scholarly journals Pemphigus Vulgaris Presented with Cheilitis

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Zaheer Abbas ◽  
Zahra Safaie Naraghi ◽  
Elham Behrangi
Keyword(s):  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Lower Lip ◽  
Additional Lesion ◽  
Autoimmune Blistering Disease ◽  
Systemic Corticosteroids ◽  
Erosive Lichen Planus ◽  
Blistering Disease ◽  
One Year

Background. Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. In 50 to 70% of cases, the initial manifestations of pemphigus vulgaris are oral lesions which may be followed by skin lesions. But it is unusual for the disease to present with initial and solitary persistent lower lip lesions without progression to any other location.Main Observations. We report a 41-year-old woman with dry crusted lesions only on the lower lip, clinically resembling actinic cheilitis and erosive lichen planus, but histopathological evaluation showed unexpected results of suprabasal acantholysis and cleft compatible with pemphigus vulgaris. We treated her with intralesional triamcinolone 10 mg/mL for 2 sessions and 2 g cellcept daily. Patient showed excellent response and lesions resolved completely within 2 months. In one-year follow-up, there was no evidence of relapse or any additional lesion on the other sites.Conclusion. Cheilitis may be the initial and sole manifestation of pemphigus vulgaris. Localized and solitary lesions of pemphigus vulgaris can be treated and controlled without systemic corticosteroids.

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

KYAMC Journal ◽  
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

scholarly journals Pemphigus Vulgaris as Oral Mucosal Ulcer Progressive to Skin Lesion:

2014 ◽  
Vol 3 (2) ◽  
pp. 43-47
Author(s):  
SM Anwar Sadat ◽  
Akhter Imam
Keyword(s):  
Public Health ◽  
Pemphigus Vulgaris ◽  
Health It ◽  
Major Threat ◽  
Floor Of The Mouth ◽  
Autoimmune Blistering Disease ◽  
Life Threatening ◽  
History Of ◽  
Blistering Disease ◽  
One Year

Among all the life threatening diseases, autoimmune diseases are among most notorious and complicated diseases. As these diseases have no specific cause or responsible organism, it is therefore very difficult to distinguish and diagnose and thus stands as a major threat for public health. It is assumed that the reason for these diseases may be from genetic to environmental factors, from stress to idiopathic origin. Pemphigus Vulgaris(PV) is such an autoimmune blistering disease that frequently affects the mucous membrane and skin. Very often it starts from oral cavity and Dentists therefore the first to recognize. This paper describes the case of a patient presenting with a one-year history of painful ulcerated gingiva, tongue, floor of the mouth and even on GIT who is finally diagnosed as having PV spreading to generalized skin. DOI: http://dx.doi.org/10.3329/updcj.v3i2.17999 Update Dent. Coll. j: 2013; 3 (2): 43-47

scholarly journals Orthodontic camouflage of skeletal Class III malocclusion with miniplate: a case report

2016 ◽  
Vol 21 (4) ◽  
pp. 89-98 ◽  
Author(s):  
Marcel Marchiori Farret ◽  
Milton M. Benitez Farret ◽  
Alessandro Marchiori Farret
Keyword(s):  
Orthognathic Surgery ◽  
Class Iii ◽  
Class Iii Malocclusion ◽  
Lower Lip ◽  
Facial Profile ◽  
Skeletal Class ◽  
Mandibular Teeth ◽  
One Year ◽  
Angle Class Iii Malocclusion

ABSTRACT Introduction: Skeletal Class III malocclusion is often referred for orthodontic treatment combined with orthognathic surgery. However, with the aid of miniplates, some moderate discrepancies become feasible to be treated without surgery. Objective: To report the case of a 24-year-old man with severe skeletal Angle Class III malocclusion with anterior crossbite and a consequent concave facial profile. Methods: The patient refused to undergo orthognathic surgery; therefore, orthodontic camouflage treatment with the aid of miniplates placed on the mandibular arch was proposed. Results: After 18 months of treatment, a Class I molar and canine relationship was achieved, while anterior crossbite was corrected by retraction of mandibular teeth. The consequent decrease in lower lip fullness and increased exposure of maxillary incisors at smiling resulted in a remarkable improvement of patient's facial profile, in addition to an esthetically pleasing smile, respectively. One year later, follow-up revealed good stability of results.

