Pharyngeal Ulcer as the First Sign of Pemphigus

Pemphigus vulgaris (PV) is a rare life threatening, autoimmune bullous disease affecting mucous membrane and skin. Pregnancy and emotional stress may be the possible triggering factors for PV. It is a very rare phenomenon with less than 27 cases of pregnancy induced PV and only 9 cases of neonatal pemphigus reported in literature. The lesions may appear transient in neonate which resolve spontaneously over 2-3 weeks. We hereby, report a case of 26-year-old female who developed PV in second trimester of pregnancy with peripartum depression, which was managed by intravenous, oral and topical steroids and selective-serotonin reuptake inhibitor (SSRI). Furthermore, the development of transient neonatal pemphigus raises the possibility of genetic linkage from maternal to fetal transmission.

Download Full-text

Non-Hodgkin’s Plasmablastic Lymphoma as Initial Presentation of Human Immunodeficiency Virus

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/23247096211014689 ◽

2021 ◽

Vol 9 ◽

pp. 232470962110146

Author(s):

Roopam Jariwal ◽

Nadia Raza ◽

Janpreet Bhandohal ◽

Everardo Cobos

Keyword(s):

Human Immunodeficiency Virus ◽

Acquired Immunodeficiency Syndrome ◽

Initial Presentation ◽

Plasmablastic Lymphoma ◽

Healthy Male ◽

Immunodeficiency Virus ◽

Life Threatening ◽

Acquired Immunodeficiency ◽

Immunodeficiency Syndrome ◽

Head Neck

Plasmablastic lymphoma (PBL) is a subtype of non-Hodgkin’s lymphoma that manifests in patients with the diagnosis of human immunodeficiency virus (HIV), more prominently in the head, neck, and oral mucosal region. The diagnosis of this rare lymphoma serves as a concomitant diagnosis of acquired immunodeficiency syndrome. The case is of a 33-year-old previously healthy male, with an unknown diagnosis of HIV with a painful right mandibular mass. He was subsequently diagnosed with PBL and HIV. This case of PBL illustrates the importance of linking a rare and potentially life-threatening diagnosis as a possible first manifestation of HIV.

Download Full-text

Is laryngeal involvement in pemphigus vulgaris rare? A review

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20160348 ◽

2015 ◽

Vol 1 (1) ◽

pp. 3

Author(s):

Mohan Bansal

Keyword(s):

Case Reports ◽

Pemphigus Vulgaris ◽

Google Scholar ◽

Cochrane Library ◽

Laryngeal Lesions ◽

Life Threatening ◽

Laryngeal Involvement

<p>The lesions of pemphigus in the larynx have been reported as case reports. However, the frequency with which it occurs is not known. The purpose of this study was to investigate the incidence of laryngeal involvement in pemphigus vulgaris and to examine the laryngeal lesions and the treatment in detail. The databases searched were PubMed, EMBASE, Cochrane Library and Google scholar. The involvement of larynx in patients with pemphigus vulgaris is common. The most common parts involved are epiglottis and arytenoids. This study indicates that laryngeal symptoms are common in pemphigus vulgaris. Laryngeal findings must be considered at the beginning of diagnosis for the better management and to prevent life threatening complications.</p>

Download Full-text

Citrobacter freundii sepsis in an immunosuppressed patient with pemphigus vulgaris

BMJ Case Reports ◽

10.1136/bcr-2018-227091 ◽

2018 ◽

Vol 11 (1) ◽

pp. e227091 ◽

Cited By ~ 3

Author(s):

Martina Ferranti ◽

Giulia Tadiotto Cicogna ◽

Andrea Sattin ◽

Mauro Alaibac

Keyword(s):

Immunosuppressive Agents ◽

Pemphigus Vulgaris ◽

Immunosuppressed Patient ◽

Citrobacter Freundii ◽

Bullous Disease ◽

Autoimmune Bullous Disease ◽

Systemic Corticosteroids ◽

Mucous Membranes ◽

Current Therapies ◽

Anti Cd20

Pemphigus vulgaris is an autoimmune bullous disease that involves the skin and mucous membranes. Current therapies aim to decrease antibody production by means of the use of systemic corticosteroids, immunosuppressive agents and, recently, rituximab, an anti-CD20 monoclonal antibody. However, the chronic immune suppression could entail complications, like infections and secondary malignancies. We describe a case of a patient with pemphigus who developed a sepsis due to Citrobacter freundii infection.

