21 research outputs found
Paraneoplastic Pemphigus Mimicking Toxic Epidermal Necrolysis Associated with Follicular Lymphoma: Possible Pathological Role of CD8 T Cells.
Usefulness of a lead delivery system consisting of a fixed-shaped sheath and a lumenless bipolar lead in a patient with absent right and persistent left superior vena cava: A case report
We report the case of an 84-year-old female with symptomatic bradycardia due to a complete atrioventricular block, who carried absent right and persistent left superior vena cava (SVC). Implantation of a pacing lead, particularly within the right ventricle (RV) in a patient with this venous anomaly is accompanied by technical difficulties. However, the apparatus consisting of a fixed-curve sheath (Model C315-S10, Medtronic, Inc., Minneapolis, MN, USA) and a lumenless fixed-screw pacing lead (Model 3830, Medtronic), allowed a rapid delivery into the RV without any complications. By rotating the Model C315-S10 sheath in the counterclockwise direction in the right atrium, its tip faced the tricuspid orifice, advanced across the tricuspid valve and confronted the RV lower septum near the apex. Then the RV-lead was fixed with acceptable pacing and sensing parameters. Utilizing a lumenless pacing lead and a preformed sheath to deliver it is a novel approach that could be helpful in pacemaker implantation in patients with absent right and persistent left SVC. Keywords: Persistent left superior vena cava, Absent right superior vena cava, Pacemaker, Sheat
Ustekinumab successfully treated a patient with severe psoriasis vulgaris with primary failure to infliximab and secondary failure to adalimumab
Biologic drugs have been recently used to treat psoriasis. However, some patients do not respond or lose therapeutic benefit with first-line use of tumor necrosis factor (TNF) antagonists. We report a case of psoriasis vulgaris, that failed to respond to TNF antagonists, infliximab and adalimumab, completely disappeared after treatment with ustekinumab, a therapeutic agent for biologically blocking p40 protein of interleukin (IL) 12 and 23. This report highlights anti-TNF agents only inhibited the TNF-α/inducible nitric oxide synthase (iNOS)-producing dendritic cells (TIP-DCs), but the plasmacytoid-DC-derived psoriatic response was re-initiated. On the other hand, ustekinumab may inhibit both the TIP-DCs and the plasmacytoid-DC-derived inflammatory response
Classification and Antigen Molecules of Autoimmune Bullous Diseases
Autoimmune bullous diseases (AIBDs), which are a group of tissue-specific autoimmune diseases of the skin, present with various blistering lesions on the skin and mucous membranes, and show autoantibodies of IgG, IgA and IgM against epidermal cell surfaces and basement membrane zone. To date, AIBDs have been classified into a number of distinct subtypes by clinical and histopathological findings, and immunological characteristics. In addition, various biochemical and molecular biological studies have identified various novel autoantigens in AIBDs, which has resulted in proposals of new subtypes of AIBDs. In this article, we summarized various distinct AIBDs, and proposed the latest and most comprehensive classification of AIBDs with their autoantigen molecules
A Rare Case of Male Bullous Lupus Erythematosus Complicated with Subsequent Annular Hypopigmentation
A 57-year-old male had been suffering from an itchy map-shaped symmetrical erosive erythema with a crust that was attached to his upper arm and buttock, and occasionally he suffered from spiking fever. Laboratory examinations showed neither anti-desmoglein 1/3 antibodies nor anti-BP 180 antibodies, and he fulfilled the criteria for a diagnosis of systemic lupus erythematosus (SLE). Histologically, there was eosinophilic necrosis of keratinocytes, liquefaction and degradation with severe lymphocyte infiltration into the epidermis and subepidermal blister formation, suggestive of a variant of SLE, bullous lupus erythematosus (BLE). One month after remission of BLE, peculiar annular hypopigmentation appeared on the peripheral borders. An immunohistochemical analysis showed a decrease in Melan A-positive melanocytes and concomitant pigment incontinentia, with dense infiltration of CD8+ T cells and IL-17A+ Th17 cells. An ultrastructural analysis revealed a decrease, but not a complete disappearance, of both melanocytes and melanosomes, and no impairment in melanosomal transfer. In this case report, we would like to introduce the development of annular depigmentation complicated with BLE, and discuss the effects of lupus condition on melanocyte damage based on immunohistological and electromicroscopic findings of those vitiliginous lesions