Case Report: Variety of Target Antigens During 1 Year Follow-Up of a Patient Initially Diagnosed With Bullous Pemphigoid

Autoimmune bullous diseases (AIBDs), presenting cutaneous and/or mucosal bullous lesions, are classified into pemphigus and pemphigoid diseases. A longtime observation for complicated AIBD cases is rarely reported. In this study, serum samples of one AIBD patient were collected at seven different time points during the disease course including a relapse, which were examined by our conventional and newly developed methods for the detection of autoantibodies. Interestingly, we found changes of both the presence and the titers of various autoantibodies in accordance with the changes of clinical features during the whole disease course, which indicated that the patient started as bullous pemphigoid and relapsed as concurrence of bullous pemphigoid and mucosal-dominant-type pemphigus vulgaris.

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Pemphigus Vulgaris and Bullous Pemphigoid of the Upper Aerodigestive Tract: A Review Article and Novel Approaches to Management

ORL ◽

10.1159/000515229 ◽

2021 ◽

pp. 1-9

Author(s):

Mohammed Hassan Hussain ◽

Faiz Tanweer ◽

Georgios Sakagiannis ◽

Manish Mair ◽

Sara Mahmood ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Patient Outcomes ◽

Multimodality Treatment ◽

Pemphigus Vulgaris ◽

Review Article ◽

Upper Aerodigestive Tract ◽

Systemic Corticosteroids ◽

Autoimmune Bullous Diseases ◽

Bullous Diseases ◽

Inflammatory Drugs

Background: Autoimmune bullous diseases are rare conditions characterized by blistering of the skin and mucous membranes. The 2 commonest forms are pemphigus vulgaris and bullous pemphigoid. The oral cavity or oropharynx may be the initial site of presentation or often the only site involved. Summary: These conditions are often misdiagnosed or overlooked leading to poorer patient outcomes. Due to the chronic nature of these conditions and the systemic effects of treatment, there is a significant associated morbidity and mortality. As such, an understanding of the fundamentals of autoimmune bullous diseases is vital to those working in otolaryngology. The mainstay of management in both conditions is topical and systemic corticosteroids. There is also a role for immunomodulating and non-steroidal anti-inflammatory drugs as adjunct or alternative therapies. Surgical intervention may be required to protect the airway. Often multimodality treatment is required involving multidisciplinary input from otolaryngologists, oral surgeons, dermatologists, and rheumatologists. This review article will highlight the aetiology, pathology, clinical features, investigations, and management of both pemphigus vulgaris and bullous pemphigoid including recent advances in management.

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Evaluation of Recombinant Antigen-Based Assays for Diagnosis of Bullous Autoimmune Diseases

Clinical and Diagnostic Laboratory Immunology ◽

10.1128/cdli.11.4.762-765.2004 ◽

2004 ◽

Vol 11 (4) ◽

pp. 762-765 ◽

Cited By ~ 4

Author(s):

G. D'Agosto ◽

A. Latini ◽

M. Carducci ◽

A. Mastroianni ◽

A. Vento ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Mucous Membrane ◽

Pemphigus Vulgaris ◽

Clinical Aspects ◽

Mucous Membrane Pemphigoid ◽

Pemphigus Foliaceus ◽

Serum Samples ◽

Cutaneous Lesions ◽

Autoimmune Bullous Diseases ◽

Mucosal Involvement

ABSTRACT The diagnosis of autoimmune bullous diseases is based on clinical observation and on the presence of autoantibodies directed to molecules involved in the adhesion systems of the skin. Immunofluorescence assays are the currently accepted method for detection of autoantibodies; such assays depend greatly on the skill of operators and are difficult to standardize. Recombinant desmoglein-1 (Dsg1), Dsg3, and BP180 peptides, the main autoantigens in pemphigus or bullous pemphigoid, have been used to develop new quantitative enzyme immunoassays (EIA) for the detection of specific antibodies. The present study was undertaken to evaluate the sensitivity and specificity of these immunoassays and to determine the correlation between the results and the clinical aspects of diseases. Serum samples from patients with pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, or mucous membrane pemphigoid, from healthy individuals, and from patients with unrelated autoimmune conditions were tested. Anti-desmoglein reactivity was detected in all the patients with pemphigus and in none of the controls. Patients with the more benign form of cutaneous disease had anti-Dsg1 antibodies, while patients with deeper cutaneous lesions or with mucosal involvement had anti-Dsg3 reactivity also, or exclusively. The BP180-based assay was positive for 66.6% of patients with bullous pemphigoid and for none of the patients with mucous membrane pemphigoid, and no reactivity was detected in the control sera. In conclusion, the anti-Dsg1 and anti-Dsg3 assays are useful in the diagnosis of pemphigus and provide information on the clinical phenotype of the disease. However, the sensitivity of EIA for detection of autoantibodies in bullous pemphigoid should be improved by the use of additional antigens or epitopes.