Download Full-text

Pemphigus vulgaris, large dose glucocorticoid therapy and staphylococcal bacteremia

The American Journal of Medicine ◽

10.1016/0002-9343(77)90127-9 ◽

1977 ◽

Vol 63 (1) ◽

pp. 152-160 ◽

Cited By ~ 5

Keyword(s):

Large Dose ◽

Pemphigus Vulgaris ◽

Glucocorticoid Therapy

Download Full-text

Bullous disorders

10.1093/med/9780199568741.003.0252 ◽

2018 ◽

Author(s):

Emily Davies

Keyword(s):

Basement Membrane ◽

Dermatitis Herpetiformis ◽

Pemphigus Vulgaris ◽

Basement Membrane Zone ◽

Mucous Membrane Pemphigoid ◽

Pemphigus Foliaceus ◽

Pemphigoid Gestationis ◽

Bullous Disease ◽

Adhesion Junctions ◽

Linear Iga Disease

This chapter focuses on immunobullous diseases. The immunobullous disorders are a group of diseases in which pathogenic autoantibodies bind to target antigens either in desmosomes (intra-epidermal intracellular adhesion junctions) or in part of the basement membrane zone, resulting in loss of adhesion, and blister formation. This chapter will focus on pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, linear IgA disease, chronic bullous disease of childhood, and dermatitis herpetiformis; it will also mention mucous membrane pemphigoid, pemphigoid gestationis, and epidermolysis bullosa acquisita.

Download Full-text

Pemphigus Vulgaris: Update on Etiopathogenesis, Oral Manifestations, and Management

Critical Reviews in Oral Biology & Medicine ◽

10.1177/154411130201300504 ◽

2002 ◽

Vol 13 (5) ◽

pp. 397-408 ◽

Cited By ~ 82

Author(s):

Crispian Scully ◽

Stephen J. Challacombe

Keyword(s):

Pemphigus Vulgaris ◽

Intravenous Immunoglobulins ◽

Current Treatment ◽

Enzyme Linked Immunosorbent Assay ◽

Ashkenazi Jews ◽

Normal Human Skin ◽

Autoimmune Blistering Disease ◽

Systemic Corticosteroids ◽

Life Threatening ◽

Systemic Infections

Pemphigus is a group of potentially life-threatening diseases characterized by cutaneous and mucosal blistering. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles. Certain ethnic groups, such as Ashkenazi Jews and those of Mediterranean origin, are especially liable to pemphigus. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibodies. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examinations, is essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected by both normal human skin and monkey esophagus or by enzyme-linked immunosorbent assay (ELISA). Before the introduction of corticosteroids, pemphigus vulgaris was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using systemic corticosteroids, with azathioprine, dapsone, methotrexate, cyclophosphamide, and gold as adjuvants or alternatives, but mycophenolate mofetil and intravenous immunoglobulins also appear promising.

Download Full-text

Oral pemphigus vulgaris

Journal of Kathmandu Medical College ◽

10.3126/jkmc.v4i3.18239 ◽

2017 ◽

Vol 4 (3) ◽

pp. 100-103

Author(s):

Mohit Sharma

Keyword(s):

