76 results match your criteria: "Oral Manifestations of Autoimmune Blistering Diseases"

Castleman disease (CD) is a rare lymphoproliferative disease known as angiofollicular lymph node hyperplasia, firstly reported in 1954. It mainly occurs in adults, presenting with a wide range of clinical manifestations, including paraneoplastic pemphigus (PNP). PNP is a rare and often life-threatening autoimmune disorder characterized by painful blisters and erosions on the skin and mucous membranes.

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A systematic review of paraneoplastic pemphigus and paraneoplastic autoimmune multiorgan syndrome: Clinical features and prognostic factors.

J Am Acad Dermatol

October 2024

Department of Dermatology and Venereology, West China Hospital, Sichuan University, Chengdu, Sichuan, China. Electronic address:

Article Synopsis
  • Paraneoplastic pemphigus (PNP), also referred to as paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare and serious autoimmune condition affecting the skin, mucous membranes, and various organs, with a high risk of mortality.
  • Researchers conducted a comprehensive analysis of 290 articles, including data from 504 patients, to gather insights on demographics, symptoms, associated tumors, treatment options, and survival outcomes.
  • Key findings indicated that older age, specific autoantibodies, and non-Hodgkin lymphoma were linked to shorter survival, while initial oral mucosal involvement and certain skin conditions were associated with longer survival; the study highlighted significant prognostic factors for PNP/PAMS patients.
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Article Synopsis
  • * A case study is presented of a 78-year-old Japanese man with bullous pemphigoid (BP) and AFVI who was treated with prednisolone but experienced serious complications, including gastrointestinal bleeding and septic shock.
  • * The study suggests that testing for coagulation disorders, such as AFVI, is essential during the treatment of autoimmune diseases like BP, potentially due to a connection between specific antibodies and clotting factors.
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  • - Primary Immune Thrombocytopenic Purpura (ITP) is a rare autoimmune condition marked by low platelet counts, often showing oral symptoms, and is even rarer in patients with Turner Syndrome (TS).
  • - A 29-year-old woman with TS presented with severe oral bleeding, blood blisters, and petechiae; lab tests confirmed her diagnosis of severe ITP.
  • - The successful treatment with corticosteroids led to a significant platelet increase, highlighting the need for dentists to recognize oral signs of ITP to ensure timely diagnosis and appropriate care, especially for those with TS who face increased health risks.
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  • * Most affected individuals were women in their 50s and 60s, with the mouth being the most common site for lesions (71.4% in PV and 91% in MMP); the cheek mucosa and gingiva were notably the most frequently impacted areas.
  • * Treatment varied, with systemic corticosteroids being the primary approach for PV patients (50%), while MMP patients often received topical therapy (53.7%); differences
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Background: The manifestations of bullous pemphigoid (BP) and herpes simplex virus (HSV) infection are similar in oral mucosa, and the laboratory detection of HSV has some limitations, making it difficult to identify the HSV infection in oral lesions of BP. In addition, the treatments for BP and HSV infection have contradictory aspects. Thus, it is important to identify the HSV infection in BP patients in time.

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Pemphigus vulgaris, a rare and life-threatening autoimmune disorder, presents with painful skin and mucosal lesions, leading to blistering sores attributed to acantholysis. This study delves into the clinical manifestations, risk factors, and diagnostic intricacies associated with pemphigus vulgaris, with a focus on a representative case highlighting the challenges in its recognition and management. We explore the case of a 60-year-old male with pemphigus vulgaris, whose initial presentation involved yellow-crusting lesions on the scalp progressing to non-pruritic lesions on the chest, neck, and inguinal areas.

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Bullous pemphigoid (BP) is an autoimmune blistering disease that affects primarily the skin and rarely involves the mucosa of the oral cavity, larynx, pharynx, or esophagus. Diagnosis is based on the clinical presentation, histology, and immunopathological examination. Laryngeal involvement in BP is extremely rare and patients may be asymptomatic or may present with dysphonia, dysphagia, and/or respiratory distress.

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A rare case of pemphigus vulgaris disguised as a malignant gingival ulcer.

BMC Oral Health

May 2023

Department of Periodontics and Oral Medicine, Xiangya Stomatological Hospital & Xiangya School of Stomatology, Hunan Key Laboratory of Oral Health Research & Hunan Clinical Research Center of Oral Major Diseases and Oral Health & Academician Workstation for Oral-maxillofacial and Regenerative Medicine, Central South University, Changsha, 410008, China.

Background: Pemphigus vulgaris (PV) is a kind of rare and severe autoimmune bullous disease. In this case, the specificity of oral PV lies in the clinical manifestations of a single palatal ulcer, and no blisters were found in the oral mucosa. This case provides a powerful reference for dentists diagnosing and treating oral PV with atypical clinical presentations.

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Immune-mediated and autoimmune diseases of the skin often present with oral cavity involvement. Autoimmune subepidermal blistering diseases and pemphigus vulgaris are classic examples. While the primary lesions (vesicles and bullae) are relatively specific, these fragile lesions evolve rapidly into erosions and ulcers, which are lesion types that overlap with many diseases.

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Associations between bullous pemphigoid and hematological diseases: Literature review on mechanistic connections and possible treatments.

Front Immunol

March 2023

Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.

Bullous pemphigoid is an autoimmune blistering disorder that primarily occurs in elderly patients. Reports indicate that BP coexists with various hematological diseases, including acquired hemophilia A, hypereosinophilic syndrome, aplastic anemia, autoimmune thrombocytopenia, and hematological malignancies. Early identification of these comorbidities contributes to a better control and reduced mortality.

