PEMPHIGUS ERYTHEMATOSUS

By Gary M. White, MD

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Pemphigus erythematosus is the combination of pemphigus foliaceus and lupus erythematosis. It is characterized by crusted erosions in sun-exposed areas. Mucosal involvement occurs but is uncommon. It is also known as Senear-Usher syndrome.

Clinical

Red, scaly, erosive bullous or hyperkeratotic lesions occur on the nose and cheeks--an appearance and distribution similar to lupus erythematosus. Other photoexposed sites such as the upper back and V of the chest may be involved as well. Sunlight may aggravate the condition. DIF shows IgG and/or C3 intercellularly as well as along the BMZ. IIF is usually positive. A low-positive ANA may be present in approximately 30% of patients.

A 70-year-old man developed a relapse of pemphigus erythematosus after atorvastatin intake [Drug Des Devel Ther 2014;8:1463-5].

Treatment

Photoprotection is key. Using sunscreen containing a physical block is best. See sunscreen for more information. A potent topical steroid may be tried. Otherwise, a systemic corticosteroid is used. Dapsone may be added if needed. Dexamethasone-cyclophosphamide pulse therapy has put patients in remission. Some have suggested the use of rituximab.

References

Indian J Dermatol 2013;58:329

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