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Chronic Pemphigus Vulgaris of the Gingiva: A Case Report With a 6-Year Follow-up

Journal of Periodontology ◽

10.1902/jop.1983.54.11.685 ◽

1983 ◽

Vol 54 (11) ◽

pp. 685-689 ◽

Cited By ~ 11

Author(s):

Walter A. Orlowski ◽

Edward Bressman ◽

John L. Doyle ◽

Abram I. Chasens

Keyword(s):

Case Report ◽

Pemphigus Vulgaris

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Changes of trace element status during aging: results of the EPIC-Potsdam cohort study

European Journal of Nutrition ◽

10.1007/s00394-019-02143-w ◽

2019 ◽

Vol 59 (7) ◽

pp. 3045-3058 ◽

Cited By ~ 6

Author(s):

Julia Baudry ◽

Johannes F. Kopp ◽

Heiner Boeing ◽

Anna P. Kipp ◽

Tanja Schwerdtle ◽

...

Keyword(s):

Reference Ranges ◽

Linear Regression Models ◽

Serum Samples ◽

Time Points ◽

Supplement Use ◽

Healthy Elderly ◽

Age Related ◽

Longitudinal Variability ◽

Over Time

Abstract Purpose We aimed to evaluate age-dependent changes of six trace elements (TE) [manganese (Mn), iron (Fe), zinc (Zn), copper (Cu), iodine (I), and selenium (Se)] over a 20-year period. Methods TE concentrations were determined using repeated serum samples taken at baseline and after 20 years of follow-up from 219 healthy participants of the EPIC-Potsdam study, using inductively coupled plasma tandem mass spectrometry. For each TE, absolute and relative differences were calculated between the two time points, as well as the proportion of individuals within normal reference ranges. Interdependence between age-related TE differences was investigated using principal component analysis (PCA). Relationships between selected factors (lifestyle, sociodemographic, anthropometric factors, and hypertension) and corresponding TE longitudinal variability were examined using multivariable linear regression models. Results Median age of our study sample was 58.32 years (4.42) at baseline and 40% were females. Median Mn, Zn, Se concentrations and Se to Cu ratio significantly decreased during aging while median Fe, Cu, I concentrations and Cu to Zn ratio significantly increased. A substantial percentage of the participants, at both time points, had Zn concentrations below the reference range. The first PCA-extracted factor reflected the correlated decline in both Mn and Zn over time while the second factor reflected the observed (on average) increase in both Cu and I over time. Overall, none of the investigated factors were strong determinants of TE longitudinal variability, except possibly dietary supplement use, and alcohol use for Fe. Conclusions In conclusion, in this population-based study of healthy elderly, decrease in Mn, Zn, and Se concentrations and increase in Fe, Cu, and I concentrations were observed over 20 years of follow-up. Further research is required to investigate dietary determinants and markers of TE status as well as the relationships between TE profiles and the risk of age-related diseases.

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Granulocyte activation in bullous diseases: Release of granular proteins in bullous pemphigoid and pemphigus vulgaris

Journal of the American Academy of Dermatology ◽

10.1016/0190-9622(93)70170-x ◽

1993 ◽

Vol 29 (2) ◽

pp. 210-215 ◽

Cited By ~ 43

Author(s):

Wolfgang Czech ◽

Jörg Schaller ◽

Erwin Schöpf ◽

Alexander Kapp

Keyword(s):

Bullous Pemphigoid ◽

Pemphigus Vulgaris ◽

Bullous Diseases

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Childhood bullous pemphigoid: a case report and 10-year follow up

International Journal of Dermatology ◽

10.1111/j.1365-4632.2007.02718.x ◽

2007 ◽

Vol 46 (5) ◽

pp. 508-510 ◽

Cited By ~ 5

Author(s):

Ana María Sáenz ◽

Francisco González ◽

Antonietta Cirocco ◽

Inés María Tacaronte ◽

Javier Enrique Fajardo ◽

...

