1314
An autoimmune, chronic, progressive, inflammatory dermatosis that presents with sclerosis, atrophy, and pruritus.
Known as krauosis vulvae or vulvar dystrophy when affecting female genitalia and balanitis xerotic obliterans when affecting male genitalia.
It is a chronic inflammatory, lymphocyte-mediated dermatosis.
Most commonly affects women during their 40s or 50s.
Children are only rarely involved.
Adults experience a more chronic disease process, while children have self-limited disease.
Associated with other autoimmune disease is such is alopecia areata, diabetes mellitus type one, Hashimoto’s thyroiditis, celiac disease, and pernicious anemia.
Specific T cell response and autoantibodies in the extracellular matrix 1 protein in basement membrane zone is present in approximally 75% of affected patients.
Genetic predisposition exists by the occurrence of familial cases in association with HLA-DQ7 and DRB1*12.9.
Occurs predominately in the anogenital area.
Approximately 6-15% patients have extragenital lesion presentation.
Lesions typically begin is polygonal papules that coalesce into porcelain white plaques.
Accounts for one in 300-1000 patients ref2242ed to dermatologists.
Female to male ratio 6 to 10:1.
More common in uncircumcised males.
It is suspected the moist environment in the prepuce may play a role its development.
Chronic exposure of susceptible epithelium to urine may also be responsible.
White people with greater involvement.
Women have a bimodal age onset in the prepubertal and postmenopausal years.
In males it has la bimodal onset with age peaks in young boys and then between 30 and 49 years of age.
Has been reported following genital jewelry placement, trauma and instrumentation.
Occurs with increased frequency in patients with atopy and
No unequivocal relationship has been established for infective agents but Borrelia burgdorferi, Epstein-Barr virus, humanpapillomavirus and acid-fast bacteria have been implicated.
More common in women with low estrogen status such as prepubertal girls and postmenopausal females.
Histopathology findings include epidermal atrophy, hyperkeratosis, follicular plugging, loss of rete ridges, generation of basal cells, marginalization of the collagen in the epidermis and lymphocytic infiltrate.
Not associated with pregnancy, contraceptive use, hormonal replacement therapy or hysterectomy.