antidesmoglein1

Anti-desmoglein-1 antibodies (anti-Dsg1) are autoantibodies directed against desmoglein 1, a calcium-dependent cell–cell adhesion protein in desmosomes of the epidermis. They are most closely associated with the pemphigus disease spectrum, particularly pemphigus foliaceus.

Desmoglein 1 is expressed mainly in the superficial layers of the epidermis. Autoantibodies against Dsg1 disrupt keratinocyte adhesion, leading to acantholysis in the upper epidermis and resulting in flaccid, crusted erosions on the skin. Mucous membranes are less commonly involved because they express relatively less Dsg1 and more desmoglein 3; thus anti-Dsg1–driven disease tends to spare mucosa, though mucosal involvement can occur in some cases, especially when anti-Dsg3 is also present.

Clinically, anti-Dsg1 antibodies are detected in patients with pemphigus foliaceus, including endemic forms such as fogo selvagem. Diagnosis is supported by serology using enzyme-linked immunosorbent assay (ELISA) to quantify anti-Dsg1 IgG, which often correlates with disease activity. Indirect immunofluorescence can show intercellular staining in the epidermis. Anti-Dsg3 antibodies, by contrast, are more typical of pemphigus vulgaris and mucocutaneous involvement, and some patients may have antibodies to both Dsg1 and Dsg3.

Treatment follows pemphigus management principles, with systemic corticosteroids and immunosuppressants as mainstays, and increasingly rituximab for refractory cases. Anti-Dsg1 titres can be used to monitor response to therapy in pemphigus foliaceus.

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