Thymoma-Associated Paraneoplastic Autoimmune Multiorgan Syndrome —From Pemphigus to Lichenoid Dermatitis
Paraneoplastic autoimmune multi-organ syndrome (PAMS) is a rare clinical condition characterized by variable and heterogeneous clinical phenotypes in the presence of neoplasias which largely depend on the activation of humoral and cellular immune responses. Clinically, these patients present with a spectrum of pemphigus-like lesions to graft-vs-host-disease-like exanthemas with a lichenoid inflammatory infiltrate in the skin. PAMS is occasionally associated with thymoma. We here present a patient with relapsing thymoma who developed PAMS with several cutaneous and extracutaneous autoimmune disorders. Peripheral blood lymphocytes, sera and lesional skin biopsies were obtained at different clinical disease stages. Peripheral T cell subsets were characterized phenotypically and the cytokine profile of the peripheral blood T cellular response against distinct epidermal and dermal autoantigens of the skin was analyzed by ELISpot assay. Serological screening was performed by ELISA and immunoblot analysis. Skin biopsies were subjected to immunohistochemical analysis of distinct T cell subsets. Thymoma tissue was analyzed for the presence of T regulatory cells and compared with adult thymus and indolent thymoma. In the present case, thymoma was the cause of the observed multi-organ autoimmune syndromes as its recurrence and surgical removal was associated with the relapse and regression of the cutaneous symptoms, respectively. Initially, the patient presented with two autoantibod...
CONCLUSIONS AND CLINICAL IMPORTANCE: There has been a remarkable expansion of knowledge on Malassezia yeasts and their role in animal disease, particularly since the early 1990's. Malassezia dermatitis in dogs and cats has evolved from a disease of obscurity and controversy on its existence, to now being a routine diagnosis in general veterinary practice. Clinical signs are well recognised and diagnostic approaches are well developed. A range of topical and systemic therapies is known to be effective, especially when predisposing factors are identified and corrected. PMID: 31957204 [PubMed - in process]
PMID: 31957203 [PubMed - in process]
Publication date: Available online 20 January 2020Source: Integrative Medicine ResearchAuthor(s): Min Hee Kim, Chunhoo Cheon, Hae Jeong Nam, Bonghyun Kim, Inhwa Choi
Publication date: Available online 20 January 2020Source: Methods in MicrobiologyAuthor(s): Maria M. Plummer, Charles S. PaviaAbstractImmunoassay detection of specific antigens and host-produced antibodies directed against such antigens constitute two of the most widely used and successful methods for diagnosing infectious diseases and many of the non-infectious autoimmune disorders. We will be reviewing some of the epidemiology, microbiology, and clinical aspects of selected organisms and discuss their diagnosis by describing some of the major antigen detection systems and serologic techniques available to health care pro...
Since its inception, Kidney International has published seminal articles describing the effects of systemic autoimmune diseases on the kidney. These manuscripts have characterized the pathology and clinical course of these diseases and perhaps most importantly have provided guidance to the nephrology community about how to treat these complex conditions using the best possible evidence available at the time. To illustrate the wealth of information contained within the Kidney International archives, consider the following papers.
Publication date: Available online 14 December 2019Source: Best Practice &Research Clinical RheumatologyAuthor(s): Ben Mulhearn, Sarah L. Tansley, Neil J. McHughAbstractAutoimmune connective tissue diseases are heterogeneous rheumatic diseases with the potential to affect multiple body systems. Autoantibodies are a characteristic feature of these diseases and are typically highly disease specific. In addition to aiding diagnosis, many autoantibodies have established associations with clinically important disease complications including internal organ involvement. In this chapter, we review the autoantibodies relevant t...
Zhou and colleagues detected ectopic lymphoid structures (ELS) that resemble tertiary lymphoid organs in pemphigus vulgaris and pemphigus foliaceous lesions. ELS were characterized by B cells reactive to the desmoglein 3 autoantigen, and were associated with active disease and B cell expansion. B cell differentiation in ELS was supported by the detection of centroblasts, plasmablasts, and plasma cells in these lesions. Chemokines that may induce B cell migration to pemphigus lesions were also detected in ELS.
Pemphigus is an autoimmune bullous disease characterized by IgG production against desmogleins. The major sites of autoantibody production are thought to be lymph nodes, spleen, and bone marrow. Previously, it has been suggested that autoreactive B cells might exist in the skin lesions in pemphigus and produce autoantibodies. In their report, Zhou et al. expanded their previous studies and reported that ectopic lymphoid-like structures were found in pemphigus skin lesions, wherein B-cell differentiation and lesional B-cell expansion might progress.
Understanding the functions of disease-associated noncoding variants is essential for understanding the molecular mechanisms driving diseases with a genetic cause and for identifying therapeutic targets. Combined computational and experimental analyses have demonstrated that IRF5 is hyperactivated by a pathogenic allele of TNPO3 through long-distance chromatin looping. This finding identifies a molecular mechanism contributing to the polygenic autoimmune diseases of systemic lupus erythematosus and systemic sclerosis.
(George Washington University) A recent study suggests that maternal depression in the postpartum period, and even beyond, is associated with the development of atopic dermatitis throughout childhood and adolescence.