syndrome is an uncommon and severe variant of lichen planus characterized by erosions or desquamation of vulval, vaginal, and gingival mucosae with a predilection for scarring and stricture formation. OBJECTIVE: We sought to define the clinical, immunopathologic, and human leukocyte antigen findings in a large cohort of patients. METHODS: The clinical presentation and outcome during long-term follow-up were documented in 40 patients. In addition, human leukocyte antigen typing for class II by polymerase chain reaction and sequence-specific primers was performed. RESULTS: During a mean follow-up period of 8.7 (SD +/- 6.8) years, long-term sequelae included strictures of the esophagus, lachrymal ducts, and external auditory canal; loss of vulval architecture; vaginal stenosis; and buccal mucosal fibrosis. The DQB1( *)0201 allele was present in 80% of patients versus 41.8% of control subjects (P <or= .0042; relative risk 3.71). CONCLUSION: Early diagnosis of this distinct subgroup of lichen planus, combined with appropriate therapeutic measures, may help to minimize the significant physical and psychologic morbidity associated with this scarring disorder.
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