BACKGROUND Castleman tumor, a rare lymphoproliferative disorder, is one of the associated tumors in paraneoplastic pemphigus. We analyzed the characteristics of a group of patients with Castleman tumor to clearly understand and to improve the prognosis of the disease. OBSERVATIONS Ten cases of paraneoplastic pemphigus associated with Castleman tumor treated in the Department of Dermatology, P...

UNLABELLED A RARE DISEASE: Paraneoplastic pemphigus is an rare autoimmune bullous skin disease recently recognized. About 50 cases have been reported since its first description in 1990. CLINICAL MANIFESTATIONS Clinical signs are polymorphous resembling the cutaneomucosal manifestations of pemphigus vulgar (skin and mucosa erosions, fragile interdermal bullae), pemphigoid (urticaria, distende...

INTRODUCTION Pemphigus is a rare autoimmune blistering disease, which requires prolonged administration of corticosteroids at high doses. Although this therapy improves the health and lives of patients, it may have various side effects, for example osteoporosis. AIM To assess the concentration of osteoprotegerin (OPG), the soluble receptor activator of nuclear factor-κβ ligand (sRANKL) and os...

BACKGROUND Pemphigus is part of a group of blistering diseases that affect the skin and mucous membranes. Based on its autoimmune origin, autoantibodies develop in pemphigus that are directed toward cell surface components of keratinocytes. However, some data cannot be explained, such as the lack of a relationship between autoantibody levels and the severity of clinical manifestations, treatmen...

OBJECTIVES Regarding the implication of viruses particularly herpes in pemphigus vulgaris, we sought to assess and compare the level of immunoglobulin G (IgG) antibodies against herpes simplex virus types 1 and 2 (HSV1 and HSV2), cytomegalovirus (CMV) and Epstein-Barr virus (EBV) in patients with pemphigus vulgaris and healthy people. MATERIALS AND METHODS In this cross-sectional study, 25 pa...

BACKGROUND There is increasing evidence that a single cycle of rituximab (375 mg per square meter [corrected] of body surface area once weekly for 4 weeks) is efficacious in patients with severe pemphigus. The approved protocol in rheumatoid arthritis is 1 g on days 1 and 15. We report herein on the efficacy and safety of this latter protocol for rituximab in 9 patients with pemphigus. OBSERV...

Pemphigus vulgaris is a rare, life-threatening, autoimmune bullous disease. After decades of systemic corticosteroids and corticosteroid-sparing immunosuppressants being used to control the disease, efficacy rituximab has been shown in randomized controlled studies. Hence constitutes first-line treatment for mild moderate-to-severe pemphigus according most recent European S2K guideline. Despite...

Pemphigus vulgaris pathogenesis had been considered well understood and was in danger of being “finished,” as was physics at the end of the 19th century. With the publication in this issue of a paper by Nguyen and colleagues (1), the understanding of pemphigus pathogenesis must undergo a major revision. This turning point brings with it the promise of a more complete picture, as well as new the...

Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a life-threatening disease associated with an underlying malignancy, most commonly of lymphoproliferative origin. The clinical picture may resemble pemphigus, pemphigoid, erythema multiforme, graft-versus-host disease, or lichen planus. Th...