Autoimmune blistering disorders are a heterogeneous group of diseases that result from autoantibodies generated against target antigens found in the skin and mucous membranes. This process leads to a variety of disruptions in keratinocyte adhesion and cellular integrity, resulting in fluid accumulation and development of blisters. Physicians should have an appreciation and understanding of autoimmune blistering disorders in the pediatric population when formulating a differential diagnosis of a patient who presents with skin blistering. Early detection and discrimination between the varied autoimmune blistering disorders can change the course of treatment and outcome. Due to the similarity in clinical presentation among different diseases within this category, histopathologic evaluation and, especially, immunofluorescence studies are necessary to establish the definitive diagnosis.
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