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pemphigus vulgaris

Kamran Balighi, Maryam Daneshpazhooh, Nessa Aghazadeh, Vahide Saeidi, Farzam Shahpouri, Pardis Hejazi, Cheyda Chams-Davatchi
HINTERGRUND UND ZIELE: Pemphigus vulgaris (PV) wird in der Regel mit systemischen Corticosteroiden und Immunsuppressiva behandelt. Avaskuläre Nekrose (AVN) des Hüftkopfes ist eine gut bekannte schwerere Komplikation einer Corticosteroid-Therapie. Die Charakteristika dieser schweren Komplikation bei PV sind nach wie vor unbekannt. PATIENTEN UND METHODEN: Nicht kontrollierte, retrospektive Untersuchung aller PV-bedingten AVN-Fälle, die in einer iranischen Klinik für bullöse Autoimmunerkrankungen zwischen 1985 und 2013 diagnostiziert wurden...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Kamran Balighi, Maryam Daneshpazhooh, Nessa Aghazadeh, Vahide Saeidi, Farzam Shahpouri, Pardis Hejazi, Cheyda Chams-Davatchi
BACKGROUND AND OBJECTIVES: Pemphigus vulgaris (PV) is typically treated with systemic corticosteroids and immunosuppressive agents. Avascular necrosis (AVN) of the femoral head is a well-recognized major complication of corticosteroid therapy. The characteristics of this serious complication in PV remain unknown. PATIENTS AND METHODS: Uncontrolled, retrospective study of all PV-related AVN cases diagnosed at an Iranian autoimmune bullous disease clinic between 1985 and 2013...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Aneliza de Fatima Moraes da Silva, Daniel Galera Bernabé, Glauco Issamu Miyahara, Eder Ricardo Biasoli, Renata Callestini, Kellen Cristine Tjioe
Oral biopsy of vesiculobullous diseases such as pemphigus vulgaris often raises questions due to some particularities involving this procedure. The adequate selection of the area to be biopsied defines if the final diagnosis will be reached, being the Achiles heel of the proper management of the patient. Here, the authors report a case of a woman who sought for treatment of generalized oral blisters and ulcers that caused severe pain. She had undergone a previous biopsy by other professionals that was inconclusive...
October 14, 2016: Journal of Craniofacial Surgery
Franziska Vielmuth, Vera Rötzer, Eva Hartlieb, Christoph Hirneiß, Jens Waschke, Volker Spindler
Purpose: The autoimmune blistering skin disease pemphigus vulgaris (PV) is caused by autoantibodies against desmosomal adhesion molecules. Patients may suffer conjunctival involvement, yet the underlying mechanisms are largely unclear. We characterized human and murine conjunctiva with respect to the PV autoantigens, and evaluated the effects and mechanisms of PV autoantibodies applied to human conjunctiva ex vivo. Methods: We obtained human conjunctiva specimens from surgical explants and established a short-term culture model to study the alterations induced by antibody fractions of PV patients (PV-IgG)...
August 1, 2016: Investigative Ophthalmology & Visual Science
Sangeetha Jeevan Kumar, S P Nehru Anand, Nandhini Gunasekaran, Rajkumar Krishnan
Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The initial clinical manifestation is frequently the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiology of this disease still remains obscure although the presence of autoantibodies is consistent with an autoimmune disease. These antibodies are targeted against the adhesion proteins of keratinocytes, leading to acantholysis (disruption of spinous layer, leading to intraepidermal clefting) and blister formation...
September 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
Sharon Baum, Assaf Debby, Sarit Gilboa, Henri Trau, Aviv Barzilai
BACKGROUND: Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease. Most patients require long-term therapy with systemic steroids, and a steroid-sparing agent is usually also utilized. Dapsone is a chemotherapeutic agent with anti-inflammatory properties that is used as a steroid-sparing agent in PV. OBJECTIVE: The aim of the present study was to evaluate the efficacy of dapsone as an adjuvant therapy in patients with PV. METHODS: A retrospective analysis of patients' files was performed...
October 4, 2016: Dermatology: International Journal for Clinical and Investigative Dermatology
Chander Grover, Bineeta Kashyap, Deepashree Daulatabad, Amit Dhawan, Iqbal R Kaur
BACKGROUND: Anti-cyclic citrullinated peptides (CCPs) are autoantibodies directed against citrullinated peptides. Rheumatoid factor (RF), an antibody against the Fc portion of IgG, is known to form immune complexes and contribute to the etiopathogenesis of various skin disorders. C-reactive protein (CRP), an acute-phase protein, increases following secretion of interleukin-6 from macrophages and T cells. Anti-CCP, RF, and CRP are well-established immune-markers, their diagnostic potential in immune-mediated skin disorders remains less widely studied...
