IgA pemphigus

An autoimmune blistering skin disease

IgA Pemphigus

Schematic of dimeric IgA

IgA pemphigus is a rare autoimmune disease characterized by the presence of blisters on the skin and mucous membranes. It is a subtype of pemphigus, which is a group of autoimmune blistering disorders. Unlike other forms of pemphigus, IgA pemphigus is mediated by immunoglobulin A (IgA) antibodies rather than immunoglobulin G (IgG).

Pathophysiology

IgA pemphigus is caused by the production of IgA antibodies against components of the epidermis. These antibodies target proteins in the desmosomes, which are structures that help maintain the integrity of the skin by holding keratinocytes together. The binding of IgA antibodies to these proteins leads to the disruption of cell adhesion, resulting in the formation of blisters.

Clinical Presentation

Patients with IgA pemphigus typically present with erythematous plaques and vesicles that may coalesce into larger blisters. These lesions are often pruritic and can appear on any part of the body, but are commonly found on the trunk and extremities. Unlike other forms of pemphigus, mucosal involvement is less common in IgA pemphigus.

Diagnosis

The diagnosis of IgA pemphigus is confirmed through a combination of clinical examination, histopathology, and direct immunofluorescence. A skin biopsy will show subcorneal pustules with neutrophilic infiltration. Direct immunofluorescence of perilesional skin will reveal IgA deposition in the epidermis.

Treatment

The management of IgA pemphigus involves the use of medications to reduce inflammation and suppress the immune response. Dapsone is often the first-line treatment due to its anti-inflammatory and immunomodulatory effects. In cases where dapsone is ineffective or not tolerated, systemic corticosteroids or other immunosuppressive agents may be used.

Prognosis

The prognosis for patients with IgA pemphigus is generally favorable, especially with appropriate treatment. The disease tends to have a chronic course with periods of remission and exacerbation. Long-term management may be necessary to control symptoms and prevent relapses.

Related Pages

• Pemphigus vulgaris
• Bullous pemphigoid
• Autoimmune blistering disease