Pemphigus Vulgaris and Bullous Pemphigoid of the Upper Aerodigestive Tract: A Review Article and Novel Approaches to Management

Abstract

<b><i>Background:</i></b> Autoimmune bullous diseases are rare conditions characterized by blistering of the skin and mucous membranes. The 2 commonest forms are pemphigus vulgaris and bullous pemphigoid. The oral cavity or oropharynx may be the initial site of presentation or often the only site involved. <b><i>Summary:</i></b> These conditions are often misdiagnosed or overlooked leading to poorer patient outcomes. Due to the chronic nature of these conditions and the systemic effects of treatment, there is a significant associated morbidity and mortality. As such, an understanding of the fundamentals of autoimmune bullous diseases is vital to those working in otolaryngology. The mainstay of management in both conditions is topical and systemic corticosteroids. There is also a role for immunomodulating and non-steroidal anti-inflammatory drugs as adjunct or alternative therapies. Surgical intervention may be required to protect the airway. Often multimodality treatment is required involving multidisciplinary input from otolaryngologists, oral surgeons, dermatologists, and rheumatologists. This review article will highlight the aetiology, pathology, clinical features, investigations, and management of both pemphigus vulgaris and bullous pemphigoid including recent advances in management.

Bullous Pemphygoid and Novel Therapeutic Approaches

“…In BP, several case reports and retrospective studies have demonstrated a good response, supporting its efficacy and safety [ 12 ]. The role of B-lymphocytes in the pathogenesis of bullous diseases encompasses several cellular functions including secretion of autoantibodies, aiding in T-cell activation, and production of pro-inflammatory cytokines.…”

Medical nutrition therapy in hospitalized patients with pemphigus vulgaris

“…6 Medical nutritional therapy, as part of a multidisciplinary approach for pemphigus patients, aims to prevent further protein loss, improve nutritional status, optimize fluid and electrolyte balance, accelerate wound healing, and help prevent relapse. 8,30 When the affected body surface area (BSA) is more than 30% or when at least two mucous membranes are affected, the condition can be categorized as severe. In addition, the response to injury increases metabolic needs and can raise energy requirements up to 250%.…”

The Management of Oral Pemphigus Vulgaris in a Hypertensive Patient: A Case Report

“…Initially, a thin-walled bulla arises on normal-looking mucosa or skin. Then, the bulla breaks rapidly and continues to spread peripherally and eventually leaves a large denuded area on the skin [ 4 ]. When pressure is applied to apparently normal skin, it may end up in the formation of a new lesion, which is called Nikolsky’s sign, resulting from pulling away the upper layer of the skin from the basal layer.…”