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.

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Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review

BMC Veterinary Research ◽

10.1186/s12917-020-02677-w ◽

2020 ◽

Vol 16 (1) ◽

Author(s):

Heng L. Tham ◽

Keith E. Linder ◽

Thierry Olivry

Keyword(s):

Clinical Signs ◽

Pemphigus Vulgaris ◽

High Dose ◽

Paraneoplastic Pemphigus ◽

Autoimmune Bullous Diseases ◽

Human Counterpart ◽

Deep Layers ◽

Bullous Diseases ◽

Pemphigus Vegetans ◽

Autoimmune Dermatoses

AbstractPemphigus is the term used to describe a group of rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and erosions of the mucous membranes and/or skin. When the autoantibodies target desmosomes in the deep layers of the epidermis, deep pemphigus variants such as pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus develop. In this article, we will review the signalment, clinical signs, histopathology and treatment outcome of pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus in dogs, cats and horses; where pertinent, we compare the animal diseases to their human homologue. Canine, feline and equine pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus have many features similar to the human counterpart. These chronic and often relapsing autoimmune dermatoses require aggressive immunosuppressive therapy. In animals, the partial-to-complete remission of pemphigus vulgaris and pemphigus vegetans has been achieved with high dose glucocorticoid therapy, with or without adjunct immunosuppressants; the prognosis is grave for paraneoplastic pemphigus.

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Oral Lesions in Autoimmune Bullous Diseases: An Overview of Clinical Characteristics and Diagnostic Algorithm

American Journal of Clinical Dermatology ◽

10.1007/s40257-019-00461-7 ◽

2019 ◽

Vol 20 (6) ◽

pp. 847-861 ◽

Cited By ~ 6

Author(s):

Hanan Rashid ◽

Aniek Lamberts ◽

Gilles F. H. Diercks ◽

Hendri H. Pas ◽

Joost M. Meijer ◽

...

Keyword(s):

Clinical Characteristics ◽

Diagnostic Algorithm ◽

Oral Lesions ◽

Autoimmune Bullous Diseases ◽

Bullous Diseases

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Prosthodontic Rehabilitation of an Oral Pemphigus Vulgaris Patient

International Journal of Prosthodontics and Restorative Dentistry ◽

10.5005/jp-journals-10019-1024 ◽

2011 ◽

Vol 1 (2) ◽

pp. 128-131

Author(s):

Ufuk Ates ◽

Bulem Yuzugullu

Keyword(s):

Early Diagnosis ◽

Dental Care ◽

Oral Hygiene ◽

Systemic Treatment ◽

Clinical Manifestations ◽

Pemphigus Vulgaris ◽

Oral Disease ◽

Dental Management ◽

Increased Risk ◽

Hygiene Measures

ABSTRACT Pemphigus vulgaris is a rare autoimmune mucocutaneous disease with high mortality if untreated. Dental management is complicated due to involvement of oral mucosa, increased risk of oral disease and difficulty in rendering dental care. A male patient with exacerbated characteristics was rehabilitated with full mouth restorations following systemic treatment of pemphigus vulgaris. Owing to the nature of the disease and discomfort in providing adequate oral hygiene measures, dietary intake and masticatory function, it is imperative to rehabilitate the patient with well-designed prosthesis. Although these diseases are relatively uncommon, clinicians must be sufficiently familiar with clinical manifestations to ensure early diagnosis and treatment.

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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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Clinical Study on Outcome of Treatment for Herpes Zoster

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2021/47661.14791 ◽

2021 ◽

Author(s):

Kishan Rasubhai Ninama ◽

Rashmi Samir Mahajan ◽

Atmakalyani Rashmi Shah ◽

Apexa Prakash Jain

Keyword(s):

