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1/18. Esthetic management of multiple recession defects in a patient with cicatricial pemphigoid.

    Cicatricial pemphigoid is one of a number of mucocutaneous disorders that can present in the oral cavity with desquamation, pain, and bleeding of the gingiva and oral mucosa. This case report describes the management of cicatricial pemphigoid in a patient with multiple sites of gingival recession using connective tissue grafting to alleviate root surface sensitivity and improve esthetics. The treatment rationale is presented and discussed in terms of timing of therapy and implications for wound healing in patients who present with desquamative gingivitis.
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keywords = oral cavity, cavity
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2/18. Immunopathological diagnosis of cicatricial pemphigoid with desquamative gingivitis. A case report.

    Cicatricial pemphigoid (CP) is a chronic subepidermal bullous dermatosis which primarily involves the mucous membranes. The oral cavity and the eye are most frequently involved. Since extension of the lesion into the pharynx and esophagus causes sore throat and dysphagia and progressive ocular lesions may cause blindness, early and valid diagnosis is very important. Here we present a case of cicatricial pemphigoid with onset at age 45 in a patient who manifested severe periodontal disease and showed the lesion on the mucous membranes of the mouth (desquamative gingivitis), skin, and eyes. Since definite diagnosis is very important, we describe how we made a differential diagnosis from other diseases which also accompany desquamative gingivitis. We examined the clinical manifestations, blood test results, HLA-genotype, histopathologic findings of the affected tissue, and immunological findings in relation to autoimmunity. Since many of the CP cases are first referred to periodontists or dentists, we believe that the diagnostic strategy described in the present study will be quite informative for making rapid and definite diagnoses of similar cases.
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ranking = 1.1127545393046
keywords = oral cavity, mouth, cavity
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3/18. Childhood oral pemphigoid: a case report and review of the literature.

    Oral pemphigoid, defined as cicatricial pemphigoid limited to the oral cavity, is a clinical subset of cicatricial pemphigoid. Like cicatricial pemphigoid, it occurs mostly in middle-aged women and it is uncommon in children under 20 years of age. There are 11 cases of childhood cicatricial pemphigoid published previously in the English language literature, and four of them are oral pemphigoid patients. We present a case of the youngest oral pemphigoid patient reported yet. Because of the rareness of cicatricial pemphigoid in children, delayed diagnosis and treatment have been shown in many cases reported earlier. Clinical awareness and early recognition are, therefore, emphasized.
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ranking = 1
keywords = oral cavity, cavity
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4/18. Antiepiligrin (laminin 5) cicatricial pemphigoid complicated and exacerbated by herpes simplex virus type 2 infection.

    A 50-year-old man with antiepiligrin (laminin 5) cicatricial pemphigoid (AeCP) involving the eyes, mouth and skin required a combination of systemic drug therapies to suppress the ocular disease. herpes simplex virus type 2 infection of the mouth and pharynx precipitated an acute deterioration, with laryngeal involvement and an increase in oral ulceration. This is an unusual complication of long-term immunosuppression and illustrates some of the difficulties in the management of patients with AeCP. Clinical improvement was obtained with oral antiviral therapy and adjustment of his immunosuppressive regimen.
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ranking = 0.22550907860923
keywords = mouth
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5/18. mucous membrane pemphigoid: nasal and laryngeal manifestations.

    mucous membrane pemphigoid (MMP) is a sub-epithelial blistering disease that primarily involves mucosal surfaces but may also involve the skin. Clinically, it appears as vesiculobullous lesions of the oral cavity and eyes, but other tissues such as the nasopharyngeal and laryngeal mucosa can also be affected. Ultimately, scarring and airway stenosis may occur. The condition should be managed by a multidisciplinary team led by a dermatologist. Immunosuppressive therapy forms the mainstay of treatment, with surgery having both a diagnostic role and a use in the treatment of complications such as airway obstruction. There must be a low threshold of suspicion for laryngeal involvement in this group of patients, so that prompt action can be taken. Once laryngeal stenosis has occurred repeated endoscopic laser excision of scar tissue can be used to maintain an adequate airway. Adjuvant use of mitomycin-C can be used beneficially in the treatment of laryngeal complications of MMP.
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keywords = oral cavity, cavity
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6/18. mucous membrane pemphigoid in a patient with chronic lymphocytic leukemia.

