IMPORTANCE Rituximab has emerged as a front-line therapy for pemphigus, but prognostic factors for achieving complete remission off therapy (CROT) with oral systemic agents remain unknown. OBJECTIVES To describe rates of CROT and relapse and identify prognostic factors for achieving CROT after rituximab therapy for pemphigus. DESIGN, SETTING, AND PARTICIPANTS A single-center, retrospective, cohort study was conducted at the University of Pennsylvania including 112 patients with pemphigus treated with rituximab with at least 12 months' clinical follow-up after the start of rituximab therapy. Multivariate regression analysis of factors predictive of CROT and Kaplan-Meier analysis of disease relapse were conducted. The study included patients treated with rituximab from

Autoimmune bullous diseases (AIBD), including pemphigus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, and pemphigoid gestationis, pose significant therapeutic challenges, especially in pregnant and post-partum breastfeeding patients or those planning to conceive. Data on the safety and efficacy of therapeutic interventions during the perinatal period are lacking because randomized controlled trials are typically not performed in this setting. However, many of the treatments for AIBD are also used in other diseases, so data can be extrapolated from studies or case reports in these other patient populations. It appears that many of the treatments for AIBD can adversely affect the fetus or neonate, and alterations in immune status caused by pregnancy-associated hormonal changes can negatively impact disease control. This article summarizes and weighs the risks and benefits of the various agents used to treat AIBD during pregnancy. We also present the available information on lactation as well as effects on male fertility.

Background Diagnostic criteria are used to identify a patient having a disease in a clinical setting, whereas classification criteria create a well-defined population for research purposes. The diagnosis and classification of amyopathic dermatomyositis (ADM) have not been recognized by most existing criteria for idiopathic inflammatory myopathies (IIMs). To address this, several criteria were proposed to define ADM either as a distinct disease entity or as a subset of the spectrum of IIMs. Objectives To discuss the diagnosis and classification of ADM and to assesses the available criteria in identifying cases of ADM and/or distinguishing it from dermatological mimickers such as lupus erythematosus. Methods We conducted an extensive literature search using the PubMed database from June 2016 to August 2018, using the search terms 'amyopathic dermatomyositis', 'diagnosis' and 'classification'. Results The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria, which are the only validated classification criteria for adult and juvenile IIM and their major subgroups, include three cutaneous items (G€ ottron sign, G€ ottron papules, heliotrope rash) to be able to classify ADM. This international and multispecialty effort is a huge step forward in the classification of skin-predominant disease in dermatomyositis. However, about 25% of the population with ADM do not meet two out of the three skin features and are misdiagnosed or classified as having a different disease entity, most commonly lupus erythematosus. Conclusions These gaps rationalize the continuous assessment and improvement of existing criteria and/or the development of validated, separate and skin-focused criteria for DM. What's already known about this topic?• The diagnosis and classification of amyopathic dermatomyositis (ADM) have not been recognized by most existing criteria for idiopathic inflammatory myopathies (IIMs).• The latest validated European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria perform well in capturing the majority of the ADM population using three skin variables: G€ ottron sign, G€ ottron papules and heliotrope rash.• However, about 25% of patients with ADM do not meet two out of the three skin features and fail the classification.What does this study add?• The gaps in the existing classification and diagnostic criteria for IIMs rationalize the continuous assessment and improvement of existing criteria and/or the development of validated and skin-focused criteria for DM. Diagnosis and classification of amyopathic dermatomyositis, J.S.S. Concha et al. 1003Heliotrope rash, G€ ottron papules, G€ ottron sign Heliotrope rash (blue-purple discoloration on the upper eyelids with or without associated oedema) Erythematous rash on the face, neck, anterior chest (V-sign) or back and shoulders (shawl sign), knees, elbows and malleoli Mechanic's hands (rough and cracked lateral and palmar areas of the fingers) Photosensitivity and pruritus