Bullous Diseases in Children: A Review of Clinical Features and Treatment Options.

Abstract

Bullous diseases are uncommon in children; however, as they have the potential to affect quality of life, occasionally have long-term side effects in the setting of scarring processes, and carry a rare risk of underlying malignancy [e.g., with paraneoplastic pemphigus (PNP)], knowledge of their clinical presentation and treatment options is essential. Given the rarity of these conditions, our current state of knowledge is largely derived from case reports and case series, with a paucity of evidence-based recommendations. In this review, we discuss the clinical presentation of and treatment options for linear immunoglobulin A disease, dermatitis herpetiformis, pemphigus vulgaris, pemphigus foliaceus, PNP, bullous pemphigoid, mucus membrane pemphigoid, epidermolysis bullosa acquisita, and inherited epidermolysis bullosa. In general, when these conditions, except for PNP, occur in childhood, they have a better prognosis than when they occur in adults. Clinical, histopathological, and immunologic features frequently overlap, but distinct differences have also been reported, most commonly in clinical presentation. Treatment is often similar to that in adults, although specific considerations are necessary for a pediatric population.