Pénfigo vulgar tipo cutáneo: Caso clínico

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Translated abstract

Pemphigus is an autoimmune bullous disease that involves skin and mucous membranes caused by autoantibodies against antigens on the surface of keratinocytes. We report a 30-year-old male presenting with a five months history of pruriginous alopecic and crusted lesions in the scalp, that extended posteriorly to the trunk and limbs. Mucous membranes were not involved. A skin biopsy was performed, showing extensive loss of epidermis and acantholysis. Immuno fluorescence was positive for C3 and intercellular and epidermic IgG. With the presumptive diagnosis of pemphigus vulgaris (PV) without mucous involvement, the patient was treated with prednisone, observing an excellent clinical response. There are only few cases published in the literature of PV without mucous involvement. Some authors refer to this subtype of PV as "Cutaneous pemphigus vulgaris".

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Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus.

Juana Alba Luis-Díaz, Victoria P Werth, Dedee Murrell … (2008)

Our scientific knowledge of pemphigus has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in pemphigus. A major obstacle in comparing therapeutic outcomes between centers is the lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus. Common terms and end points of pemphigus are needed so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. This consensus statement from the International Pemphigus Committee represents 2 years of collaborative efforts to attain mutually acceptable common definitions for pemphigus. These should assist in development of consistent reporting of outcomes in future studies.

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A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus.

Linda K Martin, Victoria P Werth, Elmer V Villaneuva … (2011)

A range of interventions has been described for the treatment of pemphigus; however, the optimal therapeutic strategy has not been established. We sought to evaluate the safety and efficacy of interventions for pemphigus vulgaris and pemphigus foliaceus. We undertook a systematic review and meta-analysis according to the methodology of the Cochrane Collaboration. We selected randomized controlled trials including participants with the diagnosis of pemphigus vulgaris or pemphigus foliaceus confirmed with clinical, histopathological, and immunofluorescence criteria. All interventions were considered. Primary outcomes studied were remission and mortality. Secondary outcomes included disease control, relapse, pemphigus severity score, time to disease control, cumulative glucocorticoid dose, serum antibody titers, adverse events, and quality of life. Eleven studies with a total of 404 participants were identified. Interventions assessed included prednisolone dose regimen, pulsed dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth factor, and traditional Chinese medicine. We found some interventions to be superior for certain outcomes, although we were unable to conclude which treatments are superior overall. Many interventions for pemphigus have not been evaluated in controlled trials. All studies were insufficiently powered to establish definitive results. There is inadequate evidence available at present to ascertain the optimal therapy for pemphigus vulgaris and pemphigus foliaceus. Further randomized controlled trials are required. Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.