Keyword(s):

Case Report ◽

Bullous Pemphigoid

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Biologics in autoimmune bullous diseases: Current scenario

Indian Journal of Dermatology Venereology and Leprology ◽

10.25259/ijdvl_886_19 ◽

2021 ◽

Vol 0 ◽

pp. 1-10

Author(s):

Anuradha Bishnoi ◽

Dipankar De ◽

Sanjeev Handa ◽

Rahul Mahajan

Keyword(s):

Monoclonal Antibody ◽

Mycophenolate Mofetil ◽

Adverse Effects ◽

Pemphigus Vulgaris ◽

New Developments ◽

Autoimmune Bullous Diseases ◽

Current Scenario ◽

Bullous Diseases ◽

Anti Cd20

Autoimmune bullous diseases can be intraepidermal (pemphigus group of disorders) or subepidermal (pemphigoid group of disorders). The treatment of these disorders chiefly comprises corticosteroids and immunosuppressant adjuvants like azathioprine and mycophenolate mofetil. Autoantibodies are the main mediators of these diseases. Rituximab, a chimeric anti-CD20 monoclonal antibody targeting B-cells, has emerged as an excellent treatment option for refractory pemphigus vulgaris in the last decade. Since then, many new biologics have been proposed/explored for managing autoimmune bullous diseases. These hold potential for greater efficacy and lesser adverse effects than conventional immunosuppressants. In this review, we discuss the role of various biologics in the treatment of autoimmune bullous diseases, followed by a brief discussion on the drawbacks to their use and new developments in this area.

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Association of long-term exposure to circulating platinum with adverse late effects in testicular cancer survivors.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.4528 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. 4528-4528

Author(s):

Hink Boer ◽

Johannes H Proost ◽

Janine Nuver ◽

Sophie Bunskoek ◽

Joyce Q. Gietema ◽

...

Keyword(s):

Testicular Cancer ◽

Late Effects ◽

Compartment Model ◽

Population Pharmacokinetic ◽

Serum Samples ◽

Time Points ◽

Two Compartment Model ◽

Testicular Cancer Survivors

4528 Background: Successful platinum(Pt)-based chemotherapy for testicular cancer (TC) comes at the price of late cardiovascular morbidity and neurotoxicity. Damage induced by circulating Pt could be an etiological mechanism. We investigated the relation between circulating Pt and late effects. Methods: In 96 consecutive TC patients (med age 29 [17-53] at chemotherapy), 3 serum samples and a 24h-urine sample were collected on several time-points med 5 yrs (1-13) after treatment. Pt concentrations ([Pt]) were measured with a sensitive voltammetric method (Lancet 2000, 355:1075). Measured [Pt] combined with cisplatin dose, age, weight and height were analyzed simultaneously to construct a population pharmacokinetic (PK) model using NONMEM. Based on the PK parameters of each patient, individual [Pt] at 1, 3, 5, 10 yrs after start and AUC were calculated. Cardiovascular status, paresthesia and markers of vascular damage were assessed after a med follow-up (FU) of 9 yrs (3-15). Results: Decay of [Pt] was best described by a two compartment model. Mean terminal T1/2 was 3.7 (±0.3) yrs. At all time-points [Pt] correlated with cisplatin dose and renal function during treatment. [Pt] at 3 yrs correlated with systolic blood pressure (BP) (r=.32, p<0.01) and creatinine clearance (CRCL) (r=-.25, p=0.02) at FU. Patients with increased BP had higher Pt AUCs than patients with normal BP (table). Pt AUCs were higher in patients with paresthesia (table). Conclusions: Known late effects of cisplatin-based chemotherapy such as hypertension and paresthesia are related to higher circulating [Pt]. Long-term exposure to Pt is involved in healthy tissue damage in TC survivors. [Table: see text]

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Bullous pemphigoid in a 3-month-old infant: case report and literature review of this dermatosis in childhood

Anais Brasileiros de Dermatologia ◽

10.1590/abd1806-4841.20132378 ◽

2013 ◽

Vol 88 (6) ◽

pp. 961-965 ◽

Cited By ~ 10

Author(s):

Eugenio Galdino de Mendonça Reis-Filho ◽

Tainah de Almeida Silva ◽

Luiza Helena de Lima Aguirre ◽

Carmelia Matos Santiago Reis

Keyword(s):

Case Report ◽

Bullous Pemphigoid ◽

Immunohistochemical Staining ◽

Collagen Type ◽

Laboratory Data ◽

Collagen Type Iv ◽

Prognostic Features ◽

Type Iv ◽

Bullous Diseases ◽

Infant Case

Bullous pemphigoid is an autoimmune subepidermal blistering dermatosis that is uncommon in childhood. We report a case of a female infant, 3 months old, which presented clinical and laboratory data for the confirmatory diagnosis of bullous pemphigoid. The authors used immunohistochemical staining for collagen type IV that allowed the differentiation of bullous pemphigoid from other subepidermal bullous diseases. Opportunely we review the clinical, immunological, therapeutic and prognostic features of this pathology in children.

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