Sweet Syndrome and Pemphigus Vulgaris

2012 ◽  
Vol 16 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Ashley O'toole ◽  
Maureen O'malley
Keyword(s):  
Clinical Presentation ◽  
Pemphigus Vulgaris ◽  
Neutrophilic Dermatosis ◽  
Rare Presentation ◽  
Sweet Syndrome ◽  
Autoimmune Blistering Disease ◽  
Mucous Membranes ◽  
Blistering Disease ◽  
Erythematous Plaques ◽  
New Onset

Background: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. Sweet syndrome is an uncommon reactive neutrophilic dermatosis. Objective: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome. Methods: Review of the literature using the PubMed and Medline databases. Results: The clinical presentation of new-onset Sweet syndrome in a patient with pemphigus has been reported in the literature only on one other occasion. Conclusion: The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease.

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

2002 ◽  
Vol 13 (5) ◽  
pp. 397-408 ◽  
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.

Sirolimus Combined with Dexamethasone for the Treatment of a Patient with Interferon Resistant Hydroa Vacciniform-Like Lymphoma

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4992-4992
Author(s):  
Zhigang Liu ◽  
Yongqian Jia ◽  
Yuping Gong ◽  
Yong Guo ◽  
Jiazhuo Liu ◽  
...  
Keyword(s):  
T Cell ◽  
Conflicts Of Interest ◽  
Serum Biochemistry ◽  
Skin Lesions ◽  
Cytotoxic T Cell ◽  
Cell Phenotype ◽  
Ebv Dna ◽  
One Year

Abstract Abstract 4992 Hydroa vacciniform-like lymphoma is an unusual pediatric cutaneous T-cell lymphoma, which rarely involved with adults. Chemotherapy and/or radiotherapy had little or no benefit. Patients may have a response to α-interferon. We report here an adult case with interferon resistant hydroa vacciniform-like lymphoma that has been successfully treated with sirolimus, an mTOR pathway inhibitor. The patient is a thirty-five year old women,she begun with recurrent cutanous rash and small vesicule around her mouth and nose 3 years ago. One year before, the symptoms were exacerbated with obvious vesiculopapular eruption, edematous, blisters, ulcers, scarring and crusts in her face. Scattered vesiculopapular could be seen in her upper chest and extremities accompanied with fever and submandibular lymphadenopathy. Facial skin biopsy showed angiocentric infiltrates from the epidermis to the subjacent dermis. The infiltrate cells showed a cytotoxic T-cell phenotype, with positive TCR gene rearrangement and EBER expression in situ hybridization. EBV serum test showed EBV-IgA and IgG positive, but serum EBV-DNA was negative. Patient was given α-interferon 300 million units, intramuscularlly, two times a week, plus prednisone 30mg per day, orally. Patients' skin lesions were improved, but present recurrent episodes of papulovesicular eruptions. For the past 3 month patient had a recurrent high fever and exacerbated vesiculopapular eruptions and blisters. Laboratory test showed anemia and neutropenia, serum biochemistry and marrow examination confirmed that a lymphoma associated hematophagocytosis was complicated. Sirolimus was started at an initial dose of 1.5mg, orally, every 12 hours, combined with dexamethasone 10mg/day, intravenously. Three days later the fever begun to resolved and the skin lesion gradually subsided. Two weeks later the skin lesions disappeared and the dose of sirolinmus begun to tapered to 1.0mg, orally, every 12 hours, with prednisone 30mg/day, orally. By three month of follow-up the patient still on stable and be observed. Disclosures: No relevant conflicts of interest to declare.

scholarly journals A Possible Role for CD8+ T Lymphocytes in the Cell-Mediated Pathogenesis of Pemphigus Vulgaris

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Federica Giurdanella ◽  
Luca Fania ◽  
Maria Gnarra ◽  
Paola Toto ◽  
Daniela Di Rollo ◽  
...  
Keyword(s):  
T Cells ◽  
Cd8 T Cells ◽  
Immunohistochemical Staining ◽  
Inflammatory Infiltrate ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Passive Transfer ◽  
Autoimmune Blistering Disease ◽  
Deficient Mice

Pemphigus vulgaris (PV) is an autoimmune blistering disease whose pathogenesis involves both humoral and cell-mediated immune response. Though the pathogenetic role of autoantibodies directed against desmoglein 3 is certain, a number of other factors have been suggested to determine acantholysis in PV. In this study we examined the possible role of CD8+ T cells in the development of acantholysis by a passive transfer of PV autoantibodies using CD8 deficient mice, and we also studied the inflammatory infiltrate of PV skin lesions by immunohistochemical staining. The results of the immunohistochemical staining to study the expression of CD3, CD4, and CD8 in PV skin lesions showed that CD4+ are more expressed than CD8+ in the inflammatory infiltrate of PV lesions, confirming the data of the previous literature. The passive transfer study showed a lower incidence of pemphigus in the group of CD8 deficient mice compared to the control one of wild-type mice. These results suggest that CD8+ T cells may play a role in the pathogenesis of PV, perhaps through the Fas/FasL pathway.

