[Cicatricial pemphigoid: management in stomatology]

Cicatricial pemphigoid is a subepidermal disease of the mucous membranes. In some patients blisters involving the oral cavity may be the only manifestation. The purpose of this work was to detail the clinical features and biological findings, highlighting the diagnostic and therapeutic difficulties.

Seventeen patients, nine women (mean age 59.6 years) and eight men (mean age 67.8 years) managed between 1990 and 2000 were reviewed retrospectively.

Mucous membranes of the mouth were the only localization in six of the seventeen patients. Other localizations (skin, eye, nose, anus) were associated, alone or in combination, in the other eleven patients. The gingivae were involved in 15/17 patients and constituted the only localization in six. Skin lesions (sometimes involving the lower limb) were variable and found in 6/17 patients. Ocular lesions were present at diagnosis in six patients, including two with severe lesions at onset. Nasal and/or laryngeal involvement was found in 6/17 patients. These lesions were often severe with major functional impact. ENT localizations were found in patients with at least three localizations and lesions extending to mucous membranes distant from the oral cavity. A biopsy was obtained from all patients and demonstrated subepidermal detachment in eleven. Direct immunofluorescence was positive in eleven. Indirect immunofluorescence, performed in twelve patients, was negative. In two patients, both histology and immunofluorescence were negative. Electron immunomicroscopy was performed in four patients and confirmed the diagnosis. Five out of six isolated oral manifestations were treated with topical steroids alone. Multiple lesions were treated case by case using daprosone and/or systemic corticosteroids associated with topical steroids in 16/17 patients. A bolus of cyclophosphamid was given for four patients with very severe lesions which did not respond to other treatments. At last follow-up (22 +/- 17 months), none of the patients was considered cured.

Cicatricial pemphigoid is a complex chronic disease. Oral manifestations may be isolated or associated with lesions of the skin or other mucous membranes. The painful aspect of oral lesions explains why the diagnosis is often made in stomatology. Careful evaluation at diagnosis is essential and requires a multidisciplinary approach. Certain diagnosis can be provided by immunohistology, particularly electron immunomicroscopy which enables distinguishing cicatricial pemphigoid from other autoimmune diseases of the dermo-epidermal junction. Treatment depends on disease severity. Isolated oral manifestations are generally well controlled by topical corticosteroids which ocular, nasal, and laryngeal localizations often require more extensive treatment.