Autoimmune blistering diseases in children

Abstract

The acquired autoimmune bullous diseases are rare in children, but early recognition and prompt treatment are important. A high index of suspicion is necessary to diagnose these conditions that frequently mimic more common childhood diseases. Because there is clinical and histological overlap between these groups of diseases, direct immunofluorescence and/or indirect immunofluorescence are necessary for diagnostic confirmation. The quality of published data regarding the treatment of autoimmune bullous diseases in children is poor, as there are no controlled or comparative trials. This makes it difficult to draw conclusions regarding the best treatment algorithms. Nevertheless, numerous case reports and small, mostly retrospective, case series do suggest treatment options. The goal of treatment is to suppress disease activity and control symptoms with therapies that minimize the potential for serious short and long term adverse effects. Since children may be especially vulnerable to certain medication side effects (e.g., growth retardation from corticosteroids), this makes choosing the best treatment even more important. This chapter will review the most common autoimmune bullous diseases occurring in children: chronic bullous disease of childhood, dermatitis herpetiformis, bullous pemphigoid, epidermolysis bullosa acquisita, and pemphigus.