Monoclonal immunoglobulin light chain (LC) crystalline inclusions within podocytes are rare, poorly characterized entities. To provide more insight, we now present the first clinicopathologic series of LC crystalline podocytopathy (LCCP) encompassing 25 patients (68% male, median age 56 years). Most (80%) patients presented with proteinuria and chronic kidney disease, with nephrotic syndrome in 28%.
View Article and Find Full Text PDFBackground: Cases of severe drug hypersensitivity, demonstrating a variable spectrum of cutaneous and systemic involvement, are reported under various names, especially drug reaction with eosinophilia and systemic symptoms (DRESS). Case definition and overlap with other severe cutaneous adverse reactions (SCAR) are debated.
Objectives: To analyse the spectrum of signs and symptoms of DRESS and distribution of causative drugs in a large multicentre series.
Background: Generalized bullous fixed drug eruption (GBFDE) is a rare cutaneous adverse reaction to drugs, and may resemble Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN), but is usually considered less severe.
Objectives: To compare the severity and mortality rate in cases of GBFDE and control cases of SJS or TEN of similar extent of skin detachment.
Methods: This was a case-control analysis of 58 patients with GBFDE matched by age and extent of skin detachment to 170 control patients with a validated diagnosis of SJS or SJS/TEN overlap.