September 2016: Indian Journal of Dermatology
C Günther
Autoimmune bullous diseases are characterized by intraepidermal or subepidermal autoantibody deposition that leads to blisters and secondary erosion. Mucous membranes are frequently affected in pemphigus vulgaris and always involved in cicatricial and mucosal pemphigoid. Mucosal lesions are detected less frequently in patients with bullous pemphigoid or epidermolysis bullosa acquisita. The diagnosis of autoimmune bullous disorders is based on determination of the subtype of autoantibodies bound in the skin and the clinical picture...
October 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
K Gleghorn, J Wilson, M Wilkerson
Rituximab is an anti-CD20 monoclonal antibody with considerable potential in dermatology due to an increase in off-label indications. Chronic graft-versus-host disease and pemphigus vulgaris are two of the most promising indications for off-label use of rituximab. It is a generally safe alternative that should be considered when traditional therapy with corticosteroids or immunosuppressants has failed or caused significant intolerance. Currently, rituximab is only FDA-approved for treatment of follicular and diffuse large B-cell non-Hodgkin's lymphoma, rheumatoid arthritis, chronic lymphocytic leukemia, granulomatosis with polyangiitis (formerly Wegener's granulomatosis) and microscopic polyangiitis...
September 2016: Skin Therapy Letter
A von Köckritz, S Ständer, C Zeidler, D Metze, T Luger, G Bonsmann
BACKGROUND: Pemphigus vegetans is a rare variant of pemphigus vulgaris, accounting for 1-2% of all pemphigus diseases. Systemic corticosteroids are the therapy of first choice in combination with immunosuppressants as steroid-sparing agents. OBJECTIVE: To highlight the exceptional but successful use of minocycline/nicotinamide monotherapy in pemphigus vegetans. METHODS: A review of the literature to date about pemphigus vegetans with special emphasis on therapy was performed...
September 7, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Julia Yu-Fong Chang, Chun-Pin Chiang, Yi-Ping Wang, Yang-Che Wu, Hsin-Ming Chen, Andy Sun
BACKGROUND: Desquamative gingivitis (DG) is principally associated with erosive oral lichen planus (EOLP), mucous membrane pemphigoid (MMP), and pemphigus vulgaris (PV). METHODS: Serum autoantibodies including antigastric parietal cell antibody (GPCA), antithyroglobulin antibody (TGA), and antithyroid microsomal antibody (TMA) were measured in 500 patients with DG, 287 EOLP without DG (EOLP/DG(-) ) patients, and 100 healthy control subjects. RESULTS: The 500 patients with DG were diagnosed as having EOLP in 455 (91%), PV in 40 (8%), and MMP in five (1%) patients...
September 7, 2016: Journal of Oral Pathology & Medicine
Rajsmita Bhattacharjee, Dipankar De, Sanjeev Handa, Ranjana W Minz, Biman Saikia, Neha Joshi
BACKGROUND: Robust evidence for the efficacy of rituximab monotherapy in pemphigus is lacking. The effects of rituximab on T-regulatory cells (Tregs) in pemphigus have not been studied. OBJECTIVE: The primary objective was to assess the efficacy of rituximab monotherapy in severe pemphigus vulgaris. The secondary objectives were to assess whether counts of different subsets of Tregs in the peripheral blood correlate with baseline clinical severity and whether clinical response in severe pemphigus is associated with an alteration in the Treg count...
September 1, 2016: Dermatology: International Journal for Clinical and Investigative Dermatology
Shagufta Rather, Atiya Yaseen, Iffat Hassan
No abstract text is available yet for this article.
July 2016: Indian Dermatology Online Journal
Euzeli da Silva Brandão, Iraci Dos Santos, Regina Serrão Lanzillotti, Adriano Menis Ferreira, Mônica Antar Gamba, Luna Azulay-Abulafia
OBJECTIVE: identify nursing diagnoses in patients with immune-bullous dermatosis. METHOD: a quantitative and descriptive research, carried out in three institutions located in Rio de Janeiro and Mato Grosso do Sul, Brazil, using the Client Assessment Protocol in Dermatology during a nursing consultation. Simple descriptive statistics was used for data analysis. RESULTS: 14 subjects participated in the study, nine with a diagnosis of pemphigus vulgaris, pemphigus two and three of bullous pemphigoid...
