Contact pemphigus: does it exist?

The entity "contact pemphigus" has been recognized for more than 50 years, however existence of the disease, which is opposed and supported by many, is questionable. Contact pemphigus is defined as pemphigus occurring at the site of local skin contact with different chemicals. Many products have been disclosed as aetiological factors such as pesticides, topical drugs (imiquimod, ketoprofen, phenol, bezoin, polymyxin B sulphate, neomycin and bacitracin), cosmetics, garlic and others. This paper summarizes the current knowledge on contact pemphigus and the chemicals responsible for its aetiology, with an emphasis on mechanisms that may elicit the disease.

Association between pemphigus and psoriasis: a systematic review and meta-analysis.

Pemphigus has been associated with other autoimmune and autoinflammatory disorders. Specifically, some case reports in the literature document coexistence of pemphigus with psoriasis, but this association is lacking larger scale investigation. With this in mind, we conducted a systematic review and meta-analysis to evaluate the association between pemphigus and psoriasis. In doing so, we found an association between the two conditions. Pemphigus was more common in patients with psoriasis than in controls (OR 2.64, 95% CI 1.24-5.59, P=0.01), with heterogeneity (I2=94%). We go on to propose pathophysiologic mechanisms and its relevance for diagnostic and management considerations.

Paraneoplastic Dermatoses: A Brief General Review and an Extensive Analysis of Paraneoplastic Pemphigus and Paraneoplastic Dermatomyositis.

Skin manifestations of systemic disease and malignancy are extremely polymorphous. Clinicians should be familiarized with paraneoplastic dermatoses in order to perform an early diagnosis of the underlying neoplasm. Lack of familiarity with cutaneous clues of internal malignancy may delay diagnosis and treatment of cancer. In this review, we described several paraneoplastic dermatoses and discussed extensively two paradigmatic ones, namely paraneoplastic pemphigus and paraneoplastic dermatomyositis.

Factors Associated With Short-term Relapse in Patients With Pemphigus Who Receive Rituximab as First-line Therapy: A Post Hoc Analysis of a Randomized Clinical Trial.

Importance: Rituximab and short-term corticosteroid therapy are the criterion standard treatments for patients with newly diagnosed moderate to severe pemphigus. Objective: To examine factors associated with short-term relapse in patients with pemphigus treated with rituximab. Design, Setting, and Participants: This post hoc analysis of a randomized clinical trial (Comparison Between Rituximab Treatment and Oral Corticosteroid Treatment in Patients With Pemphigus [RITUX 3]) conducted from January 1, 2010, to December 31, 2015, included patients from 20 dermatology departments of tertiary care centers in France from the RITUX 3 trial and 3 newly diagnosed patients treated according to the trial protocol. Data analysis was performed from February 1 to June 30, 2019. Exposure: Patients randomly assigned to the rituximab group in the RITUX 3 trial and the 3 additional patients were treated with 1000 mg of intravenous rituximab on days 0 and 14 and 500 mg at months 12 and 18 combined with a short-term prednisone regimen. Main Outcomes and Measures: Baseline (pretreatment) clinical and biological characteristics (Pemphigus Disease Area Index [PDAI] score, ranging from 0-250 points, with higher values indicating more severe disease) and changes in anti-desmoglein (DSG) 1 and anti-DSG3 values as measured by enzyme-linked immunosorbent assay during the 3 months after rituximab treatment were compared between patients with disease relapse and those who maintained clinical remission during the first 12 months after treatment. The positive and negative predictive values of these factors were calculated. Results: Among 47 patients (mean [SD] age, 54.3 [17.0] years; 17 [36%] male and 30 [64%] female) included in the study, the mean (SD) baseline PDAI score for patients with relapsing disease was higher than that of the patients with nonrelapsing disease (54 [33] vs 28 [24]; P = .03). At month 3, 7 of 11 patients with relapsing disease (64%) vs 7 of 36 patients with nonrelapsing disease (19%) had persistent anti-DSG1 antibody values of 20 IU/mL or higher and/or anti-DSG3 antibody values of 130 IU/mL or higher (P = .01). A PDAI score of 45 or higher defining severe pemphigus and/or persistent anti-DSG1 antibody values of 20 IU/mL or higher and/or anti-DSG3 antibody values of 130 IU/mL or higher at month 3 provided a positive predictive value of 50% (95% CI, 27%-73%) and a negative predictive value of 94% (95% CI, 73%-100%) for the occurrence of relapse after rituximab. Conclusions and Relevance: The findings suggest that initial PDAI score and changes in anti-DSG antibody values after the initial cycle of rituximab might help differentiate a subgroup of patients with high risk of relapse who might benefit from maintenance rituximab infusion at month 6 from a subgroup of patients with low risk of relapse who do not need early maintenance therapy. Trial Registration: NCT00784589.

