Autoimmune diseases and their manifestations on oral cavity: diagnosis and clinical management

Oral signs are frequently the first manifestation of autoimmune diseases. For this reason, dentists play an important role in the detection of emerging autoimmune pathologies. Indeed, an early diagnosis can play a decisive role in improving the quality of treatment strategies as well as quality of life. This can be obtained thanks to specific knowledge of oral manifestations of autoimmune diseases. This review is aimed at describing oral presentations, diagnosis, and treatment strategies for systemic lupus erythematosus, Sjögren syndrome, pemphigus vulgaris, mucous membrane pemphigoid, and Behcet disease

Clinical and histological features of gingival lesions : a 17-year retrospective analysis in a northern Italian population

Objectives: Only few studies on gingival lesions considered large enough populations and contemporary literature does not provide a valid report regarding the epidemiology of gingival lesions within the Italian population. The histopathological and clinical appearance of 538 gingival lesions from northern Italians are described and discussed here. Study Design: The case records of patients referred for the diagnosis and management of gingival lesions, from October 1993 to October 2009, were reviewed. Data regarding the histological type of lesion were also obtained from the biopsy register for each case, and blindly re-examined. Results: We reported a greater frequency of benign lesions (reactive and/or inflammatory) in non-plaque/non- calculus induced gingival disorders. We confirmed an unambiguous prevalence of oral squamous cell carcinoma above all other malignant neoplasia, and a prevalence of neoplastic malignant lesions in the maxilla, with a slight increase in females and a drift of the incidence peak from the seventh to the eighth decade. There was a prevalence of precancerous gingival lesions in the maxilla, with a higher incidence in females and with a drift from the sixth to the seventh decade. We also reported a prevalence of oral lichen planus and lichenoid lesions as major manifestations of desquamative gingivitis. Conclusions: The high frequency of gingival involvement of such different diseases emphasizes the importance of histological characterization and differential diagnosis for periodontists, but more prospective studies are needed to better describe the true incidence of the non-plaque related gingival diseases

HIV Patient with Mucous Membrane Pemphigoid: A Case Report

Background: Mucous Membrane Pemphigoid, a new denomination of cicatricial pemphigoid, encompasses a group of chronic subepithelial autoimmune blistering diseases that predominantly affect the oral cavity and the eyes (conjunctivitis and symblepharon).Case Details: A rare case of Mucous Membrane Pemphigoid (MMP) in a human immunodeficiency virus (HIV) positive patient is discussed with clinicohistopathological presentation.Conclusion: Since our patient was HIV-positive and had lesions restricted to the oral mucosa with ocular involvement, only topical and intralesional steroids were preferred as the first line of treatment. Systemic corticosteroid therapy raises a concern regarding immunosuppression.Keywords: HIV, MMP, Cicatricial pemphigoid

Benign Mucous Membrane Pemphigoid: A Case Report

Benign Mucous Membrane Pemphigoid (MMP) is a vesiculobullous (VB) lesion that affects oral and nasal mucosa, oropharynx, conjunctiva, and genital mucosa. It is considered an autoimmune disease. Autoantibodies are produced due to several external factors or genetic factors. It creates many complications, such as defects in vision, voice alteration (hoarseness), airway obstruction, and dyspareunia. The subepithelial blisters form due to these antibodies. Treatment modalities differ from topical steroids to systemic steroid therapy based on the nature and severity of clinical symptoms

