Autoreactivity to bullous pemphigoid 180: is this the link between subepidermal blistering diseases and oral lichen planus?

Abstract

Subepidermal blistering diseases are antibody-mediated diseases. The antigens differ between disease type, but bullous pemphigoid (BP)180 (collagen XVII) is a common finding in several clinical conditions. We report four patients with autoimmune blistering disease [linear Ig A disease (n=2), bullous pemphigoid (n=1), and epidermolysis bullosa acquisita (n=1)], all of whom also developed oral lichen planus (LP). In all cases, the diagnosis of the blistering disease predated the development of the oral lesions. All patients were under the care of dermatologists and of oral medicine/surgery doctors for the diagnosis and management of the oral LP. All had circulating antibodies to BP180 protein. To our knowledge, the association between blistering diseases and oral LP has not been reported previously, and may reflect autoimmunity to BP180.

Epithelial antigenic specificities of circulating autoantibodies in mucosal lichen planus

“…Furthermore, oral LP has been reported to appear in some patients subsequent to blistering skin diseases with autoimmunity to BP180 . In these instances, it has been suggested that anti‐BP180 autoantibodies may induce a disruption of the basement membrane that would release other proteins and expose them to a cell‐mediated immune attack, which represents the pathological hallmark of LP.…”

Lichen Planus Pemphigoides: From Lichenoid Inflammation to Autoantibody-Mediated Blistering

“…In contrast to LPP, the skin changes seen in bullous LP develop in the absence of autoantibodies against structural proteins of the skin, especially COL17 (9). In terms of pathogenesis, given that in the majority of cases of LPP the development of lichenoid skin lesions precedes the formation of blisters, it has been hypothesized that lichenoid inflammation itself may actually promote the development of an autoimmune response, targeting proteins of the epidermal basement membrane (10–14).…”

Composition of hemidesmosomes in basal keratinocytes of normal buccal mucosa and oral lichen planus

“…Several hemidesmosomal proteins may serve as autoantigens. Collagen α ‐1(XVII) chain‐reactive T cells have been found in dermal lichen planus, and autoantibodies towards collagen α ‐1(XVII) chain antibodies have been described in oral lichen planus (51–54). Autoantibodies have been found to be reactive with integrin‐ α 6 in oral pemphigoid, as well as with the collagen α ‐1(XVII) chain and dystonin in mucous membrane pemphigoid (55), bullous pemphigoid and in elderly patients with chronic non‐bullous pruritic disorders (56).…”