Epidemiological and clinical characteristics of patients with pemphigus treated in Bialystok in 2001-2018.

BACKGROUND: Pemphigus is a group of potentially fatal dermatological autoimmune disorders. AIM: Analysis of cases of pemphigus vulgaris (PV) and pemphigus foliaceus (PF) newly diagnosed and treated at the Department of Dermatology and Venereology, Medical University of Bialystok, North-east Poland in years 2001-2018. MATERIAL AND METHODS: A retrospective analysis and comparison of sociodemographic, epidemiological and clinical characteristics of PV and PF patients, including: age, gender, residency, initial severity of skin lesions, involvement of mucous membranes, co-morbidities and their treatment, efficacy of therapy. RESULTS: Sixty-two new cases - 41 (66.13%) of PV and 21 (33.87%) of PF were diagnosed. The average age of PV patients was 54.85±12.35 years and those of PF - 63.81±31.52 years, P<0.05. Females constituted 75.61% and 61.90% in PV and PF group, respectively. Majority of patients with PV were residents of urban and these with PF - of rural areas (70.73% and 66.67%, respectively). On admission, 14 patients with PV (34.15%) and 11 with PF (52.38%) had more than 30% of body surface area involved. In 22 (53.66%) PV mucous membrane (oropharyngeal cavity) was involved. Ten (24.39%) patients with PV and 12 (57.14%) - with PF had more than one concomitant disorder (P<0.05). In treatment prednisone in monotherapy or with additional immunosuppressive agent was mainly used. The disease relapsed within three years after achieving clinical and immunological remission in 29.27% of PV and in 38.10% of PF patients. CONCLUSIONS: PF patients are older than PV ones, more frequently live in rural areas, have more comorbidities. Females constitute majority of pemphigus patients. The disease may relapse in about one third of patients. Because of frequent comorbidities, also these related to pemphigus treatment, patients with pemphigus require complex and multispecialistic medical care.

Clinicopathological findings and clinical outcomes in 49 cases of feline pemphigus foliaceus examined in Northern California, USA (1987-2017).

BACKGROUND: Few studies have described the pathophysiology, clinical course, treatment outcomes and quality of life (QoL) of cats with pemphigus foliaceus (PF). OBJECTIVE: Describe clinicopathological features, treatment outcomes and impacts on QoL in feline PF. ANIMALS: Forty-nine client-owned cats with PF that presented to a veterinary teaching hospital between 1987 and 2017. METHODS AND MATERIALS: Medical records and histopathological reports were reviewed to obtain clinicopathological data and treatment outcomes. Owners were contacted and requested to complete a questionnaire to obtain long-term follow-up and evaluate the impacts of PF on QoL of cats and owners. RESULTS: Domestic short/medium/long hair breeds were most commonly affected, with pinnae, head, haired face, nasal planum and ungual folds most frequently involved. Associated pruritus and systemic signs of illness were common. Vasculopathological changes were noted in a small proportion of cats. Corticosteroid monotherapy was sufficient to induce complete remission in the majority of cats. Pemphigus foliaceus and its management had a negative impact on QoL of both cats and owners. Receiving/administering medications, attending veterinary appointments, and financial and time commitments were cited sources of stress for affected cats and/or owners. CONCLUSIONS AND CLINICAL IMPORTANCE: Results illustrate that affected cats generally respond favourably to treatment but do require long-term therapy. The exact aetiology of the vasculopathological changes was unclear; it may reflect the stage or severity of disease or suggest the presence of a cutaneous adverse drug reaction. Clinicians managing cats with PF should be aware of the potential negative impact on QoL of owners and cats and adjust management accordingly.

