Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis

Montagnon, Carmen M.; Tolkachjov, Stanislav N.; Murrell, Dédée F.; Camilleri, Michael; Lehman, Julia S.

doi:10.1016/j.jaad.2020.11.075

Cited by 26 publications

(26 citation statements)

References 89 publications

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“…PNP is a severe and progressive blistering dermatosis, thought to result from neoplasia-triggered autoimmunity against multiple self-antigens, such as Dsg3, Dsg1, desmoplakins, and bullous pemphigoid antigen-1. 1 In contrast, PF is considered a relatively benign and often idiopathic condition caused by autoantibodies against Dsg1 expressed in cutaneous epithelium. Unlike PNP, which has early and striking mucosal involvement, PF spares mucosal surfaces due to the absence of autoantibodies against Dsg3, which is primarily expressed in mucosal epithelium.…”

Section: Discussionmentioning

confidence: 99%

“…PF is further distinguished from PNP by negative indirect immunofluorescence labeling of rat bladder epithelium. 1 The association of PF with underlying malignancy has not been well established. 1 Recent studies documented significantly higher incidences of lymphoproliferative malignancies and solid cancers (esophageal and laryngeal cancers, lung cancers, and nonmelanoma skin cancers) in patients with PV and PF, suggesting a paraneoplastic association in pemphigus disorders beyond PNP.…”

Section: Discussionmentioning

confidence: 99%

“…1 The association of PF with underlying malignancy has not been well established. 1 Recent studies documented significantly higher incidences of lymphoproliferative malignancies and solid cancers (esophageal and laryngeal cancers, lung cancers, and nonmelanoma skin cancers) in patients with PV and PF, suggesting a paraneoplastic association in pemphigus disorders beyond PNP. [2][3][4][5] In light of these emerging findings, we recommended and coordinated age-appropriate cancer screenings for our patient.…”

Section: Discussionmentioning

confidence: 99%

“…An association between pemphigus disorders and malignancy has primarily been established in the paraneoplastic pemphigus (PNP) subtype. 1 Emerging evidence suggests that pemphigus vulgaris (PV) and pemphigus foliaceus (PF), the 2 most common types of pemphigus, may also be associated with an increased incidence of certain malignancies. [2][3][4][5] Here, we report a patient with no known medical history who presented with new-onset PF and was subsequently diagnosed with an invasive mammary carcinoma from investigation of a possible paraneoplastic etiology.…”

Section: Introductionmentioning

confidence: 99%

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Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

et al. 2021

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

et al. 2021

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“…Pemphigus is a AIBD skin disease caused by the production of autoantibodies that target desmosomal adhesion molecules including desmoglein (Dsg) and desmocollin (Dsc), among others (1). Pemphigus diagnosis is based on clinical, histological, direct immunofluorescence (DIF) and serological findings (2). Dsg3 and Dsg1 are the major target antigens in pemphigus, but in the last 20 years, new relevant findings have demonstrated the role of non-Dsg autoantibodies in pemphigus cell-cell detachment (3,4) even acting synergistically when Dsg autoantibodies are absent (5).…”

Section: Introductionmentioning

confidence: 99%

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

et al. 2021

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The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.

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Cost-Utility Analysis of Rituximab vs Mycophenolate Mofetil for the Treatment of Pemphigus Vulgaris

et al. 2022

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ImportanceThere is an increasing body of literature that supports the use of rituximab as a first-line steroid-sparing agent in pemphigus vulgaris. However, the cost of rituximab is substantial compared with conventional agents, and there are limited health economic data to justify its use.ObjectiveTo evaluate the cost-effectiveness of rituximab biosimilars relative to mycophenolate mofetil as a first-line steroid-sparing agent for moderate to severe pemphigus vulgaris.Design, Setting, and ParticipantsA cost-utility analysis over a 24-month time horizon was conducted from the perspective of the Australian health care sector using a modeled cohort of treatment-naive adult patients with moderate to severe pemphigus vulgaris. A Markov cohort model was constructed to simulate disease progression following first-line treatment with rituximab biosimilars or mycophenolate mofetil. The simulated cohort transitioned between controlled disease, uncontrolled disease, and death. Efficacy and utility data were obtained from available published literature. Cost data were primarily obtained from published government data. One-way and probabilistic sensitivity analyses were performed to assess uncertainty. Primary outcomes were the changes in cost and quality-adjusted life-years (QALYs), and incremental cost-effectiveness ratio (ICER) over the 24 months.InterventionsRituximab biosimilars and mycophenolate mofetil.ResultsThe simulated cohort of treatment-naive patients had a mean age of 50.8 years, a female-to-male ratio of 1.24, and moderate to severe disease as classified by the Harman criteria. First-line rituximab biosimilars were associated with a cost reduction of AU$639 and an improvement of 0.07 QALYs compared with mycophenolate mofetil, resulting in an ICER of −AU$8818/QALY. Rituximab biosimilars were therefore more effective and less costly compared with mycophenolate mofetil. Sensitivity analyses demonstrated that rituximab biosimilars remained cost-effective across a range of values for cost, utility, and transition probability input parameters and willingness-to-pay thresholds.Conclusions and RelevanceIn this cost-utility analysis, rituximab biosimilars were cost-effective compared with mycophenolate mofetil for moderate to severe pemphigus vulgaris. Further investigation into its cost-effectiveness over a longer time horizon is necessary, but the favorable results of this study suggest that the high acquisition costs of rituximab biosimilars may be offset by its effectiveness and provide economic evidence in support of its listing on the Pharmaceutical Benefits Scheme for pemphigus vulgaris.

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Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis

Cited by 26 publications

References 89 publications

Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

Cost-Utility Analysis of Rituximab vs Mycophenolate Mofetil for the Treatment of Pemphigus Vulgaris

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