Herpes Zoster ◽

Lupus Erythematosus ◽

De Novo ◽

Clinical Manifestations ◽

Pemphigus Vulgaris ◽

Clinical History ◽

Skin Lesions ◽

Enzyme Linked Immunosorbent Assay ◽

Oral Acyclovir ◽

Varicella Zoster

Introduction: Herpes Zoster (HZ) is caused by reactivation of Varicella Zoster Virus (VZV). It is characterised by occurrence of grouped vesicles on erythematous base which involves the entire dermatome innervated by a single spinal or cranial sensory ganglion and is associated with radicular pain. Antivirals (Acyclovir, Famciclovir and Valacyclovir) started within 72 hours of onset of lesions are the agents of choice. Aim: To study the clinical manifestations, comorbidities, efficacy and safety of Acyclovir, complications and sequelae associated with HZ. Materials and Methods: A 3-year longitudinal cohort study was conducted in 212 adult patients (>18 years of age) suffering with HZ in the Department of Dermatology, Dhiraj General Hospital, Pipariya, Gujarat, India. In this study 212 patients with HZ were prescribed oral Acyclovir in a dose of 800 mg 5 times a day for 7 days. All patients were analysed in terms of clinical manifestations, pre-existing co-morbidities and incidence of complications. The clinical history and findings were recorded in a prestructured proforma. All patients were subjected to cytological examination (Tzanck smear) and Human immunodeficiency viruses (HIV) testing Enzyme-Linked Immunosorbent Assay (ELISA). Diagnosis was made primarily on the basis of clinical findings and presence of multinucleated giant cells in Tzanck smear. All the patients were treated with Oral Acyclovir. Cases were followed-up fortnightly for six weeks and evaluated for relief of symptoms, treatment outcome and complications/sequelae. Results: Two hundred and twelve cases were studied. One hundred and forty-two cases were in the 4th and 5th decades of life. Sixty-three cases had comorbidities like diabetes mellitus in 31, autoimmune diseases like pemphigus vulgaris, systemic lupus erythematosus, rheumatoid arthritis and inflammatory bowel disease in 19 and AIDS in 8 cases. Five cases had malignancy/lymphomas and were receiving chemotherapy for the same. In the majority, HZ occurred de novo without any comorbidities. The most common dermatomes involved were cervical and thoracic. Out of 212 cases Oral Acyclovir 800 mg was well tolerated by 74. Most common complication was Postherpetic Neuralgia (PHN), seen in 80 cases. Conclusion: The treatment of HZ with Oral Acyclovir 800 mg 5 times a day for 7 days is efficacious for healing of skin lesions and also reduces the chances of PHN if instituted within 72 hours.

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A Clinical Profile and Co-morbidities of Pemphigus Vulgaris Patients: A Study of 35 Cases

KYAMC Journal ◽

10.3329/kyamcj.v10i2.42789 ◽

2019 ◽

Vol 10 (2) ◽

pp. 106-109

Author(s):

Md Abdur Razzaque ◽

Zulfikar Ali ◽

Kazi Shihab Uddin ◽

Md Imtiajul Islam ◽

Sajib Kumar Nath

Keyword(s):

Pemphigus Vulgaris ◽

Skin Lesions ◽

Cutaneous Lesions ◽

Female Ratio ◽

Medical College ◽

Autoimmune Blistering Disease ◽

Blistering Disease ◽

High Index Of Suspicion ◽

Male To Female ◽

Sixth Decade

Background: Pemphigus vulgaris (PV), an autoimmune blistering disease involving the skin and mucosa. PV frequently begins with oral lesions and progresses to skin lesions. Autoimmune bullous skin disorders are associated with IgG or IgA auto- antibodies against distinct adhesion molecules of the epidermis and dermal epidermal basement membrane zone, respectively. These auto- antibodies lead to a loss of skin adhesion which shows up clinically as the formation of blisters or erosions. Objectives: To characterize the clinical parameters and co-morbidities of PV patients from a single tertiary medical centre in Bangladesh. Material and Methods: This observational study was done including 35 PV patients attending in the department of Dermatology and Venereology, Khwaja Yunus Ali Medical College Khawja Eunus Ali Medical College from 2010 to 2014. Thirty patients of pemphigus diagnosed clinically confirmed and treated over a 4-year period (2010-2014). Results: Majority of the patients 45.7% belongs to age group 41-50 years. Mean age 47.12±11.13. The male to female ratio in our study sample was 1:1.5. The youngest patient was 17 years old and the oldest 68. For both genders, the risk of onset peaked during the fifth and sixth decade of life. Out of 35 patients, 15(42.9%) was presented with mucosal lesions only, while 13 patients 37.1% had mucocutaneous lesions and 7 patients 20% had only cutaneous lesions. The most common comorbidies were hypertension 20.0%, osteoporosis 17.1%, and diabetes 8.6%, thyroid disease 8.6%, psoriasis 5.7%, rheumatoid arthritis 2.7%, rheumatic fever 2.9%, autoimmune hepatitis 2.9%, and myasthenia gravis 2.9%. Conclusion: The associated comorbidities of PV emphasize the need for dermatologists to keep a high index of suspicion and actively evaluate patients to determine their presence. KYAMC Journal Vol. 10, No.-2, July 2019, Page 106-109

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Regional variations in antigenic properties of skin. A possible cause for disease-specific distribution of skin lesions.

