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.

An Unusual Case of Pemphigus Vulgaris

<b><i>Introduction:</i></b> Pemphigus is a potential life-threatening skin disorder belonging to the group of the autoimmune bullous diseases affecting the skin and mucosa. The most common subtypes are pemphigus foliaceus (PF) and pemphigus vulgaris. <b><i>Case Presentation:</i></b> We present the case of a young woman with scalp manifestations diagnosed as seborrhiasis who came to our office where a more careful history and clinical examination directed us toward another diagnostic suspicion. The histological examination confirmed our suspicion of pemphigus and therefore we believe it is important to report our experience to avoid misdiagnosis. <b><i>Discussion/Conclusion:</i></b> Our case may be useful in the literature to identify cases of PF with atypical manifestations that may mimic other diseases.

Case Report: Circulating Anti-SARS-CoV-2 Antibodies Do Not Cross-React With Pemphigus or Pemphigoid Autoantigens

It is hypothesized that SARS-CoV-2 has the potential to elicit autoimmunity due to molecular mimicry between immunogenic proteins of the virus and human extracellular molecules. While in silico and in vitro evaluation of such immune cross-reactivity of human antibodies to SARS-CoV-2 proteins with several different tissue antigens has been described, there is limited information specifically pertaining to the immunological effects of COVID-19 and vaccines against SARS-CoV-2 on the development of autoimmune bullous diseases (AIBDs). Twelve seropositive post-COVID-19 individuals and 12 seropositive healthy volunteers who received two doses of the mRNA COVID-19 vaccine from Pfizer-BioNTech have been included in this case series investigation. Serum samples of these blood donors were tested for autoantibodies to the main immunobullous autoantigens, i.e., desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, and type VII collagen. Our study revealed that none of the 24 anti-SARS-CoV-2 IgG-positive subjects had concomitant antibody reactivity with any of the tested autoantigens. These results argue against a relationship between SARS-CoV-2 infection/vaccines and AIBDs with respect to disease-triggering antibody cross-reactivity.

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

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. It requires a combination of three major features: clinical, histopathological, and immunological. Clinically, oral lesions are the first manifestations of the disease in 50-90% of the patients with widespread blisters affecting the oral mucosa. On the skin, lesions are characterized by flaccid blisters that rapidly progress into erosions and crust formation. Umbilical lesions as a clinical manifestation of PV are peculiar and have rarely been reported, and they are not yet completely elucidated. Umbilical region involvement in patients with pemphigus was assessed in a limited study totalling just 10 patients. This localisation may be a valuable hint easing the diagnosis at the clinical level for patients with oral mucosal blisters. Dentists must be familiar with the clinical manifestations of PV to make an early diagnosis and start an early treatment which determines the prognosis of the disease. To the best of the authors’ knowledge, the coexistence of these lesions with the oral lesions as a first sign of PV in the absence of skin involvement was reported in only one case of pemphigus vegetans (PVe). In this paper, we describe an observation of a female patient that was diagnosed with PV that begun with simultaneous oral and umbilical locations which coexisted for a period of 4 months before the appearance of other cutaneous lesions. We highlight the role of dentists, by being familiar with the clinical manifestations of PV, to make an early diagnosis to start an early treatment which determines the prognosis of the disease and to follow closely the evolution of lesions to change treatment if required. We also discuss the clinical, histological, and immunological features of the disease that enabled the differential diagnosis as well as the appropriate therapeutic management.

Acquired Hemophilia in Association With Pemphigus Vulgaris: An Uncommon Coexistence: A Case Report

Acquired Hemophilia (AHA) is a relatively rare disease that occurs in patients with no previous family history of hemophilia. The spontaneous development of autoantibodies (IgG1 and IgG4) against factor VIII has been reported as the most probable cause of AHA. AHA has been reported in association with other conditions, including some autoimmune bullous skin diseases, such as bullous pemphigoid, pemphigus vulgaris, and pemphigus foliaceous. To the best of our knowledge, only 21 cases of AHA with skin autoimmune bullous diseases have been reported so far. Herein, we report a 63-year-old male with a previous history of pemphigus vulgaris who developed large ecchymotic areas on his lower abdomen and forearms after the second infusion of rituximab. Based on coagulation factors evaluation, he was diagnosed with AHA. Treatment with factor VII led to the improvement in his coagulation status, but unfortunately, he passed away because of inferior wall myocardial infarction four days later.