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Systemic lupus erythematosus (SLE) is an autoimmune disease with varying dermatological findings. We review a unique presentation of SLE with a literature review. A previously healthy early adolescent female presented with painful, oral mucosal bullae filled with sanguineous fluid.

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Background: Bullous pemphigoid is the most common autoimmune subepidermal blistering disorder with a low incidence in childhood. Combined immunodeficiencies (CIDs) are a group of monogenic inborn errors of immunity (IEIs) characterized by T- and B-cell dysfunction leading to recurrent infections, lymphoproliferation, predisposition to malignancy, and autoimmunity. Here, we report two Afghan siblings with a diagnosis of CID and extremely rare manifestation of diffuse bullous pemphigoid skin lesions.

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Case report: Minimal manifestations of mucous membrane pemphigoid in a young adult.

Front Med (Lausanne)

November 2022

Department of Dermatology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

A male patient presented to our department at the age of 23 suffering from recurrent painful erosions in the urethral outlet area. In closer clinical examination gingival erosions, primarily around the teeth were identified as well. Indirect immunofluorescence on salt split skin with epidermal IgG deposition and positive anti-BP230 IgG ELISA diagnostics hinted toward the presence of mucous membrane pemphigoid (MMP).

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Behçet's disease (BD) is a systemic inflammatory disease of unknown etiology. BD is characterized by relapsing oral and genital ulcers, several different cutaneous features, relapsing bilateral uveitis, and involvement of internal organs, showing vascular, gastrointestinal, and neurological manifestations. Serologically, BD is not characterized by disease-specific autoantibodies.

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Pemphigus vulgaris (PV) is a rare painful and blistering autoimmune mucocutaneous disorder that appears in middle-aged adults. Oral lesions typically precede cutaneous involvement and tend to be more recalcitrant to therapy. The objective of this article is to present a case of oral and cutaneous PV in an atypical patient, a 23-year-old woman.

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Oral mucous membrane pemphigoid in a group of Thai patients-A 15-year retrospective study.

J Dent Sci

April 2022

Department of Oral Medicine and Periodontology, Faculty of Dentistry, Mahidol University, Bangkok, Thailand.

Article Synopsis
  • Mucous membrane pemphigoid (MMP) is a rare autoimmune disorder primarily affecting the mouth, leading to painful oral lesions, and this study focused on its characteristics in Thai patients and treatment approaches.
  • Fourteen patients were diagnosed with MMP, with common symptoms including chronic pain and bleeding gums, and most had lesions on their gums.
  • The main treatment was a combination of topical and systemic corticosteroids, with an average control time of about 2 months, indicating the need for multidisciplinary care and proper oral hygiene.
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Article Synopsis
  • - Pemphigus Vulgaris (PV) is a serious autoimmune disorder resulting in blisters on the skin and mucous membranes, notably impacting oral health in humans and other mammals.
  • - The disease involves the production of auto-antibodies targeting desmogleins, leading to a process called acantholysis, although the exact mechanisms of this process are not fully understood.
  • - The text introduces a new concept called apoptolysis, suggesting that apoptotic enzymes may play a significant role in the development of acantholysis in PV, proposing a new perspective on the disease's mechanisms.
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Article Synopsis
  • This analytic study reviewed existing data on Oral Fixed Drug Eruptions (OFDEs), focusing on patient characteristics, drug classifications, and manifestations.
  • The average age of patients with OFDEs was around 38.9 years, with most cases occurring in individuals aged 30 to 60, and the primary drugs involved were analgesics, antibiotics, and antifungals.
  • Common manifestations included erythematous lesions and lichenoid eruptions, often appearing one to three days after drug intake, highlighting the need for clinicians to be knowledgeable about OFDEs for better patient assessment.
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Pemphigus defines a group of rare autoimmune blistering diseases that affect the skin and mucous membranes, with pemphigus vulgaris being the most common form that has increased morbidity and mortality in the absence of an early diagnosis and treatment. We report the case of a 24-year-old male with an atypical form of pemphigus vulgaris with cutaneous onset and subsequent involvement of the oral cavity. The management of the patient initially consisted of long-term systemic corticosteroid therapy.

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Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the basal membrane zone of skin and surface-close epithelia and predominant mucosal lesions. The oral cavity and conjunctivae are most frequently affected, albeit clinical manifestations can also occur on the skin. MMP-associated lesions outside the oral cavity typically lead to scarring.

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Brunsting-Perry pemphigoid: a systematic review.

Int J Dermatol

November 2022

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Background: Brunsting-Perry pemphigoid (BPP) is a rare, autoimmune bullous skin disorder classified within the spectrum of mucous membrane pemphigoid (MMP).

Materials And Methods: An a priori protocol was designed based on PRISMA guidelines. PubMed and Scopus databases were searched for English-language articles concerning BPP published between 1950 and July 2021.

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Pemphigus-The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic.

Biomedicines

October 2021

Department of Dermatology and Venereology, University Hospital Centre Zagreb, School of Medicine University of Zagreb, Šalata 4, 10000 Zagreb, Croatia.

Pemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus vulgaris and pemphigus foliaceus.

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Simultaneous Oral and Umbilical Locations as a First Sign of Pemphigus Vulgaris.

Case Rep Dent

October 2021

Department of Dental Medicine, Sahloul Hospital (Sousse), Dental Faculty of Monastir, University of Monastir, Tunisia.

The place of pemphigus vulgaris (PV) among autoimmune bullous diseases (AIBD) is well established. It is an acquired chronic, autoimmune, vesiculobullous disease in which IgG antibodies target desmosomal proteins to produce intraepithelial mucocutaneous blistering. The diagnosis is often challenging for the clinicians.

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