Pemphigus foliaceus (PF) is rarely described in the pediatric population with less than 40 cases reported in the literature. We report the case of an 11-year-old girl who was diagnosed with PF after 6 months of starting with symptoms and who responded well to therapy with oral dapsone. Although therapeutic guidelines for PF in children are lacking, oral corticosteroids in combination with dapso...

Pemphigus is an antibody-mediated autoimmune skin disease in which loss of cell-to-cell contacts in the epidermis results in blister formation. Patients with pemphigus develop antibodies that bind to the keratinocyte cell surface, the site of primary pathology. The purpose of this study was to characterize the antigen(s) to which pemphigus antibodies bind. Because we could detect pemphigus anti...

© 2014 The Authors. doi: 10.2340/00015555-1781 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Pemphigus erythematosus (PE) is a variant of pemphigus foliaceus. PE patients exhibit the immunological features of both pemphigus and lupus erythematosus (LE), i.e. granular immunoglobulin (Ig) and C3 deposition in the basement membrane zone (BMZ), intercellular IgG and C3 depos...

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...