A Retrospective Study of Pemphigus in a Tertiary Care Centre in South India

BACKGROUND Pemphigus is a group of rare, life-threatening autoimmune bullous diseases of the skin and mucosa which result in intraepidermal blistering. Associated autoimmune conditions and the extensive mucocutaneous detachment impair the quality of life. Immunosuppressive treatment adds to the morbidity in patients. We wanted to assess the clinical presentation of pemphigus among the study subjects along with the outcome of pemphigus patients with the extent of the disease and results of pus culture. METHODS Retrospective analysis of case records of patients with pemphigus in a tertiary care hospital during a period of 3 years was done in 2015. Demographic data, clinical findings, underlying medical disorders, details of deaths, investigation reports and treatment details were recorded. RESULTS Common age group affected was 51-60 years (31.5 %) among a total of 54 pemphigus patients. There were 26 males and 28 (51. 9 %) females and in majority of the patients (44.4 %) duration was less than 6 months. Out of 47 patients, oral mucosal involvement was grade 1 in 20 (37 %) cases, grade 2 in 12 (22.2 %) and in 15 (27.8 %) patients it was grade 3.Histopathological examination showed suprabasal cleft in 82.1 %, subcorneal blister in 5.7 %, acantholytic cells in 48.6 % and row of tombstone appearance in 9.4 %. Out of 36 patients, DIF test reported intercellular IgG in 65 % and C3 in 50 % .Diabetes was found in 14 (25.9 %), candidiasis in 12 (22.2 %), thyroid disease in 6 (11.1 %) and hypertension in 3 patients. Three patients died. Among 10 patients with more than 30 % body surface area involvement, 1 (10 %) patient died. Two (13 %) deaths occurred among 15 patients in whom pus C&S was positive. CONCLUSIONS Pemphigus patients were of slightly higher (51 - 70 years) age group and the 3 patients died were females. Autoimmune disorders like diabetes and thyroid disorders were found among many patients and their family members. Infection could be an important cause of death, but no statistically significant association was found in the present study. KEYWORDS Pemphigus, Steroids, Comorbidity, Deaths

The epidemiology of autoimmune bullous diseases in Sudan between 2000 and 2016

Objectives Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to describe the epidemiological profile and to estimate the burden of autoimmune bullous diseases in Sudan. Methods This was a retrospective cross-sectional study conducted at Khartoum Dermatological and Venereal Diseases Teaching Hospital. We used routinely collected health care data, and included all patients with an autoimmune bullous disease who presented to the hospital between 2001 and 2016. Results Out of the 4736 patients who were admitted to the hospital during the study period, 923 (19.5%) had an autoimmune bullous disease. The average rate of patients at the hospital was 57.7 per year representing 1.3 per 100,000 population per year. After exclusion of patients where the final diagnosis was missing, 585 were included in the further analysis. Pemphigus vulgaris was the most common disease (50.9%), followed by bullous pemphigoid (28.2%), linear IgA disease/chronic bullous disease of childhood (8.4%), and pemphigus foliaceous (8.2%). Pemphigoid gestationis and IgA pemphigus constituted 1.4% and 1.2% of the cohort, respectively. Paraneoplastic pemphigus, mucous membrane pemphigoid, lichen planus pemphigoidis, bullous systemic lupus erythematosus, and dermatitis herpetiformis were rare. None of the patients had epidermolysis bullosa acquisita. Conclusions The clinico-epidemiological characteristics vary among the types of autoimmune bullous diseases. Females were more predominant in most of them. Sudanese patients tended in general to present at a younger age than other populations. The pool of Sudanese patients with autoimmune bullous diseases is large which requires investigation for the local risk factors and presents a field for future trials.

Biologics in autoimmune bullous diseases: Current scenario

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.