BP180 dysfunction triggers spontaneous skin inflammation in mice

Significance BP180 is a cell–cell matrix adhesion molecule and is critical for attachment of epidermis to underlying dermis. We generated a dysfunctional BP180 mouse strain (ΔNC16A mice). The ΔNC16A mice at 8 weeks start developing spontaneous itch and skin inflammation characterized by increased skin thickness, infiltrating immune cells, increased levels of IgE in circulation, proinflammatory mediators in the skin, and impaired skin barrier. Bullous pemphigoid (BP) patients also present increased TSLP in lesional skin and circulation. Our findings suggest that BP180 regulates pruritic skin inflammation. Elucidation of molecular and cellular mechanisms underlying the role of BP180 in skin inflammation should help better understanding of pathophysiology of skin inflammation and identify therapeutic targets for skin inflammatory diseases such as BP and atopic dermatitis.