Pênfigo neonatal em filho de mãe com pênfigo vulgar: relato de caso / Neonatal pemphigus in an infant born to a mother with pemphigus vulgaris: a case report

RESUMO Objetivo: Relatar um caso de pênfigo neonatal em paciente que manifestou lesões cutâneas extensas e críticas ao nascimento. Descrição do caso: Recém-nascido do sexo masculino com lesões vesicobolhosas extensas em região anterior do tórax e abdome, desde o nascimento. Admitido na ala pediátrica de um hospital para diagnóstico etiológico e tratamento. Com base na história materna e na avaliação clínica, concluiu tratar-se de pênfigo vulgar neonatal. O paciente apresentou evolução satisfatória, sem a necessidade de intervenção farmacológica. Comentários: Os casos descritos na literatura e as referências avaliadas revelam o pênfigo neonatal como uma doença de ocorrência rara, porém cujo conhecimento e diagnóstico precoce têm grande relevância clínica, considerando-se que geralmente se manifesta com lesões epidérmicas extensas e de aspecto crítico, embora apresente curso clínico transitório e benigno, sem necessidade de tratamento específico e sem relação com doença futura.

ABSTRACT Objective: To report on the case of a patient with neonatal pemphigus that had extensive and critical skin lesions at birth. Case description: A newborn male with extensive vesico-bullous lesions on the anterior side of his chest and abdomen at birth. He was admitted to the pediatric ward of a hospital for an etiological diagnosis and for treatment. Based on maternal history and a clinical evaluation, the patient was diagnosed with neonatal vulgar pemphigus. His progression was satisfactory and, in the end, he did not need pharmacological interventions. Comments: The cases reported in the literature and the references evaluated reveal that neonatal pemphigus is rare, but that knowledge about the disease allows for an early diagnosis to be made. This has great clinical relevance considering that the disease usually manifests itself in the form of extensive epidermal lesions, even though it is transient and benign, it does not require specific treatment, and it does not have any relation with possible future diseases.

NEONATAL PEMPHIGUS IN AN INFANT BORN TO A MOTHER WITH PEMPHIGUS VULGARIS: A CASE REPORT. / PÊNFIGO NEONATAL EM FILHO DE MÃE COM PÊNFIGO VULGAR: RELATO DE CASO.

OBJECTIVE: To report on the case of a patient with neonatal pemphigus that had extensive and critical skin lesions at birth. CASE DESCRIPTION: A newborn male with extensive vesico-bullous lesions on the anterior side of his chest and abdomen at birth. He was admitted to the pediatric ward of a hospital for an etiological diagnosis and for treatment. Based on maternal history and a clinical evaluation, the patient was diagnosed with neonatal vulgar pemphigus. His progression was satisfactory and, in the end, he did not need pharmacological interventions. COMMENTS: The cases reported in the literature and the references evaluated reveal that neonatal pemphigus is rare, but that knowledge about the disease allows for an early diagnosis to be made. This has great clinical relevance considering that the disease usually manifests itself in the form of extensive epidermal lesions, even though it is transient and benign, it does not require specific treatment, and it does not have any relation with possible future diseases.

OBJETIVO: Relatar um caso de pênfigo neonatal em paciente que manifestou lesões cutâneas extensas e críticas ao nascimento. DESCRIÇÃO DO CASO: Recém-nascido do sexo masculino com lesões vesicobolhosas extensas em região anterior do tórax e abdome, desde o nascimento. Admitido na ala pediátrica de um hospital para diagnóstico etiológico e tratamento. Com base na história materna e na avaliação clínica, concluiu tratar-se de pênfigo vulgar neonatal. O paciente apresentou evolução satisfatória, sem a necessidade de intervenção farmacológica. COMENTÁRIOS: Os casos descritos na literatura e as referências avaliadas revelam o pênfigo neonatal como uma doença de ocorrência rara, porém cujo conhecimento e diagnóstico precoce têm grande relevância clínica, considerando-se que geralmente se manifesta com lesões epidérmicas extensas e de aspecto crítico, embora apresente curso clínico transitório e benigno, sem necessidade de tratamento específico e sem relação com doença futura.

