T cells reactive with the NC16A domain of BP180 are present in vulval lichen sclerosus and lichen planus

Abstract

We have demonstrated that in > 40% of our vulval LS and LP patients, the NC16A domain of BP180 is a target for circulating T cells, and in vulval LS and LP there are associated autoantibodies to BP180.

Desmoglein 3–specific CD4+ T cells induce pemphigus vulgaris and interface dermatitis in mice

“…Despite the long-standing notion that these lymphocytes might recognize skin-associated autoantigens, strong evidence for autoimmunity and putative autoantigens is lacking. Interestingly, however, in LS and LP, both of which exhibit interface dermatitis, autoantibodies against extracellular matrix protein 1 (28) and type XVII collagen (29) have been reported, suggesting the existence of autoimmunity in these patients, further implying the possibility that skininfiltrating T cells might also recognize the same autoantigen. CD8 + T cell lines established from the lesional skin of LP patients exerted cytotoxic activity against immortalized autologous keratinocytes, supporting the involvement of skin-targeted autoimmunity in the development of interface dermatitis (30).…”

Systematic review and meta-analysis of randomized controlled trials on topical interventions for genital lichen sclerosus

“…5 In addition, there is evidence of both autoantibody and T-cell reactivity to basement membrane proteins. 6,7 The high incidence of LS in postmenopausal women suggests a pathogenic role of reduced estrogen levels; however, a protective effect from estrogens, ie, women before menopause will not develop LS, has not been observed. 2,8 In men, a cause of chronic exposure of a susceptible epithelium to urine as a result of naviculomeatal dysfunction and urinary incontinence in the uncircumcised has been proposed.…”

A case of bullous pemphigoid associated with lichen sclerosus et atrophicus

“…[234] Our review of the English literature found only three cases of BP associated with LSA. [2345] Meyrick Thomas et al .…”

“…[234] Our review of the English literature found only three cases of BP associated with LSA. [2345] Meyrick Thomas et al . [2] who investigated autoimmune-related phenomena in 350 women with histologically confirmed LSA demonstrated that 21.5% had one or more autoimmune diseases including alopecia areata (9%), vitiligo (6%), hypothyroidism (6%), and BP (1%); 42% presented with an autoantibody.…”

“…With respect to autoantibodies against BP antigens, Baldo et al . [3] reported that the noncollagenous 16A domain of BP180 was a target for circulating T-cells in over 40% of their patients with vulval lichen sclerosus or lichen planus; 4 of 19 manifested associated autoantibodies to BP180. A lichen sclerosus cohort study of 149 patients revealed elevated anti-BP antibodies in 5 (3.4%), BP180 in 4, and BP230 in one patient.…”