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.

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.

scholarly journals MANIFESTASI DAN PENATALAKSANAAN PEMFIGUS VULGARIS DI RONGGA MULUT

2018 ◽  
Vol 2 (2) ◽  
pp. 117-124
Author(s):  
Indra Gunawan ◽  
Riani Setiadhi
Keyword(s):  
Case Report ◽  
Early Recognition ◽  
Pemphigus Vulgaris ◽  
Oral Lesions ◽  
Oral Manifestations ◽  
Skin Involvement ◽  
Mucous Membranes ◽  
Blistering Disease ◽  
Mouth Wash ◽  
Desmoglein 3

Background : Pemphigus Vulgaris (PV) is an autoimmune intraepithelial blistering disease involving the skin and mucous membranes. Oral lesions could be the first sign of the disease followed by the involvement of skin and other mucosa sites.Objectives : This is oral manifestations of pemphigus vulgaris case report, intended to help clinicians to recognize and treat the oral lesions.Case Reported : Male 27 yo admitted to RSUP Dr.Hasan Sadikin hospital with multiple blisters on the skin and oral mucosa, was diagnosed with Pemphigus Vulgaris.Case Management : Patient was treated with steroid mouth wash and oral paste. After two months of treatment, the patient was fully recovered from oral lesion.Discussion : In PV, autoantibodies are produced against desmosomes spesifically desmoglein 3 which responsible for holding the cells of the epithelium together. The loss of adhesive function due to anti Dsg 3 antibodies result in bulla formation on the oral mucosa.The aetiology for PV is still uncertain. Conclusion :Early recognition and treatment of oral lesions is important as it may prevent skin involvement. Early treatment, patient’s compliance and multi disciplinal teamwork ensure the treatment succes for this disease

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 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 Pravastatin-Induced Eczematous Eruption Mimicking Psoriasis

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Michael P. Salna ◽  
Hannah M. Singer ◽  
Ali N. Dana
Keyword(s):  
Case Report ◽  
Clinical Presentation ◽  
The Elderly ◽  
Case Presentation ◽  
Dermatology Clinic ◽  
Systemic Treatments ◽  
Eczematous Dermatitis ◽  
Erythematous Plaques ◽  
New Onset

Background. Statins, an example of the most commonly prescribed medications to the elderly, are not without side effects. Dermatologic events are often overlooked as arising from medications, particularly those which are taken chronically. Moreover, elderly patients are prone to pharmacologic interactions due to multiple medications. In this report, we describe a case of a statin-induced eczematous dermatitis with a psoriasis-like clinical presentation and review the skin manifestations that may arise from statin therapy.Case Presentation. An 82-year-old man with gout and hypercholesterolemia presented to dermatology clinic with new onset of pruritic, scaly erythematous plaques bilaterally on the extensor surfaces of his arms. He had never had similar lesions before. Despite various topical and systemic treatments over several months, the rash continued to evolve. The patient was then advised to discontinue his long-term statin, which led to gradual resolution of his symptoms. He was subsequently diagnosed with statin-induced eczematous dermatitis.Conclusions. This case report describes an adverse cutaneous reaction to statins that is rarely reported in the literature. Medications, including longstanding therapies, should be suspected in cases of refractory dermatologic lesions.

Mean Platelet Volume in Pemphigus Vulgaris

Angiology ◽  
2017 ◽  
Vol 69 (4) ◽  
pp. 303-307 ◽  
Author(s):  
Khalaf Kridin ◽  
Wesal Shihade ◽  
Shira Zelber-Sagi
Keyword(s):  
Mean Platelet Volume ◽  
Pemphigus Vulgaris ◽  
Platelet Volume ◽  
Inflammatory Parameters ◽  
Autoimmune Blistering Disease ◽  
Before And After ◽  
Blistering Disease ◽  
The Mean ◽  
Study Participants ◽  
Control Study

Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucous membranes. The mean platelet volume (MPV) is an index of platelet activation reported to be influenced by inflammation. We investigated the MPV in patients with PV relative to controls. This case–control study included 160 patients with PV and 640 age- and sex-matched controls. The MPV, platelet count, and other inflammatory parameters were measured for all study participants. A significantly decreased MPV was observed in patients with PV compared with controls, 9.1 (1.5) fL versus 10.3 (1.4) fL, P < .001. A significant negative association between MPV and PV was demonstrated in multivariate analysis (odds ratio, 0.59; 95% confidence interval, 0.51-0.68, P < .0001). Patients having PV with laryngeal involvement had significantly lower MPVs than those without involvement of the larynx, 8.81 (1.5) fL versus 9.32 (1.4) fL, P = .032. Disease activity (before and after treatment) does not affect the MPV. Patients with PV showed decreased MPV values compared to healthy controls. The MPV may be a feasible diagnostic marker in patients with PV.

scholarly journals A Sweet Voice: Acute Febrile Neutrophilic Dermatosis of the Larynx

2021 ◽  
pp. 014556132110220
Author(s):  
Brian A. Walker ◽  
Lindsey B. Stull ◽  
J. Peyton Hines
Keyword(s):  
Whole Body ◽  
Neutrophilic Dermatosis ◽  
Unknown Etiology ◽  
Sweet Syndrome ◽  
Documented Case ◽  
Acute Febrile Neutrophilic Dermatosis ◽  
Erythematous Plaques ◽  
Laryngeal Involvement