Diagnosing Pemphigus Foliaceus: A Rare Blistering Disease Masquerading as a Common Dermatologic Disorder.

Pemphigus foliaceus (PF) is an autoimmune dermatologic disease that typically presents with painful, superficial blisters that evolve into scaling erosions in a seborrheic distribution. This case study intends to demonstrate that due to the relative scarcity of the disease and its distribution on the body, PF can easily be misdiagnosed. We present a 43-year-old African American male that presented to the dermatology clinic with an 18-month history of non-pruritic, violaceous, scaling patches and plaques most prominent on the malar cheeks, upper chest and upper back. He had been evaluated at an outside hospital with a high suspicion for cutaneous lupus erythematosus (CLE) and seborrheic dermatitis. However, repeated biopsies revealed non-specific spongiotic dermatitis, not consistent with CLE or seborrheic dermatitis. Over the subsequent months, he received treatment for both conditions without improvement in his symptoms. When he was referred to our dermatology clinic, repeat biopsies were obtained which demonstrated acantholysis and dyskeratosis in the granular layer, consistent with PF. Direct immunofluorescence revealed intercellular IgG staining most prominent in the epidermis, also consistent with PF. Finally, enzyme-linked immunosorbent assay for anti-desmoglein 1 returned positive, confirming the diagnosis. Upon review of the previous biopsies, focal areas of acantholysis and dyskeratosis were noted in the granular layer, which would have pointed away from a diagnosis of CLE or seborrheic dermatitis if PF was included in the clinical differential diagnosis. This case serves as a reminder that when there is a discrepancy in clinical-pathologic correlation, it is important to revisit the case and consider other pathologies.

Pemphigus: cartography on the coordination of health policies. / Pênfigo: uma cartografia sobre as articulações das políticas em saúde.

Pemphigus is a group of autoimmune blistering diseases that affect the skin and mucous membranes, the global incidence of which is 0.75 to 5 / 1.000.000 cases a year. The approach taken derives from the relations of power/truth and subjectivity that produce modes of experience from the perspective of hospitals and public policies. It involves cartographic qualitative research, supported from a Deleuzian standpoint. The objective was to discuss pemphigus as a chronic disease and discuss health policies and their impact on care practices. This cartography was conducted in a philanthropical hospital, which is a national reference for the treatment of pemphigus. The research was conducted from September 2012 to February 2013. The analysis of materials included: medical records, field diary reports of all healthcare activities, such as medical consultations, technical procedures performed by nursing staff and medical staff and reports of patients, families, physicians, and nursing staff. The analyses were created from a Foucauldian historical and genealogical perspective. The conclusion reached is that health policies for chronic patients with pemphigus are not presented as a condition to be avoided / prevented, but as abnormal groups and bio identities that have no effect on the whole population.

Pênfigos são um grupo de doenças bolhosas autoimunes que acometem a pele e mucosas, cuja incidência mundial é de 0,75-5/1.000.000 casos ao ano. Nossa problematização parte das relações de poder/verdade e subjetivação que produzem modalidades de experiência sob a perspectiva hospitalar e das políticas públicas. Pesquisa qualitativa cartográfica, sustentada sob uma perspectiva Deleuziana. Nossos objetivos foram problematizar o pênfigo como doença-crônica e discutir as políticas de saúde e sua repercussão nas práticas de cuidado. Percorremos esta cartografia em um hospital filantrópico de referência nacional para tratamento de pênfigo. Esta pesquisa foi construída de setembro de 2012 a fevereiro de 2013. Os materiais de análise foram: prontuários, relatos em diário de campo de todas as atividades assistenciais realizadas, tais como: consultas médicas, procedimentos técnicos realizados pela equipe de enfermagem e equipe médica e relatos de pacientes, familiares, médicos, e equipe de enfermagem. Nossas análises foram construídas sob uma perspectiva histórico-genealógica Foucaultiana. Consideramos que as políticas de saúde a doentes crônicos com pênfigo não se apresentam como uma condição a ser evitada/prevenida, mas como uma anormalidade de grupos, bioidentidades que não têm efeito no conjunto da população.