    PURPOSE: To describe a case of mucous membrane pemphigoid (MMP) with ocular manifestations in a patient subsequently diagnosed with B-cell chronic lymphocytic leukemia (CLL). methods: Case report and literature review. RESULTS: A 64-year-old man presented with redness and irritation of both eyes. The patient also had blistering lesions on the roof of his mouth, trunk, and buttocks as well as bilateral conjunctival ulceration and symblepharon formation. A complete blood count performed before initiating treatment of presumed mucous membrane pemphigoid revealed a markedly increased white blood cell count (83,920) with a predominance of lymphocytes. Hematologic evaluation including immunophenotyping flow cytometry resulted in the diagnosis of B-cell chronic lymphocytic leukemia. cyclophosphamide treatment improved his MMP and decreased the white blood cell count. CONCLUSIONS: mucous membrane pemphigoid has been reported in association with a variety of nonhematologic malignancies. This case suggests that mucous membrane pemphigoid may also occur in B-cell CLL and supports the concept that autoantibodies play a role in the pathogenesis of this cicatricial conjunctival disease.
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ranking = 0.11275453930461
keywords = mouth
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7/18. mucous membrane pemphigoid of the vulva.

    BACKGROUND: mucous membrane pemphigoid is a rare autoimmune blistering disease primarily affecting mucosal surfaces. Blistering and scarring may occur in the eyes, mouth, esophagus, larynx, and on the vulva. Scarring can result in severe structural changes to the vulva that may mimic the findings of other inflammatory dermatologic disorders of the vulva, including lichen sclerosus and lichen planus. CASE: A 58-year-old woman presented with vulvar erosions, esophagitis, and laryngeal blisters. The clinical picture and the histopathology of a vulvar biopsy were suggestive of erosive lichen planus. Direct immunofluorescence, however, revealed findings diagnostic of mucous membrane pemphigoid. CONCLUSION: This case illustrates the importance of examining extragenital mucosal surfaces of any woman presenting with vulvar lesions. In addition, it demonstrates the importance of vulvar biopsy and the usefulness of direct immunofluorescence to differentiate between conditions with similar clinical and histopathologic changes.
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ranking = 0.11275453930461
keywords = mouth
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8/18. Cicatricial pemphigoid of the oropharynx after allogeneic stem cell transplantation for relapsed follicular lymphoma.

    A 44-year-old woman with refractory follicular lymphoma underwent allogeneic stem cell transplantation (SCT) and achieved complete remission. Grade III acute graft-versus-host disease (GVHD) developed on day 23, but no chronic GVHD occurred. The patient developed severe erosion with bullous lesions in the oral cavity 18 months after SCT. At that time, the lymphoma remained in complete remission, and she had no clinical or laboratory findings suggesting chronic GVHD. A biopsy of the oral mucosa showed moderate lymphoplasmacytic infiltration and subepidermal bullae, and direct immunofluorescence staining demonstrated linear deposition of C3 at the dermo-epidermal junction. An immunoblotting assay using human epidermal extracts confirmed the presence in her serum of an antibody against the 230-kd bullous pemphigoid antigen 1 (BPAG1). A diagnosis of cicatricial pemphigoid (CP) was made, and complete resolution of the CP was achieved with prednisolone therapy. The occurrence of autoimmune blistering diseases is rare after allogeneic SCT.
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ranking = 1
keywords = oral cavity, cavity
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9/18. Successful treatment of mucous membrane pemphigoid with tacrolimus.

    mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune, chronic inflammatory ubepithelial vesiculobullous disorders, predominantly affects the mucous membranes of the oral cavity and eyes of the elderly population. oral manifestations of pemphigoid include desquamative gingivitis, ulcers, erythematous patches, erosions, vesicles and bullae located on the attached gingiva, palate, buccal mucosa, labial mucosa, and tongue. Diagnosis is based on history, clinical features and a biopsy stained with hematoxylin and eosin (H&E), and also direct and indirect immunofluorescence. Topical and systemic corticosteroids are the most commonly used medications for managing pemphigoid. Recently, topical tacrolimus has been successfully used in the treatment of ocular and skin pemphigoid. In this report we present a patient with longterm recalcitrant MMP that did not respond to conventional treatment but as treated successfully with tacrolimus ointment.
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keywords = oral cavity, cavity
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10/18. Benign mucous membrane pemphigoid: a case report.

    A relatively rare systemic disease, benign mucous membrane pemphigoid (cicatricial pemphigoid) usually starts in the mouth and is clinically characterized by bullae that rupture and form an ulcer. A distinctive Nikolsky's sign is apparent using gentle air blasts or finger pressure. The pharynx, larynx, nose, esophagus, genitals and eyes can also be affected. Involvement of the conjunctivae can lead to scarring and ensuing blindness. The following case of a healthy 77-year-old man, diagnosed as suffering from mucous membrane pemphigoid, is of particular interest since several confusing clinical observations, including poor oral hygiene, the possibility of a contact dermatitis or an adverse antibiotic reaction, made the diagnosis more difficult. A careful medical history, examination and consultation process is paramount to initiating proper treatment and subsequent relief of symptoms. Benign mucous membrane pemphigoid must always be considered in any patient with desquamative epithelium of the oral mucosa.
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ranking = 0.11275453930461
keywords = mouth
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