Six months before consulting, a 71-year old man developed buccal and genital erosions which gradually became worse. Physical examination showed signs of superficial desquamative
gingivitis, wide erosions on the bony palate, erythemato-erosive balanoposthitis and 7
bullae or skin erosions on the upper part of the back. At histopathological examination of a cutaneous
bulla there was dermoepidermal cleavage and an inflammatory infiltrate without eosinophils. At direct immunofluorescence, linear deposits of
IgG and C3 were present along the basement membrane. A search for anti-skin
autoantibodies was negative at indirect immunofluorescence but positive at immunoblotting (240 Kd band). The
cicatricial pemphigoid was treated with
dapsone alone in doses of 100 mg/day. Treatment was continued for 6 months, resulting in complete cure of the mucosal and cutaneous lesions. An immunoelectromicroscopic study, performed according to the technique described by Prost et al., on a fragment of skin from around the
bullae, showed deposits of granular
IgG in the lamina lucida and the lamina densa and deposits of C3 in the lamina densa. This case of
cicatricial pemphigoid exhibited 3 features which are not usually found in
bullous pemphigoid. Clinically, the buccal lesions were located on the gums and on the hard palate, i.e. where the mucosa adheres to the underlying bone through the periosteum. This location is habitual in
cicatricial pemphigoid and differs from that of the
bullous pemphigoid lesions which affect the free mucosa lining the cheeks and the soft palate. Treatment with
dapsone was dramatically successful in our patient whose lesions disappeared in 6 months.(ABSTRACT TRUNCATED AT 250 WORDS)