Atopic dermatitis (AD) is a common, chronic, relapsing-remitting inflammatory disease that can be challenging to treat. Patients with mild disease are usually managed well with good skin care practices including moisturization and appropriate bathing along with intermittent use of topical therapies such as topical corticosteroids and/or topical calcineurin inhibitors during flares. Patients with frequent flares may benefit from proactive application of topical therapies twice a week to the most troublesome areas. Patients with severe disease often present significant treatment challenges. Systemic therapies are usually required for severe AD but have varying degrees of success and can be associated with side-effect profiles that require counseling and close monitoring. Phototherapy has been shown to have success in treating moderate-to-severe AD, but several factors can limit its utility and efficacy including cost and access. New therapies are in development targeting specific pathways relevant for AD. Dupilumab was the first biologic treatment approved in North America, Europe, and Japan for adults with moderate-to-severe AD. Although this treatment can lead to rapid improvement in the majority of patients, there are inadequate responders. In this review, we discuss the clinical challenges and treatment options for moderate-to-severe refractory AD.
Delayed myocardial enhancement MRI is a highly valuable but non-specific imaging technique that is ancillary in the diagnosis of a variety of diseases including myocardial viability, cardiomyopathy, myocarditis and other infiltrative myocardial processes. The lack of specificity stems from the wide variety of differential diagnoses that may present with overlapping patterns of delayed enhancement. Many of these differential diagnoses have been presented and discussed in this article.
Malignant rhabdoid tumors (MRTs) are well recognized in the kidney and extrarenal sites such as soft tissues, retroperitoneum, and bladder but are classified as atypical teratoid/rhabdoid tumors in the central nervous system. The unifying features of both extracranial MRT and atypical teratoid/rhabdoid tumors are the exon deletions/mutations of the SMARCB1 (SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1) gene in 22q11.23 and resulting loss of SMARCB1/INI1 (integrase interactor 1) protein expression by immunohistochemistry. We herein report a case of extrarenal rhabdoid tumor confined to the bladder in a 3-year-old child, diagnosed by histopathology and confirmed by immunohistochemical and molecular studies. This is only the fourth molecularly proven primary MRT of the bladder to be reported. The patient’s peripheral blood was negative for the deletions observed in the tumor, thereby confirming a sporadic origin for the tumor. Given the possible dismal outcome, urgency for definitive diagnosis to institute intensive multimodality therapy, histopathologic differential diagnosis with rhabdomyosarcoma and urothelial carcinoma with rhabdoid features, and lack of consensus management guidelines, oncologists, urologists, and pathologists must be aware of this entity. Evaluation for a germ line SMARCB1 alteration may greatly aid risk stratification and family planning.
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