2016: Revista Latino-americana de Enfermagem
Silvia Méndez-Flores, Esperanza Avalos-Diaz, Judith Dominguez-Cherit, Marcela Saeb-Lima, Lilly Esquivel-Pedraza
Pemphigus is a group of autoimmune intraepidermal bullous diseases; being pemphigus foliaceus (PF) and pemphigus vulgaris (PV) the most common subtypes. Pustular variants are scarcely reported for both PV and PF. The purpose of this manuscript was to describe the clinical, microscopic and immunologic findings of an atypical case of PF presenting with pustules, including a review of the literature. PF is described as blisters and because this entity is rare, it is not known for the general medical community that they are other clinical features that can be seen as this one we present here with pustules...
August 12, 2016: Journal of Cutaneous Pathology
Asuka Yoshifuku, Kazuyasu Fujii, Hisao Kawahira, Hiromi Katsue, Atsunori Baba, Yuko Higashi, Yumi Aoyama, Takuro Kanekura
Pemphigus vulgaris (PV) is an autoimmune blistering disease elicited by anti-desmoglein (DsG) 3 antibody. Although skin lesions tend to be distributed over the entire body, in some patients, they are confined to a restricted area. We report two patients who presented with long-lasting localized PV without detectable anti-DsG antibodies after suffering antibody-positive systemic PV. Initial treatment with prednisolone (PSL) was successful in both patients, but a local relapse occurred on the cheek or lower lip after a reduction in the PSL dose...
July 2016: Indian Journal of Dermatology
Kabir Sardana, Pooja Agarwal, Shivani Bansal, Beena Uppal, Vijay K Garg
CONTEXT: A prospective study was carried out to examine the efficacy of cyclophosphamide and azathioprine in pemphigus vulgaris. AIMS: To compare the clinical and serological effect of azathioprine and cyclophosphamide in pemphigus patients. MATERIALS AND METHODS: Prospective, institutional based study was conducted twenty-one patients of pemphigus vulgaris were initiated on either azathioprine (n = 9) or cyclophosphamide (n = 7) in addition to prednisolone and were evaluated clinically (mucosal and cutaneous severity) and serologically enzyme-linked immunosorbent assay (ELISA) at 0, 3 and 6 months...
July 2016: Indian Journal of Dermatology
D Miyamoto, M N Sotto, C S V Otani, L M I Fukumori, N V Pereira, C G Santi, C W Maruta, M N N Burnier, M M Rebeis, V Aoki
BACKGROUND: Erythroderma is a clinical skin syndrome shared by patients with cutaneous disorders of distinct etiologies as a result of the combined actions of chemokines, adhesion molecules, and cytokines, such as vascular endothelial growth factor (VEGF). OBJECTIVE: To evaluate the profile of serum levels of VEGF and soluble vascular endothelial growth factor receptor 1 (sVEGFR-1) in pemphigus foliaceus (PF) patients with erythroderma. METHODS: We conducted a retrospective study, which included 1) a chart review of all PF patients from the Autoimmune Blistering Clinic, University of Sao Paulo, Brazil, from January 1991 to December 2014, together with an evaluation of demographic variables, hospitalization duration and complications and 2) analysis of the circulating VEGF and sVEGFR-1 levels in PF patients with erythroderma by ELISA...
August 11, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Parichehr Ghalayani, Fateme Rashidi, Zahra Saberi
OBJECTIVES: Regarding the implication of viruses particularly herpes in pemphigus vulgaris, we sought to assess and compare the level of immunoglobulin G (IgG) antibodies against herpes simplex virus types 1 and 2 (HSV1 and HSV2), cytomegalovirus (CMV) and Epstein-Barr virus (EBV) in patients with pemphigus vulgaris and healthy people. MATERIALS AND METHODS: In this cross-sectional study, 25 patients with pemphigus vulgaris and 27 healthy individuals comprised the experimental and control groups, respectively...
November 2015: Journal of Dentistry of Tehran University of Medical Sciences
Sushil S Savant, Anupam Das, Piyush Kumar
No abstract text is available yet for this article.
August 4, 2016: Indian Journal of Dermatology, Venereology and Leprology
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