ICOS Gene Polymorphism May Be Associated with Pemphigus

2010 ◽  
Vol 14 (6) ◽  
pp. 291-297 ◽  
Author(s):  
Joanna Narbutt ◽  
Aleksandra Lesiak ◽  
Izabela Klich ◽  
Jolanta Dorota Torzecka ◽  
Anna Sysa-Jedrzejowska ◽  
...  
Keyword(s):  
Gene Polymorphism ◽  
Pemphigus Vulgaris ◽  
Control Group ◽  
Pemphigus Foliaceus ◽  
Autoimmune Blistering Disease ◽  
Genes Encoding ◽  
Blistering Disease ◽  
Ctla4 Gene ◽  
Genetically Determined ◽  
The Relationship

Background: Pemphigus is an autoimmune blistering disease mediated by circulating IgG autoantibodies directed against desmogleins 3 and/or 1. As pemphigus is a T cell–mediated disease, one may assume that genetically determined disregulation of costimulatory signal may be involved in its pathogenesis. Objective: The aim of the present study was to evaluate the relationship between polymorphisms in genes encoding costimulatory receptors, CTLA4 and ICOS, and pemphigus in the Polish population. Methods: The study included 54 patients with pemphigus: 40 with pemphigus vulgaris (PV) and 14 with pemphigus foliaceus (PF). Additionally, 176 healthy unrelated blood donors served as controls. +49A/G CTLA4 and IVS1+173 ICOS polymorphisms were identified using a modified polymerase chain reaction–restriction fragment-length polymorphism. Results: Analysis of the frequency of genotypes and alleles of +49A/G CTLA4 gene polymorphism showed no statistically significant differences between the PV and PF patients and the controls. The distribution of genotypes in IVS1+173 ICOS polymorphisms was significantly different in both PV ( p < .01) and PF ( p = .0004) patients when compared to controls. The carriers of the allele C were more frequent in PV or PF in comparison with the control group ( p < .001 for both groups). Conclusions: Our results suggest that genetically determined abnormal function of costimulatory receptors in T cells may be associated with the pathogenesis of pemphigus.

Ustekinumab-induced Sarcoidosis in a Patient with Psoriatic Arthritis

2020 ◽  
Vol 15 (2) ◽  
pp. 163-166 ◽  
Author(s):  
Senol Kobak ◽  
Huseyin Semiz
Keyword(s):  
Psoriatic Arthritis ◽  
Skin Lesions ◽  
Chronic Inflammatory Disease ◽  
Acute Phase Reactants ◽  
Caseating Granuloma ◽  
Endobronchial Ultrasonography ◽  
Chest X Ray ◽  
Dose Corticosteroid ◽  
One Year

Background: Psoriatic Arthritis (PsA) is a chronic inflammatory disease that may affect different joints. Sarcoidosis is a Th-1 cell-related chronic granulomatous disease characterized by non-caseating granuloma formation. The coexistence of both the diseases is a rare entity. Ustekinumab, an IL12 / 23 inhibitor, has shown efficacy and safety in the treatment of PsA. Objective: This study presents a case with ustekinumab-induced sarcoidosis in a patient with PsA. Case Report: A 52 years old female patient with complaints of pain and swelling of the wrists, MCP, PIP and DIP joints and skin lesions was referred to our Rheumatology clinic. On her medical history, she had been under follow up for 5 years with the diagnosis of psoriasis and one year ago, she started to receive ustekinumab prescribed by a dermatologist. On physical examination, she had psoriasis skin lesions and arthritis of both wrists, MCP, PIP, DIP joints. Bilateral hilar lymphadenopathies were detected in the chest X-ray and thorax computed tomography. In laboratory tests, acute phase reactants and serum angiotensin-converting enzyme levels were high. Endobronchial ultrasonography biopsy was performed and non-caseating granuloma consistent with sarcoidosis was reported. Ustekinumab was discontinued, methotrexate and low-dose corticosteroid were started. The patient was clinically stable in the 6th month of the treatment and the findings were regressed. Conclusion: Sarcoidosis development appears to be a new paradoxical effect of ustekinumab therapy, being another biological agent.

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