Journal of Experimental Medicine ◽

10.1084/jem.164.6.2125 ◽

1986 ◽

Vol 164 (6) ◽

pp. 2125-2130 ◽

Cited By ~ 34

Author(s):

L Sison-Fonacier ◽

J C Bystryn

Keyword(s):

Regional Differences ◽

Skin Diseases ◽

Pemphigus Vulgaris ◽

Skin Lesions ◽

The Body ◽

Cutaneous Lesions ◽

Specific Distribution ◽

Antigenic Properties ◽

Possible Cause ◽

Disease Specific

The possibility that the distribution of skin lesions in some cutaneous diseases is due to variations in the antigenic properties of skin was investigated by mapping the expression of the skin-specific pemphigus vulgaris and bullous pemphigoid antigens in different regions of the body. The expression of both antigens was relatively stable within the same region, but varied between regions in a pattern that was distinct for each antigen. For each antigen there was a correlation between regions of high expression and location of skin lesions in autoimmune diseases involving the antigen. The results indicate that there are marked regional differences in the antigenic properties of skin and suggest this may influence the distribution of cutaneous lesions in some skin diseases.

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Desmoglein Compensation Theory: An explanations for Early Appearance of Oral Lesions as Compared to Skin Lesions in Pemphigus Vulgaris

International Archives of BioMedical and Clinical Research ◽

10.21276/iabcr.2016.2.3.3 ◽

2016 ◽

Vol 2 (3) ◽

Cited By ~ 1

Author(s):

Safia Siddiqui ◽

◽

Mohammad Asad Haroon ◽

Shamim ul Hasan ◽

Aeman Khalid

Keyword(s):

Pemphigus Vulgaris ◽

Skin Lesions ◽

Oral Lesions ◽

Early Appearance ◽

Compensation Theory

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Analysis of clinical and diagnostic features and criteria for the effectiveness of treatment of autoimmune bullous dermatoses pemphigus group

Ukrainian Journal of Dermatology Venerology Cosmetology ◽

10.30978/ujdvk2021-1-51 ◽

2021 ◽

pp. 51-57

Author(s):

М.Е. Zapolsky ◽

М.М. Lebediuk ◽

N.B. Prokofyeva ◽

V.V. Goncharenko ◽

S.V. Nerubashchenko

Keyword(s):

Immunofluorescence Microscopy ◽

Clinical Manifestations ◽

Diagnostic Algorithm ◽

Pemphigus Vulgaris ◽

Diagnostic Features ◽

Iga Pemphigus ◽

Modern Classification ◽

Pemphigus Erythematosus ◽

Pemphigus Vegetans

The modern classification of autoimmune bullous dermatoses is represented by three groups of diseases that have not only clinical, but also histomorphological, histochemical and immunogenetic similarities. Autoimmune bullous dermatoses are a group of heterogeneous diseases accompanied by the formation of blisters and erosions on the skin and/or mucous membranes under the influence of specific autoimmune complexes. The main clinical manifestations and diagnostic markers of autoimmune bullous dermatoses (ABD) belonging to the pemphigus group are considered.Objective — to analyze the prevalence of clinical forms of pemphigus in the southern region of Ukraine for the period from 2010 to 2020.Materials and methods. The observation group consisted of 88 patients aged 21 to 75 years. The features of clinical manifestations of typical and atypical forms of pemphigus have been studied, a diagnostic algorithm and criteria for the effectiveness of treatment in the early stages of the disease have been developed.Results and discussion. Pemphigus vulgaris was found in 61 (69.3 %), pemphigus vegetans — in 7 (7.9 %), pemphigus erythematosus — in 6 (6.8 %), herpetiform pemphigus — in 4 (4.5 %), IgA pemphigus — in 3 (3.4 %), paraneoplastic pemphigus — in 3 (3.4 %) patients.Conclusions. Modern clinical diagnostic features of ABD (pemphigus group) require the inclusion of immunofluorescence microscopy, direct and indirect enzyme immunoassay in laboratory screening. Not only positive clinical dynamics, but also the rate of disappearance of autoantibodies that induce the diseases (desmoglein, desmoplakin, periplakin, etc.) should be considered the criteria of the effectiveness of treatment of patients with ABD. At the same time, knowledge of the clinical features of ABD and the diseases that mimic them remains an important aspect.

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