Pathophysiology of autoimmune bullous diseases: Nature versus nurture

Abstract

Pemphigus and pemphigoid are the prototypical immunobullous diseases. Although it has been well established that they are caused by deposition of autoreactive antibodies directed against adherence proteins within the skin, the specific genetic and environmental factors leading to development of these diseases continue to be an area of investigation. Herein, we discuss several of the potential environmental triggers that may induce patients to develop immunobullous diseases including medications, viral infections, UV exposure or other radiation injury and dietary factors. In addition, the potential genetic and immunologic mechanisms contributing to the pathogenesis of pemphigus and pemphigoid will be reviewed. The multifactorial nature of these diseases contributes to their complexity and highlights the importance of a detailed personal and family history when caring for these patients.

Bullous Pemphigoid

“…While the incidence in Europe ranges from 7 to 43 per million [10], this rate is 2.6 per million in Basra and 14 per million in the North East Scotland [2]. It is often seen in the elderly population (particularly in over 65 years of age) and more frequent in men [5,11]; however, it has been cited in many studies that it is seen with equal frequency in both genders [4]. Patients older than 90 years have also a relative risk 300 times higher than those younger than 60 years [5].…”

“…It is often seen in the elderly population (particularly in over 65 years of age) and more frequent in men [5,11]; however, it has been cited in many studies that it is seen with equal frequency in both genders [4]. Patients older than 90 years have also a relative risk 300 times higher than those younger than 60 years [5]. Although it is rare in children, its incidence has been increasing [7].…”

“…Bullous pemphigoid (BP) is a chronic, acquired, autoimmune bullous disease characterized by subepidermal bullae [1][2][3][4]. It is the most common bullous disease, and its incidence has been gradually increasing [2,5]. It is usually seen in the elderly, but rarely may also be seen in children [2,6,7].…”

“…It is usually seen in the elderly, but rarely may also be seen in children [2,6,7]. In general, the clinical manifestations are tense bullae, urticarial lesions and intense pruritus [3,5,7]. Although mucosal findings are not common, oral findings are observed in 10-25% of cases [4,5].…”

“…In general, the clinical manifestations are tense bullae, urticarial lesions and intense pruritus [3,5,7]. Although mucosal findings are not common, oral findings are observed in 10-25% of cases [4,5]. Autoantibodies against hemidesmosomal proteins BP230 (BPAG1) and BP180 (BPAG2 or type XVII collagen) are blamed for the pathogenesis [2].…”