TY - CHAP M1 - Book, Section TI - Linear Immunoglobulin A Dermatosis and Chronic Bullous Disease of Childhood A1 - Nicholas, Matilda W. A1 - Rao, Caroline L. A1 - Hall III, Russell P. A2 - Kang, Sewon A2 - Amagai, Masayuki A2 - Bruckner, Anna L. A2 - Enk, Alexander H. A2 - Margolis, David J. A2 - McMichael, Amy J. A2 - Orringer, Jeffrey S. Y1 - 2019 N1 - T2 - Fitzpatrick's Dermatology, 9e AB - AT-A-GLANCELinear Immunoglobulin A DermatosisRare blistering disease with onset typically after fourth decade of life.Linear band of immunoglobulin (Ig) A at the dermal–epidermal basement membrane.Clinical presentations may mimic dermatitis herpetiformis, bullous pemphigoid, and cicatricial pemphigoid.May occur in association with many drugs, including vancomycin.May occur in association with inflammatory bowel diseases, but is only rarely associated with gluten-sensitive enteropathy.Rarely seen in association with malignancy, specifically lymphoid malignancy.Histology shows subepidermal collection of neutrophils at the basement membrane, often collecting in papillary tips with subepidermal blisters.Patients have relatively low titers of circulating IgA autoantibodies, most frequently against portions of BPAG2 (type XVII collagen), or rarely against BPAG1, LAD 285, type VII collagen, and others.Most patients respond dramatically to treatment with dapsone; some require adjunctive systemic corticosteroids.Prognosis is variable with both spontaneous remissions and longstanding disease.Chronic Bullous Disease of ChildhoodRare blistering disorder of childhood presenting predominantly in children younger than 5 years of age.Linear IgA at the dermal–epidermal basement membrane.Clinical presentation of tense bullae, often in perineum and perioral regions, giving a “cluster-of-jewels” appearance. New lesions sometimes appear around the periphery of previous lesions with a collarette of blisters.Histology shows subepidermal collection of neutrophils at the basement membrane, similar to linear IgA bullous dermatosis.Most patients respond dramatically to treatment with dapsone.Spontaneous remissions, often within 2 years, are frequent. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1162816136 ER -