Systemic lupus erythematosus and pemphigus vulgaris: association or coincidence

Few cases have been published relating systemic lupus erythematosus (SLE) and pemphigus vulgaris (PV). We describe a patient with this association. A 35-year old woman who started to develop persistent pain and morning stiffness of proximal inter and metacarpo-phalangeal joints. During the following year, the patient recalled the onset of blisters on both legs, face, arms and thorax, as well as erosions appearing on oral mucous membranes. We observed generalized multiple erosions on her trunk and legs, flaccid bullae located on her right thigh and multiple erosions on oral mucous membranes. A skin biopsy reported PV Direct immunofluorescence on the perilesional skin specimen, showed beehive intercellular IgG deposits in the epidermis (+++), suggesting PV; granular discontinuous IgM and C3 deposits in the dermal-epidermal union (+++), suggesting SLE. Direct immunofluorescence of the healthy unexposed skin specimen, reported granular discontinuous IgG deposits in the dermal-epidermal union and beehive intercellular IgG deposits in the lower levels of the epidermis (+++); granular continuous IgM deposits in the dermal-epidermal union (+++). The results of rheumatic studies were obtained as follows: ANA :3 +, Anti-DNA, Anti-Sm, Anti-Ro and Anti-La :4 + . The definite diagnosis was PVand SLE. Treatment with 50 mg of prednisone daily with good evolution.