Acute periodontal lesions

This is a review and update on acute conditions affecting the gingival tissues, including abscesses in the periodontium, necrotizing periodontal diseases, and other acute conditions that cause gingival lesions with acute presentation, such as infectious process not associated with oral bacterial biofilms, muco-cutanenous disorders, and traumatic and allergic lesions. A periodontal abscess is clinically important since it is a relatively frequent dental emergency, it can compromise the periodontal prognosis of the affected tooth, and because bacteria within the abscess have been identified, mainly by the type of etiology, and there are clear diffrences between those affecting a previously existing periodontal pocket ahd those affecting healthy sites. Therapy for this acute condition consists of drainage and tissue debridement, with individual evaluation of the need for systemic antimicrobial therapy. the definitive treatment of the pre-existing condition should be accomplished after the acute phase is controlled. Necrotizing periodontal disease (NPD) present three typical clinical features : papilla necrosis, gingival bleeding, and pain. Although the prevalence of these diseases is not high, their importance is clear, since they represent the most severe conditions associated with dental biofilm, with very rapid tissue destruction. In adittion to bacteria, the etiology of NPD includes numerous factors that alter the host response and predispose to these diseases, including HIV infection, malnutrition, stress, and tobacco smoking. The treatment consists of superficial debridement, careful mechanical oral hygiene, rinsing with chlorhexidine, and daily re-evaluation. Systemic antimicrobials may be used adjunctively in severe cases or in non-responding conditions and the best option is metronidazole.Once the acute disease is under control, definitive treatment should be provided, including the adequate therapy for the pre-existing gingivitis or periodontitis. Among other acute conditions affecting the periodontal tissues, but not caused by the microorganisms present in oral biofilms , are infectious diseases, muco-cutaneous diseases and traumatic or allergic lesions. In most cases, the gingival envolvement is not severe, though they are common and may prompt a dental emergency visit. These conditions may the direct result of a trauma or the consequence of the breaking of vesicles and bullae. A proper differential diagnosis is important for an adequate management of the case

Desquamative Gingivitis: Early Sign of Mucous Membrane Pemphigoid and Pemphigus Vulgaris

Early signs and symptoms of autoimmune bullous diseases such as mucous membrane pemphigoid (MMP) or pemphigus vulgaris (PV) develop in the oral cavity in almost all cases. Desquamative gingivitis (DG) is a clinical manifestation common to several diseases or disorders and is frequently associated with autoimmune bullous diseases. This is a retrospective study of 37 patients with MMP (24 cases) or PV (13 cases) including 10 males and 27 females with a mean age of 58.4 years. The study indicates that DG is an early sign of autoimmune bullous diseases such as MMP or PV. About 70.3% of the oral lesions were confined only to the gingiva, and DG was the only manifestation of the diseases. Since some lesions remain limited to the oral cavity for a long period of time, patients diagnosed with MMP or PV should be closely followed because they must be immediately referred to other experts when they develop lesions on parts of their body other than the oral cavity. The oral healthcare provider should collaborate with other healthcare experts including dermatologists, ophthalmologists, and otolaryngologists to evaluate and manage patients with autoimmune bullous diseases in the oral cavity

Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) Dermatology DepartmentUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) Dermatology and Pathology DepartmentsUNIFESP, EPM, Dermatology DepartmentUNIFESP, EPM, Dermatology and Pathology DepartmentsSciEL

Oral autoimmune vesicobullous diseases: Classification, clinical presentations, molecular mechanisms, diagnostic algorithms, and management

Mucocutaneous blistering autoimmune diseases are a group of systemic, rare, chronic disorders characterized by humoral-mediated immunologic mechanisms against epithelial, basement membrane, and subepithelial tissues. Morbidity and mortality can be completely different among these diseases, with outcome being dependent on an early and accurate diagnosis, systemic comorbidities, and the patient's response to treatment. Definitive diagnosis is based on clinical and histopathologic findings. Because clinical presentations among these diseases are often similar, different immunofluorescence tests and ELISAs are used to confirm the specific diagnosis. Oral mucosa may often be the first site of clinical manifestation from which the disease spreads to other mucosal surfaces and skin. Thus, often dentists and oral medicine specialists may be the first to encounter patients with such diseases. In this review we discuss the most frequent autoimmune vesicobullous disorders, namely pemphigus vulgaris, mucous membrane pemphigoid, bullous pemphigoid, and linear IgA disease

Paraneoplastic pemphigus: insight into the autoimmune pathogenesis, clinical features and therapy

Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed "paraneoplastic pemphigus". We included also papers in French, German, and Spanish. We found 613 papers for "paraneoplastic pemphigus". Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed “paraneoplastic pemphigus”. We included also papers in French, German, and Spanish. We found 613 papers for “paraneoplastic pemphigus”. Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist

Desquamative Gingivitis

Desquamative gingivitis (DG) is characterized by erythematous, epithelial desquamation, erosion of the gingival epithelium, and blister formation on the gingiva. DG is a clinical feature of a variety of diseases or disorders. Most cases of DG are associated with mucocutaneous diseases, the most common ones being lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris. Proper diagnosis of the underlying cause is important because the prognosis varies, depending on the disease. This chapter presents the underlying etiology that is most commonly associated with DG. The current literature on the diagnostic and management modalities of patients with DG is reviewed