Coexistence of pemphigus herpetiformis and systemic lupus erythematosus

A female patient with coexistence of pemphigus herpetiformis and systemic lupus erythematosus is described. She presented to our Department with pruritic vesicles on her trunk and extremities, which were later accompanied with butterfly like erythema on her face and with central nervous system (CNS) manifestations. The diagnosis of pemphigus herpetiformis was based on the clinical picture and immunofluorescence finding, because the histopathologic finding is not always typical for the diagnosis. The diagnosis of systemic lupus erythematosus was based on positive ANA and anti-dsDNA, presence of butterfly-like erythema on her face, and CNS manifestations. The patient was treated by corticosteroids in combination with immunosuppressants, which should ensure good control of both diseases. The coexistence of pemphigus herpetiformis and systemic lupus erythematosus has not been reported in recent literature.