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.
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).


CONCLUSIONS
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.
Setterfield
Jane F
JF
St John's Institute of Dermatology, St Thomas' Hospital, London SE1 7EH, UK.
Neill
Sallie
S
Shirlaw
Penelope J
PJ
Theron
Janice
J
Vaughan
Robert
R
Escudier
Michael
M
Challacombe
Stephen J
SJ
Black
Martin M
MM
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000483:Alleles; D005260:Female; D005882:Gingival Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D006683:HLA-DQ Antigens / Q000276:immunology*; D059866:HLA-DQ beta-Chains; D006801:Humans; D008010:Lichen Planus / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D008875:Middle Aged; D012720:Severity of Illness Index; D013577:Syndrome; D014623:Vaginal Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D014845:Vulvar Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*
D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen