Both clinically and immunopathologically, pemphigus erythematosus combines features of pemphigus and lupus. Patients have intercellular autoantibodies and subcorneal acantholysis as seen in pemphigus foliaceus. Patients also frequently have positive antinuclear antibodies (ANA) and a positive lupus band on direct immunofluorescence.
Pemphigus erythematosus occurs with increased frequency in patients with a history of thymoma, myasthenia gravis, and other autoimmune diseases. Some of these patients also have autoantibodies directed against striated muscle. Certain HLA subtypes are represented more frequently in pemphigus erythematosus (HLA-A10, HLA-A26, HLA-DRW6).
Pemphigus erythematosus can be drug induced. The most notable medications associated with this condition are d-penicillamine and captopril. Both of these agents contain a highly negatively charged sulfhydryl group that has been implicated as the potential cause of acantholysis. Pemphigus erythematosus has been reported to occur in lesions of ultraviolet burns and thermal burns. More rarely, pemphigus erythematosus has been reported to occur in the setting of malignancy, most commonly bronchogenic carcinoma.
Pemphigus erythematosus is a chronic autoimmune blistering disease that is generally regarded as a limited form of pemphigus foliaceus. It is less likely to rapidly progress than other forms of pemphigus and responds to similar immunosuppressant therapies.
L10.4 – Pemphigus erythematosus
36739006 – Pemphigus erythematosus
- Pemphigus foliaceus – Presents with widespread crusted erosions involving predominantly the face, chest, and back.
- Polymorphous light eruption
- Lupus erythematous, subacute – Characteristic histopathology and usually positive for ANA and anti-Ro.
- Pemphigus vulgaris and systemic lupus erythematosus (SLE)
- Impetigo – Acute development of inflamed crusted erosions that are often quite painful.
- Seborrheic dermatitis – Chronic eczematous dermatitis in the absence of blistering and autoantibodies.