Juvenile pemphigus (JP) is a rare disease. Of 274 pemphigus patients who were under our care during the last 26 years, 4 (1.4%) were in the JP category. The course and prognosis of the disease in the 4 patients who have been followed for 4–19 years are presented. Two patients are still in complete remission and off treatment for 4 and 6 years. In view of the literature and our experience, it appears that the clinical manifestations and course of JP are variable, similar to adult pemphigus. However, it seems that JP has a favorable prognosis quoad vitam.
New classes of drugs including the proteasome inhibitors (PI) bortezomib and, more recently, carfilzomib and the immunomodulatory agent lenalidomide have shown improved outcomes for multiple myeloma (MM) patients during the past decade. However, most of the studies reporting outcomes for patients receiving these drugs have relied on older data sets derived from large institutions that included patients not receiving their treatment at those facilities and represented only those eligible for clinical trials or were from sites where treatment options were limited. We have analyzed data from 258 MM patients who have received treatment with at least one of three agents: bortezomib, carfilzomib, and lenalidomide in a single clinic specializing in MM with respect to their responses and other outcomes to treatment regimens including these agents. Response rates were similar between these three drugs when used for the first time and again during subsequent treatment regimens. As expected, the clinical benefit rates (CBRs) were better for patients receiving their first treatment when compared to their use in subsequent treatment regimens. The CBRs were similar during their 2nd, 3rd, and 4th treatments containing these agents. Many patients refractory to these agents showed responses to regimens containing these same drugs when used in different combinations. In addition, patients refractory to one PI often responded to the other PI. The results of this study demonstrate that novel agents can be used repeatedly in novel combinations with significant clinical benefit for patients with MM.
Two patients with esophageal involvement of pemphigus vulgaris are presented, who were diagnosed histopathologically and by direct immunofluorescence. In most dermatological textbooks the possibility of esophageal involvement is not mentioned. In the English medical literature 11 cases have been reported. It seems that if upper gastrointestinal endoscopy had been done on a routine basis it might have shown that esophageal involvement is more widespread than present findings indicate.
One of the common lesions in Crohn's colitis is erythema nodosum, a septal panniculitis that may appear before diagnosis or in conjunction with flare up. We report a case of Crohn's colitis where the presenting sign was neutrophilic lobular panniculitis with few granulomas. The possibility that this case represents a forme fruste of metastatic Crohn's is suggested. As skin biopsies are not usually performed by gastroenterologists in erythema nodosum-like skin lesions in Crohn's patients it may well be that the incidence of lobular panniculitis is higher than reported in the literature.
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