Clinical Manifestations ◽

Pemphigus Vulgaris ◽

Oral Literature ◽

Therapeutic Approaches ◽

The Past ◽

Mucosal Surfaces ◽

Life Threatening ◽

Delays In Diagnosis ◽

Oral Involvement ◽

Dental Professionals

Pemphigus is a group of potentially life-threatening autoimmune mucocutaneous diseases characterized by epithelial blistering affecting cutaneous and/ or mucosal surfaces. Pemphigus affects 0.1-0.5 patients per 100,000 population per year. Oral lesions of pemphigus are seen in up to 18% of patients at dermatology out-patient clinics, but despite the frequency of oral involvement, and novel therapeutic approaches, there are surprisingly few recent studies of either the oral manifestations of pemphigus or their management, and delays in diagnosis are still common. Most patients are initially misdiagnosed and improperly treated for many months or even years. Dental professionals must be sufficiently familiar with the clinical manifestations of pemphigus vulgaris to ensure early diagnosis and treatment, since this in turn determines the prognosis and course of the disease. Pemphigus has been reviewed in the oral literature in the past decade, but several advances in the understanding of the etiopathogenesis, pemphigus variants, and management warrant an update. Here, we report a case of pemphigus vulgaris that was misdiagnosed in its earliest stage. Oral ulceration may arise from a variety of causes. This case illustrates that, although rare, pemphigus vulgaris may need to be included in differential diagnosis.

Download Full-text

Incidence of Spontaneous Pulmonary AVM Rupture in HHT Patients

Journal of Clinical Medicine ◽

10.3390/jcm10204714 ◽

2021 ◽

Vol 10 (20) ◽

pp. 4714

Author(s):

Adam Fish ◽

Katharine Henderson ◽

Alex Moushey ◽

Jeffrey Pollak ◽

Todd Schlachter

Keyword(s):

Spontaneous Rupture ◽

Pulmonary Hemorrhage ◽

Initial Presentation ◽

Lifetime Prevalence ◽

True Incidence ◽

Life Threatening ◽

Life Threatening Complication

The spontaneous rupture of pulmonary AVMs, resulting in pulmonary hemorrhage and hydrothorax, is a life-threatening complication. While this phenomenon has been previously reported, the true incidence is not yet known. This study retrospectively reviewed records of 801 HHT patients with pulmonary AVMs to identify a single lifetime episode of hemothorax or pulmonary hemorrhage secondary to pulmonary AVM rupture. The lifetime prevalence and incidence of pulmonary AVM rupture in HHT patients was 2.7% and 0.16% respectively. In these patients, AVM rupture represented the initial presentation of HHT in nine (40.9%) cases and was life-threatening in nine (40.9%) cases. All cases occurred in virgin lesions, and subsequent embolization was curative. While a feared complication, pulmonary AVM rupture is rare and is likely effectively prevented by existing embolization techniques and indications.

Download Full-text

Neurological and gastrointestinal symptoms as an initial presentation of pediatric thyroid storm: report of three cases

Journal of Pediatric Endocrinology and Metabolism ◽

10.1515/jpem-2021-0219 ◽

2021 ◽

Vol 0 (0) ◽

Author(s):

Masao Nogami ◽

Shadia Constantine ◽

Shuji Sai

Keyword(s):

Gastrointestinal Symptoms ◽

Antithyroid Drug ◽

Severe Case ◽

Initial Presentation ◽

Thyroid Storm ◽

Gi Symptoms ◽

Old Japanese ◽

Threatening Condition ◽

Life Threatening ◽

History Of

Abstract Objectives Thyroid storm (TS) is a rare but life-threatening condition caused by decompensated hyperthyroidism. There is no consensus on how to diagnose pediatric TS. We report three pediatric cases of TS presenting with central nervous system (CNS) and gastrointestinal (GI) symptoms as the initial presentation of Graves’ disease. Case presentation They were previously healthy adolescents without family history of thyroid disease. CNS symptoms varied from agitation to coma. GI symptoms included abdominal pain, vomiting, and diarrhea. Their laboratory studies revealed thyrotoxicosis and positive result of thyroid-stimulating antibody (TSAb). They were admitted to the intensive care unit (ICU) and received the combination of an antithyroid drug, Lugol’s solution, a beta antagonist, and hydrocortisone. The most severe case was a 13 year-old Japanese girl who presented with loss of consciousness and hemodynamic shock. She died after 5 days of intensive treatment. Conclusions Pediatricians should consider TS in the differential diagnosis when a patient exhibits both CNS and GI symptoms.

Download Full-text