Significance Statement Acute febrile neutrophilic dermatosis (Sweet syndrome) is a rare idiopathic condition characterized by fever and whole-body rash of tender erythematous plaques of unknown etiology. Otorhinolaryngologic manifestations of the disease can be severe, yet they are sparsely reported in the literature. We present the first documented case of laryngeal involvement of Sweet syndrome.

scholarly journals Case Report: A rare presentation and diagnosis of gingival melanoacanthoma caused by teeth whitening strips: A Case Report

F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 1452
Author(s):  
Hamad Albagieh ◽  
Ashwag Aloyouny ◽  
Shatha Alharthi
Keyword(s):  
Case Report ◽  
Oral Cavity ◽  
Clinical Presentation ◽  
Rare Presentation ◽  
Pigmented Lesions ◽  
Pigmented Lesion ◽  
Causative Factors ◽  
Local Sources ◽  
Mucous Membranes

Background: Oral melanoacanthoma is not common. It occurs mostly on the buccal mucosa. Since it happens suddenly and progresses rapidly, it clinically resembles melanoma. Melanoacanthoma occurs in regions susceptible to trauma. The clinical presentation of the lesion is not enough to diagnose it; therefore, tissue biopsy is necessary to exclude malignancy. Case report: We report a case of oral melanoacanthoma in a rare mucosal location in a 21-year old female patient in whom generalized gingival melanoacanthoma was related to the use of the teeth whitening strips. This irritating factor increased melanocyte activity in the gingival tissues and labial mucosa. Discussion: Oral melanoacanthoma is a rarely encountered pigmented lesion in the oral cavity and is especially uncommon in the gingiva. It is a reactive lesion affecting the mucous membranes with no risk of malignant transformation. This case report shows that teeth whitening strips may trigger oral melanoacanthoma in susceptible individuals. Long-term irritation of the oral tissues may increase the number of dendritic melanocytes throughout the epithelium and accordingly increase the brown pigmentation of the oral cavity.  Eliminating all possible local sources of irritation and ruling out other causative factors are the standard first step in the treatment of oral melanoacanthoma. Conclusions: This case shows the importance of including oral melanoacanthoma in the differential diagnosis of diffuse gingival pigmented lesions.

scholarly journals Pemphigus Vulgaris in Old Patient

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Bassel Tarakji
Keyword(s):  
Differential Diagnosis ◽  
Autoimmune Disease ◽  
Oral Mucosa ◽  
Oral Prednisolone ◽  
Pemphigus Vulgaris ◽  
The Other ◽  
Oral Lesions ◽  
Oral Medicine ◽  
Mucous Membranes ◽  
Blistering Disease

Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The first clinical manifestation is often the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiological factors of this disease still remain unknown, although the presence of autoantibodies is consistent with an autoimmune disease. A 73-year-old man had bullous lesions on gingiva, oral mucosa first, then scalp, trunk, and face. An oral medicine specialist suspects the lesion in differential diagnosis in the first presentation of oral lesions and follows up the patient, and then these bullous lesions presented on the skin. In this article, a patient had received oral prednisolone (80 mg/kg/day) and azathioprine, then tapered oral prednisolone to 40 mg/day, with a reduction of 5 mg/day every three weeks. The patient shows remission of these lesions, and complication of this treatment includes osteoporosis, hyperglycemia, and hypertension.

scholarly journals Epidemiology and Factors Associated with Remission of Pemphigus Vulgaris and Foliaceus in Singapore

2020 ◽  
Vol 49 (6) ◽  
pp. 367-376
Author(s):  
Sophie CS Cai ◽  
Xiahong Zhao ◽  
Mark BY Tang ◽  
Yen Loo Lim
Keyword(s):  
Antibody Titre ◽  
Pemphigus Vulgaris ◽  
National Registry ◽  
Pemphigus Foliaceus ◽  
Prednisolone Dose ◽  
Asian Patients ◽  
Autoimmune Blistering Disease ◽  
Antibody Titres ◽  
Blistering Disease ◽  
Study Methodology

Background: Pemphigus is a chronic, relapsing immunobullous disease. There is limited data on the clinical course and prognostic factors of pemphigus in Asian patients. Methods: We conducted a retrospective cohort study of all newly diagnosed pemphigus vulgaris (PV) and pemphigus foliaceus (PF) patients seen at the National Skin Centre from 1 January 2004 to 31 December 2009. Demographic and clinical data on co-morbidities, treatment and remission were recorded. Mortality information was obtained from the National Registry of Diseases. Prognostic endpoint was overall remission at last visit. Results: Sixty- one patients (36 PV and 25 PF) were recruited. Among PV patients, higher initial prednisolone dose (P = 0.017) and the use of azathioprine (P = 0.028) were significantly associated with overall remission at last visit. However, higher desmoglein 1 antibody titres at diagnosis (P = 0.024) and the use of dapsone (P = 0.008) were negatively associated with overall remission at last visit. Among PF patients, only higher desmoglein 1 antibody titre at diagnosis (P = 0.041) was found to be associated with lower overall remission at last visit. There was no mortality during the 3-year follow-up period in both PV and PF. Conclusions: Higher initial prednisolone dose and the use of azathioprine in PV desmoglein 1 antibody titre at diagnosis in PV and PF might be prognostic markers for achieving remission. Use of dapsone was associated with lower overall remission in PV, but this might be confounded because dapsone was used as an adjuvant therapy in recalcitrant cases. Owing to study methodology and limitations, further evaluation is needed for better prognostication of pemphigus. Ann Acad Med Singapore 2020;49:367–76 Key words: Autoimmune blistering disease, Dermatology, Pemphigus foliaceus, Pemphigus vulgaris, Remission

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