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Following their use of immunofluorescent methods for establishing a diagnosis in about fifteen cases of mainly inflammatory disorders of the buccal mucosa, the authors discuss the procedures used and their value in buccal bullous diseases, and also in lupus erythematosus, lichen, and aphthosis.
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Pemphigus vulgaris is an autoimmune bullous disorder characterized by autoantibodies directed against desmoglein 3. A group of 19 pemphigus vulgaris sera were characterized by immunoblotting, immunofluorescence, immunoprecipitation, and the passive transfer mouse model. The aim of these studies was to determine the specificity of the autoantibody response in these patients. All patients had clinical and histologic evidence of pemphigus vulgaris. Fogo selvagem sera (n = 8), bullous pemphigoid sera (n = 8), antinuclear antibodies positive sera from patients with lupus erythematosus (n = 2), and normal human sera (n = 8) were used as controls. All pemphigus vulgaris patients showed titers of IgG autoantibodies by indirect immunofluorescence > or = 1:60, predominantly of the IgG4 subclass and immunoprecipitated recombinant desmoglein 3 expressed in the baculovirus system. Patients with disease localized to the mucous membranes showed no reactivity with desmoglein 1 and only one had weak reactivity with mouse skin by indirect immunofluorescence (titer = 1:20). Sera of four of these mucosal patients were tested in the mouse model and three of four did not elicit skin or mucosal disease in the animals. In contrast, sera from all seven patients with disease involving the skin and mucous membranes (generalized disease) produced disease in neonatal mice. In one patient the disease evolved from pure mucosal involvement associated with anti-desmoglein 3 antibodies to a disorder involving mucosas and skin. This transition was associated with the appearance of anti-desmoglein 1 antibodies in the patient's serum. These studies indicate that the autoantibody response in pemphigus vulgaris is heterogeneous. Epitopes recognized by some pemphigus vulgaris sera are species specific and others may be mucosal specific.
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Among the numerous idiopathic immune-mediated diseases that can be drug-induced, such as pemphigus, psoriasis, lichen, etc, drug-induced lupus is the most widely commented upon and investigated. The terms drug-induced lupus (DIL) and drug-induced lupus erythematosus (DILE) are preferred, but other ones are also used--drug-related lupus, lupus-like syndrome, and lupus erythematosus medicamentosus. This review discusses the general issues in DILE, such as pathogenic mechanisms, clinical forms, and diagnostic criteria, and provides more detailed information for some of the implicated drugs: minocycline, statins, terbinafine, etc.
Immunofluorescence studies were made on light (L) chain of immunoglobulins in patients with pemphigus. These patients fell into two groups: a subcorneal bulla group, consisting of 10 patients with Senear-Usher syndrome, and one with pemphigus foliaceus, and a suprabasal bulla group consisting of 8 patients with pemphigus vulgaris. In the former group there was deposition of L chains of either type alone in the intercellular spaces of the upper epidermis: kappa type alone in 9 and lambda type alone in 2 patients. Deposition of L chains of both types was observed in the intercellular spaces of the lower epidermis in all patients of the latter group. No significant difference existed between groups as to the titre of circulating intercellular antibodies of either type. Some comments are made on the significance of the L chain of immunoglobulins in a pemphigus.