Pênfigo: uma cartografia sobre as articulações das políticas em saúde / Pemphigus: cartography on the coordination of health policies

Resumo Pênfigos são um grupo de doenças bolhosas autoimunes que acometem a pele e mucosas, cuja incidência mundial é de 0,75-5/1.000.000 casos ao ano. Nossa problematização parte das relações de poder/verdade e subjetivação que produzem modalidades de experiência sob a perspectiva hospitalar e das políticas públicas. Pesquisa qualitativa cartográfica, sustentada sob uma perspectiva Deleuziana. Nossos objetivos foram problematizar o pênfigo como doença-crônica e discutir as políticas de saúde e sua repercussão nas práticas de cuidado. Percorremos esta cartografia em um hospital filantrópico de referência nacional para tratamento de pênfigo. Esta pesquisa foi construída de setembro de 2012 a fevereiro de 2013. Os materiais de análise foram: prontuários, relatos em diário de campo de todas as atividades assistenciais realizadas, tais como: consultas médicas, procedimentos técnicos realizados pela equipe de enfermagem e equipe médica e relatos de pacientes, familiares, médicos, e equipe de enfermagem. Nossas análises foram construídas sob uma perspectiva histórico-genealógica Foucaultiana. Consideramos que as políticas de saúde a doentes crônicos com pênfigo não se apresentam como uma condição a ser evitada/prevenida, mas como uma anormalidade de grupos, bioidentidades que não têm efeito no conjunto da população.

Abstract Pemphigus is a group of autoimmune blistering diseases that affect the skin and mucous membranes, the global incidence of which is 0.75 to 5 / 1.000.000 cases a year. The approach taken derives from the relations of power/truth and subjectivity that produce modes of experience from the perspective of hospitals and public policies. It involves cartographic qualitative research, supported from a Deleuzian standpoint. The objective was to discuss pemphigus as a chronic disease and discuss health policies and their impact on care practices. This cartography was conducted in a philanthropical hospital, which is a national reference for the treatment of pemphigus. The research was conducted from September 2012 to February 2013. The analysis of materials included: medical records, field diary reports of all healthcare activities, such as medical consultations, technical procedures performed by nursing staff and medical staff and reports of patients, families, physicians, and nursing staff. The analyses were created from a Foucauldian historical and genealogical perspective. The conclusion reached is that health policies for chronic patients with pemphigus are not presented as a condition to be avoided / prevented, but as abnormal groups and bio identities that have no effect on the whole population.

Italian Guidelines in Pemphigus - adapted from the European Dermatology Forum (EDF) and European Academy of Dermatology and Venerology (EADV).

Pemphigus is a life threatening autoimmune epidermal blistering disease involving skin and mucous membranes. Pemphigus usually affects middle age men and women involving oral mucosa first and then spreading on the skin. It is caused by the presence of autoantibodies (IgG and less frequently by IgA) directed against desmogleins, and/or other glycoproteins that plays a critical role in cell-cell attachment. Upon a predisposing genetic background, different agents have been shown to act as triggers for the pathogenesis of pemphigus. This guideline for the diagnosis and treatment of pemphigus has been developed by an Italian group of experts taking in account the Italian legislation and local pharmacological governance. Guidelines are adapted from the original article under the guidance of the European Dermatology Forum in collaboration with the European Academy of Dermatology and Venereology. It summarizes evidence-based and expert-based recommendations (S2 level).

Spectrum of orocutaneous disease associations: Immune-mediated conditions.

There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies.

Different signaling patterns contribute to loss of keratinocyte cohesion dependent on autoantibody profile in pemphigus.

