Simultaneous occurrence of herpetiform pemphigus and endemic pemphigus foliaceus (fogo selvagem)

A 32–year‐old woman from Franco da Rocha, one of the last endemic areas of pemphigus foliaceus (fogo selvagem, FS) in the state of Sao Paulo, Brazil,’ 2 developed generalized FS based on clinical (see Fig. 1), histologic and immunologic criteria. 3 She had subcorneal vesicles with acantholytic cells histologically, circulating intercellular immunoglobulin G (IgG) autoantibodies at a titer of 640, and deposits of IgG in epidermal intercellular spaces. Her sister also had FS. The course of the disease was chronic with periods of remission and relapses. Six years later, she developed pruritic erythematous edematous plaques with overlying vesicles and blisters in a herpetiform pattern on the abdomen and forearm (Fig. 2) that resembled lesions of dermatitis herpetiformis. The lesions disappeared with prednisone 40 mg/day. Five years later, while affected with typical lesions of FS, she developed intensely pruritic erythematous urticarial edematous plaques (Fig. 3), some of which were covered with vesicles typical of herpetiform pemphigus (HP) (Fig. 4). Histologically these lesions showed epidermal spongiosis, with eosinophilic exocytosis and acantholysis (Fig. 5). Direct immunofluorescence (DIF) was positive for granular intercellular deposits of IgG and C3 in the epidermis (Fig. 6). IgA Studies were negative. Circulating Intercellular IgG autoantibodies were present at a titer of 80. By immunoblotting, the sera reacted to a 160 kD antigen which co‐migrated with desmoglein–1 detected by monoclonal antibody (AE 23) (kindly provided by Dr. Tung‐Tien Sun, New York University Medical Center, New York) and by pemphigus foliaceus sera (Fig. 7). Thus, after 12 years of FS, the patient developed a mixed bullous disease with concomitant clinical and laboratory features typical of HP and FS.