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Khalaf Kridin Department of Dermatology, Rambam Health Care Campus, Haifa, Israel Abstract: Pemphigus vulgaris (PV) is a life-threatening disease belonging to the pemphigus group of autoimmune intra-epidermal bullous diseases of the skin and mucosae. The therapeutic management of PV remains challenging and, in some cases, conventional therapy is not adequate to induce clinical remission. The cornerstone of PV treatment remains systemic corticosteroids. Although very effective, long-term corticosteroid administration is characterized by substantial adverse effects. Corticosteroid-sparing adjuvant therapies have been employed in the treatment of PV, aiming to reduce the necessary cumulative dose of corticosteroids. Specifically, immunosuppressive agents such as azathioprine and mycophenolate mofetil are widely used in PV. More recently, high-dose intravenous immunoglobulins, immunoadsorption, and rituximab have been established as additional successful therapeutic options. This review covers both conventional and emerging therapies in PV. In addition, it sheds light on potential future treatment strategies for this disease. Keywords: azathioprine, meycophenolate mofetil, rituximab, intravenous immunoglobulins, immunoadsporption, emerging

OBJECTIVE: COVID-19 toes represent the main dermatological COVID-19 cutaneous manifestation in pediatric patients. Its diagnosis exposes the whole family to social stigma and this aspect was not previously evaluated. PATIENTS AND METHODS: This was a multicenter, case-control, observational study that compared the family impact of COVID-19 toes vs. psoriasis (PsO). We enrolled 46 pediatric patients (23 with psoriasis and 23 with COVID-19 toes, age and gender matched) and their parents/caregivers that had to fill the Dermatitis Family Impact (DFI) questionnaire. RESULTS: DFI index did not differ significantly between both subgroups (p=0.48), and in psoriatic patients did not correlate with both Psoriasis Area Severity Index (PASI) (p=0.59) and itchVAS (p=0.16). CONCLUSIONS: COVID-19 toes, a transitory dermatosis, exerted a similar impact/perturbation on family dynamics than PsO, a well-known stigmatizing, chronic inflammatory dermatosis. [ABSTRACT FROM AUTHOR]

Background: Although the association of bullous pemphigoid (BP) and psoriasis is well‐established, the clinical and immunological features of patients with coexisting BP and psoriasis are yet to be investigated. Objective: We aimed to estimate the prevalence of psoriasis amongst patients with BP and to elucidate the clinical and immunological characteristics of BP patients with comorbid psoriasis. Methods: A retrospective cohort study including all consecutive patients diagnosed with BP throughout the years 2009–2019 in a tertiary referral centre. Results: The study encompassed 273 patients with BP, of whom 11 (4.0%; 95% CI, 2.3–7.1%) had comorbid psoriasis. The onset of psoriasis preceded that of BP in 81.8% of patients by a median (range) latency of 26.5 (5.0–34.0) years. Compared to BP patients without psoriasis, those with BP and comorbid psoriasis were significantly younger at the onset of BP [71.8 (9.3) vs. 79.4 (9.8) years; P = 0.023], had a milder erosive phenotype [erosion/blister BPDAI mean (SD)score; 5 (4.1) vs. 22.3 (15.2); P = 0.025], lower levels of anti‐BP180 NC16A serum autoantibodies [236.6 (266.3) vs. 556.2 (1323.6) U/mL; P = 0.008] and a higher prevalence of isolated linear C3 deposits (36.4% vs. 14.1%; P = 0.043) and a lower prevalence of linear immunoglobulin G deposits (36.4% vs. 68.7%; P = 0.025) along the dermal–epidermal junction by direct immunofluorescence microscopy. Conclusions: Patients with BP and comorbid psoriasis present at a younger age with milder erosive phenotype and lower levels of pathogenic autoantibodies. [ABSTRACT FROM AUTHOR]

