Autoimmune blistering dermatoses in children

Autoimmune blistering skin disorders represent arare group of autoantibody-induced dermatoses against desmosomal and hemidesmosomal molecules. The common age of onset for pemphigus and pemphigoid, as well as dermatitis herpetiformis, encompasses the adult age, but all these disorders can be observed neonatally and/or during childhood. If the disease occurs postpartum or neonatally, physicians should consider transplacental transmission of pathogenic maternal immunoglobulin G (IgG)-autoantibodies, and both mother and child should be included in the diagnostic work up. If the disorder is suspected in childhood, early immunoserological testing and skin biopsies for direct immunofluorescence analyses are recommended for the correct diagnosis and subsequently for the right choice of treatment. First-line recommendations are nonhalogenated topical steroids, followed by oral dapsone. All therapies require preliminary examinations, e.g. enzyme-activity testing (as is glucose-6-phophate dehydrogenase in dapsone treatment). In refractory cases, further treatment choices like high-dose intravenous immunoglobins, plasmapheresis/immunoadsorption or targeted therapies like anti-CD20 autoantibody therapies are indicated. An intense dermatological support and good medical care are essential for an age-appropriate development of the child and to lower possible treatment-associated adverse events.