INTRODUCTION Pemphigus vulgaris (PV) is a chronic and infrequent autoimmune mucocutaneous disease that is characterized by the loose blisters and erosions on the skin and mucous membrane. Middle-aged adults are affect most frequently and the elderly and juvenile cases are infrequent. Herein, we reported a case of pemphigus vulgaris in an elderly patient. CASE PRESENTATION We reported a case o...

Pemphigus is a severe autoimmune-blistering disease of the skin and mucous membranes caused by autoantibodies reducing desmosomal adhesion between epithelial cells. Autoantibodies against the desmosomal cadherins desmogleins (Dsgs) 1 and 3 as well as desmocollin 3 were shown to be pathogenic, whereas the role of other antibodies is unclear. Dsg3 interactions can be directly reduced by specific ...

Fifty patients with pemphigus (45 pemphigus vulgaris, 5 pemphigus foliaceus) were treated with dexamethasone-cyclophosphamide pulse therapy. The pulse consisted of 136 mg dexamethasone dissolved in 5% dextrose given in a drip over a period of 1-2 hours on 3 consecutive days. In addition, 500 mg cyclophosphamide was added in the drip on the first day. Such pulses were given at monthly intervals....

© 2014 The Authors. doi: 10.2340/00015555-1811 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Pemphigus is an autoimmune intraepidermal blistering disease (1). The autoimmune target of the antibodies is desmoglein, a cadherin-like cell-to-cell adhesion molecule found in desmosomes. In pemphigus vulgaris (PV), the main antigen is desmoglein 3 (Dsg 3), which is located on t...

In this study a robust and simple classification into different stages of pemphigus, based on a set of therapeutic benchmarks including a definition of disease activity, is proposed. This staging method was validated against ELISA titres for autoantibodies against desmoglein-1 and -3. The proposed staging method was applied a priori to five newly diagnosed pemphigus patients in the course of th...

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...

The tegumentary system becomes a scenario for immune responses. The knowledge of these conditions has led to induction of complementary animal models, better knowledge of the pathophysiology, and new tools [1] for diagnosis and therapy of these autoimmune skin disorders. In this special issue from Autoimmune Diseases we have invited several papers to address the dermatological issue. One paper ...

© 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...