Pemphigus is an autoimmune blistering skin disease caused primarily by autoantibodies against desmoglein (Dsg)1 and 3. Here, we characterized the mechanisms engaged by pemphigus IgG from patients with different clinical phenotypes and autoantibody profiles. All pemphigus vulgaris (PV) and pemphigus foliaceus (PF) IgG and AK23, a monoclonal mouse antibody against Dsg3, caused loss of cell cohesion, cytokeratin retraction and p38MAPK activation. Strong alterations in Dsg3 distribution were caused by mucosal (aDsg3 antibodies), mucocutaneous (aDsg1 + aDsg3) as well as atypical (aDsg3) PV-IgG. All PV-IgG fractions and AK23 compromised Dsg3 but not Dsg1 binding and enhanced Src activity. In contrast, rapid Ca2+ influx and Erk activation were induced by mucocutaneous PV-IgG and pemphigus foliaceus (PF) IgG (aDsg1) whereas cAMP was increased by mucosal and mucocutaneous PV-IgG only. Selective inhibition of p38MAPK, Src or PKC blocked loss of keratinocyte cohesion in response to all autoantibody fractions whereas Erk inhibition was protective against mucocutaneous PV-IgG and PF-IgG only. These results demonstrate that signaling patterns parallel the clinical phenotype as some mechanisms involved in loss of cell cohesion are caused by antibodies targeting Dsg3 whereas others correlate with autoantibodies against Dsg1. The concept of key desmosome regulators may explain observations from several experimental models of pemphigus.

Evaluating the Efficacy of Double-Filtration Plasmapheresis in Treating Five Patients With Drug-Resistant Pemphigus.

Double-filtration plasmapheresis is an effective and safe treatment for pemphigus. We retrospectively evaluated the decrease in autoantibody titer and pemphigus disease area index following double-filtration plasmapheresis in five patients with moderate to severe pemphigus, who were physically and/or serologically unresponsive to 1.0 mg/kg per day of prednisolone and other supportive drugs and ointments. The percentage reduction in autoantibodies 85.6 ± 14.4% (P = 0.00014), and that in pemphigus disease area index was 75.4 ± 24.3% (P = 0.0023). No side-effects were observed. All patients exhibited clinical improvement after undergoing double-filtration plasmapheresis, and the prednisolone dose was reduced by 41 ± 8.9 mg (P = 0.0005) approximately 3 months after double-filtration plasmapheresis. To our knowledge, this is the first report evaluating the efficacy of double-filtration plasmapheresis with pemphigus disease area index, and it demonstrated that double-filtration plasmapheresis is a safe "subtracting" treatment for patients with drug-resistant pemphigus.

Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016.

Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases. Considerable advances have been made in describing incidences and prevalences of these diseases and linking comorbidities with autoantibody reactivities and clinical variants, for example, dipeptidyl peptidase-IV inhibitor-associated noninflammatory bullous pemphigoid. Although new entities are still being described, diagnosis of most autoimmune blistering diseases can now be achieved using standardized and widely available serological test systems. Various experimental mouse models of pemphigus and pemphigoid disease are increasingly being used to understand mechanisms of central and peripheral tolerance and to evaluate more specific treatment approaches for these disorders, such as molecules that target autoreactive T and B cells and anti-inflammatory mediators, that is, dimethyl fumarate, phosphodiesterase 4, and leukotriene B4 inhibitors in pemphigoid disorders, and chimeric antigen receptor T cells in pemphigus. Very recent experimental data about the immunopathology and the determinants of autoantibody formation and keratinocyte susceptibility in pemphigus were discussed. With regard to cellular mechanisms leading to the loss of cell-cell adhesion, new ideas were shared in the field of signal transduction. Major steps were taken to put the various partly contradictory and controversial findings about the effects of pemphigus autoantibodies and other inflammatory mediators into perspective and broaden our view of the complex pathophysiology of this disease. Finally, two investigator-initiated multicenter trials highlighted doxycycline and dapsone as valuable medications in the treatment of bullous pemphigoid.

Pemphigus: Pathogenesis to Treatment.

Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. These lesions correlate histologically with splits forming in the epidermis, leaving a blister roof composed of a few cell layers. Standard treatment of pemphigus involves oral corticosteroids, often with the addition of adjuvant therapies, to improve disease control, minimize corticosteroids side-effects, and increase the odds of remission. [Full article available at http://rimed.org/rimedicaljournal-2016-12.asp].