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Hematopoietic Stem Cell Transplantation in Patients with Autoimmune Bullous Skin Disorders
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Bullous skin diseases due to other conditions include paraneoplastic pemphigus and drug-induced bullous conditions. Paraneoplastic pemphigus is primarily associated with B cell lymphoproliferative diseases, although other malignancies, especially thymoma and certain sarcomas, have also been reported. Furthermore, many cases associated with Castelman’s disease have been reported. Autoantibodies are IgG 1 subclass and recognize a number of intra-and extracellular antigen domains including desmoplakin I & II, desmoglein and bullous pemphigoid-I antigen. Drug-induced pemphigus is caused by certain drugs such as penicillamine, captopril, enalapril, penicillins, cephalosporins, and rifampin. Vancomycin is commonly associated with drug induced linear IgA dermatosis. Both paraneoplastic and drug-induced pemphigus will be excluded from further discussion since treatment for paraneoplastic pemphigus is directed against the underlying malignancy, and drug-induced pemphigus recovers in 15-50% of cases with withdrawal of the offending drug.
Benign Oral and Dental Disease
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Konrad S. Staines, Alexander Crighton
Paraneoplastic pemphigus is suspected when a pemphigus lesion is difficult to manage. It is usually associated with lymphoproliferative disease or thymoma and the oral lesions may be the sole manifestation. A degree of suspicion is needed but an appropriate referral to a general physician for assessment is essential. Biopsies for pemphigoid and pemphigus must be taken from perilesional tissue otherwise no epithelium will be present on the specimen for proper examination.
Inflammatory dermatoses affecting the nail
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Paraneoplastic pemphigus, also called paraneoplastic autoimmune multiorgan syndrome, is characterized by intractable erosive lesions and blisters, sometimes with severe erosive nail lesions.251 It is mostly due to malignant lymphomas, chronic lymphocytic leukemia, Castleman's syndrome, thymoma, and a number of other less common malignancies.252
Castleman Disease Presenting with Pseudotumour Cerebri and Myasthenia Gravis: A Case Report and Literature Review
Published in Neuro-Ophthalmology, 2019
Alexander S. Fein, Humberto E. Trejo Bittar, Manisha R. Shende, Ernest M. Scalzetti, Melissa W. Ko
FDCS, an extremely rare neoplasm, has been reported in twenty-two other patients with CD, and has been shown to develop from the hyperplastic tissue in CD tumours.30–37 It is almost always found within unicentric, hyaline vascular CD, as it was in our patient. None of these patients had papilledema, and only one had MG and was included in our review of cases above.7 Paraneoplastic pemphigus was the only other autoimmune disorder reported, and all three of those patients died from either sepsis or respiratory failure soon after presentation. The remaining FDCS patients had varying presentations and outcomes, from complete resolution or local recurrence to metastasis, treatment failure and death.
Epidemiology of Castleman disease associated with AA amyloidosis: description of 2 new cases and literature review
Published in Amyloid, 2019
Antoine Fayand, David Boutboul, Lionel Galicier, Jean-Emmanuel Kahn, David Buob, Jean-Jacques Boffa, Alexandre Cez, Eric Oksenhendler, Gilles Grateau, Stéphanie Ducharme-Bénard, Sophie Georgin-Lavialle
CD diagnosis was made at a median age of 41 years (interquartile range Q1–Q3: 28.5–51). At CD diagnosis, systemic symptoms or persistently elevated inflammatory markers had been present for a median of 2 years. Sixty-five percent of CD cases were diagnosed following the exploration of unexplained AAA. There were 23 UCD and 14 MCD cases. Histological subtypes included 25 PC, five HV and seven mixed. No paraneoplastic pemphigus was diagnosed among UCD patients. AAA involved the kidney in 73% of patients, with a nephrotic syndrome in 82% of them, and the digestive tract in 27% of patients. Table 2 summarizes the characteristics of UCD and MCD cases associated with AAA.
Long-term experience with rituximab therapy for treatment-resistant moderate-to-severe pemphigus
Published in Journal of Dermatological Treatment, 2022
Burçin Cansu Bozca, Aslı Bilgiç, Soner Uzun
Infections are perhaps the most worrisome side effect of RTX treatment. However, studies regarding long-term biologic treatment for rheumatoid arthritis (56) and accumulating reports regarding RTX treatment for pemphigus (57) suggest that there is only a low risk of serious infections, such as tuberculosis and opportunistic infections. For example, a review of 136 patients, including patients with paraneoplastic pemphigus, identified 17 serious adverse events (including 2 deaths) in 14 of 103 patients (13.5%) with PV and 1 serious adverse event (death) in 20 patients with PF (5%) (33). The other events were bacterial sepsis (n = 4), bacterial pneumonia (n = 3), Pneumocystis jirovecii pneumonia (n = 2), pulmonary embolism (n = 2), and neutropenia (n = 2). Deep vein thrombosis, which we identified in one case, has been reported as a possible adverse event during RTX treatment (33,58). Nevertheless, the most common side effects are mild-to-moderate infusion reactions, which are usually observed at the first infusion, and severe infusion reactions are infrequent (<1%), which include anaphylaxis, anaphylactoid reactions, and angioedema (59). We did not identify any severe infusion reactions and only 2 of the 53 patients died. One of the deaths involved a cardiac complication, and we cannot rule out the potential contribution of RTX, although the other death was caused by sepsis and did not appear to be directly related to RTX. Thus, caution is warranted regarding immune dysregulation that can be caused by pemphigus, as well as the risk of infection that is related to immunosuppressive treatment. However, we do not believe that these side effects should discourage dermatologists from using RTX to effectively treat patients with pemphigus, as their disease itself can be fatal.