Autoimmune blistering diseases (AIBD) are associated with significant impact on quality of life with numerous associated psychiatric comorbidities.1-3 We sought to investigate disease-related risk factors for the development of depression, anxiety and post-traumatic stress disorder (PTSD) in AIBD patients. In patients with active disease, Hospital Anxiety and Depression Scale (HADS) anxiety and depression surveys were utilized. Assessing the risk factors for anxiety, depression and post-traumatic stress disorder through the International Pemphigus and Pemphigoid Foundation. [Extracted from the article]

Background: Current treatment paradigms in anti‐p200 pemphigoid rely on low levels of evidence, primarily originating from case reports and case series. Objective: To systematically review the utilized treatment modalities for anti‐p200 pemphigoid and to synthesize the available clinical outcomes of treated patients. Methods: We conducted a systematic review of the literature using Ovid‐Medline (1946–2018), Embase (1947–2018) and Web of Science (1900–2018) databases with a broad and inclusive search strategy along with a subsequent search of retrieved articles. All case reports and case series of patients with anti‐p200 pemphigoid were included. Results: Sixty‐eight eligible studies comprising 113 anti‐p200 pemphigoid patients with a mean age of 65.5 years were included in the qualitative synthesis. The clinical outcome of patients following treatment was reported for 91 (80.5%) patients, of whom 83 (91.2%) had achieved complete remission at least once. Complete remission on‐therapy was observed in 51 (56.0%) and complete remission off‐therapy in 12 (13.2%) patients. Thirty‐six (39.6%) patients had experienced at least one flare during the duration of follow‐up. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the leading therapeutic approach (63.0%) required for disease control. Systemic and topical corticosteroids as monotherapy were sufficient to control the disease in 19.6% and 13.0% of cases, respectively. Dapsone was the most commonly used (41.3%) adjuvant agent. The highest rates of complete remission were achieved in patients managed by systemic corticosteroids as monotherapy (100%) and in those managed by systemic corticosteroids with adjuvant agents (90.7%). Conversely, 45.5% of patients treated only by topical corticosteroids experienced at least one relapse during follow‐up. Conclusion: The vast majority of patients had reached a complete remission during the course of the disease, whereas a considerable proportion of patients experienced at least one relapse. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the most frequently utilized therapeutic approach. [ABSTRACT FROM AUTHOR]

The relationship between HS and OSA and nSD weakened in multivariate models, suggesting that other risk factors do contribute to OSA and nSD. HS is believed to induce a systemic pro-inflammatory state.4 We propose that OSA and nSD, which are also accompanied by systemic inflammatory states, may be triggered by the cytokine milieu of HS in susceptible individuals. A previous retrospective cohort study of 19 945 individuals with HS and 296 170 matched controls in the United States interrogated the incidence of OSA in HS patients and controls. [Extracted from the article]

Aims.: Immunological hypotheses have become increasingly prominent suggesting that autoimmunity may be involved in the pathogenesis of schizophrenia. Schizophrenia was found to be associated with a wide range of autoimmune diseases. However, the association between pemphigus and schizophrenia has not been established yet. We aimed to estimate the association between pemphigus and schizophrenia using a large-scale real-life computerised database. Methods.: This study was conducted as a cross-sectional study utilising the database of Clalit Health Services. The proportion of schizophrenia was compared between patients diagnosed with pemphigus and age-, gender- and ethnicity-matched control subjects. Univariate analysis was performed using χ 2 and Student's t -test and a multivariate analysis was performed using a logistic regression model. Results.: A total of 1985 pemphigus patients and 9874 controls were included in the study. The prevalence of schizophrenia was greater in patients with pemphigus as compared to the control group (2.0% v. 1.3%, respectively; p = 0.019). In a multivariate analysis, pemphigus was significantly associated with schizophrenia (OR, 1.5; 95% CI, 1.1–2.2). The association was more prominent among females, patients older than 60 years, and Jews. Conclusions.: Pemphigus is significantly associated with schizophrenia. Physicians treating patients with pemphigus should be aware of this possible association. Patients with pemphigus should be carefully assessed for comorbid schizophrenia and be treated appropriately. [ABSTRACT FROM AUTHOR]

Linked Article: Lee et al. Br J Dermatol 2020; 182:327–334. [ABSTRACT FROM AUTHOR]