[New aspects of the clinical course of dermatoses localized in the mouth mucosa and the lips].
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BACKGROUND: Several diseases may affect both skin and oral mucosa. This article presents hereditary, autoimmune and other inflammatory conditions within this group. MATERIAL AND METHODS: Review of the literature as well as the authors' own experience in the field. RESULTS AND INTERPRETATION: Lichen planus, erythema multiforme and lupus erythematosus are relatively common disorders with generally favourable prognosis. The much less frequent bullous diseases pemphigoid and pemphigus are characterized by a high morbidity, often requiring a deliberate use of immunosuppressants. Stevens-Johnson syndrome, toxic epidermal necrolysis and the more severe forms of epidermolysis bullosa are potentially life-threatening conditions. It is recommendable that medical doctors and dentists are familiar with the clinical presentations and diagnostic procedures of diseases affecting skin and oral mucosa.
Bullous pemphigoid antigen 180 (BP180) is the target of autoantibodies in various subepidermal blistering diseases. The most common one is bullous pemphigoid (BP). The pathological importance of anti-BP180 antibodies has been demonstrated in a passive transfer mouse model. However, sensitive assays for routinely detecting circulating antibodies directed against both intra- and extracellular domains of BP180 are only available in specialized laboratories. In addition, most current assays use prokaryotic recombinant fragments of BP180 that lack conformation-dependent epitopes. A simple and very sensitive immunofluorescence (IF) assay based on eukaryotic cells is described here. Sf21 insect cells were transfected with full-length (FL) BP180. As revealed by FACS and confocal laser scanning microscopy the protein was expressed as type II transmembrane protein as in human keratinocytes. By testing serial dilutions of BP180-specific mouse monoclonal antibodies, the eukaryotic IF assay was demonstrated to be more sensitive compared to conventional assays including (1) indirect IF microscopy of human salt-split skin, (2) Western blotting (WB) of the keratinocyte-derived BP180 ectodomain, (3) WB of recombinant BP180 NC16A, and (4) WB of FL-BP180 extracted from Sf21 insect cells. When applied to sera from patients with BP (n = 65), pemphigoid gestationis (n = 16), and cicatricial pemphigoid (n = 7), the novel assay revealed that 58 (89%), 13 (81%), and 6 (84%), respectively, were positive. In contrast, all control sera (pemphigus, n = 20; epidermolysis bullosa acquisita, n = 5; anti-laminin 5 cicatricial pemphigoid, n = 5; systemic lupus erythematosus, n = 5; atopic dermatitis, n = 7; contact dermatitis, n = 3; normal human sera, n = 30) were negative indicating that the assay is highly specific. In addition, reactivity of the assay was conserved to a large extent when the cells had been stored at -20 degrees C for 3 months. Thus, this assay meets the demands of a simple and effective diagnostic tool for detecting circulating antibodies against FL-BP180 and may also be used in laboratories without access to molecular biological technology.
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The immunologic, pathogenetic and clinical relationships between HLA antigens and oral immunologic diseases are described. The HLA typing is useful for an early diagnosis because it allows to single out the subjects at risk in a particular studied family.
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BACKGROUND: BAFF [B-cell activating factor belonging to the tumour necrosis factor (TNF) family] is a member of the TNF superfamily that regulates B-lymphocyte proliferation and survival. It has been demonstrated that increased levels of soluble BAFF are associated with systemic autoimmunity in patients with systemic lupus erythematosus, rheumatoid arthritis and Sjögren's syndrome, and in animal models of spontaneous autoimmune diseases. However, the significance of circulating BAFF in autoimmune bullous diseases is unknown. OBJECTIVES: To examine whether BAFF levels are elevated in the autoimmune blistering diseases pemphigus vulgaris (PV) and bullous pemphigoid (BP). METHODS: We examined sera obtained from 21 patients with PV, 39 patients with BP and 22 healthy donors. We performed enzyme-linked immunosorbent assays for soluble BAFF and each disease-specific antibody: antidesmoglein-3 antibody for PV and anti-BP180 antibody for BP. RESULTS: Significant elevations of serum BAFF levels were found in the patients with BP, but not with PV. There was apparently no significant association between the serum BAFF levels and titres of anti-BP180 antibodies in the patients with BP. However, serum BAFF levels tended to be more elevated in patients with a shorter disease duration. There was a tendency that BAFF levels increased before the anti-BP180 antibody levels increased at the onset of BP and quickly decreased in response to treatment. CONCLUSIONS: BAFF may be a useful marker for early activation of an autoimmune diathesis and may play a critical role in triggering activation of self-antigen-driven autoreactive B cells in BP.
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The intent and purpose of the authors is to familiarize the reader with the principles that govern the diagnosis and management of autoimmune mucocutaneous blistering diseases. The purpose was not to be all-inclusive but illustrative. There are several other blistering diseases that have not been discussed because of the limited scope of this article. Of these, toxic epidermal necrolysis, erythema multiforme, and the like warrant the same attention and early recognition. These are covered in different articles. For the same reasons, certain genetically linked blistering diseases have not been discussed.