Dermatitis Herpetiformis and Linear IgA Bullous Dermatosis

Linear IgA bullous dermatosis

“…There are some important differences between the two diseases: (i) DH is associated with a gluten‐sensitive enteropathy in almost all cases which responds to a gluten‐free diet, 93 whereas in LABD there is no such association and no improvement with a gluten‐free diet; 94 (ii) DH is closely associated with the HLA‐B8‐DR3 haplotype but there is no association with LABD; 95 (iii) the IgA found in the skin of DH patients is in granular deposits in the dermal papillae and no circulating BMZ‐specific antibodies have been detected in DH. 96 Thus while DH and LABD share some features, they differ significantly with regard to immunopathology.…”

A necessidade da imunofluorescência direta no diagnóstico da dermatose bolhosa por IgA

“…Assim, IFD é o método laboratorial fundamental para o diagnóstico exato, já que somente dados clínicos e histopatológicos não são suficientemente sensíveis e específicos para o diagnóstico preciso das dermatoses bolhosas autoimunes. Dessa forma, a deposição linear e homogênea de IgA na ZMB confirma o diagnóstico de DbIgA, haja vista que na DH o IgA se deposita de forma granular em derme papilar, enquanto no PB há deposição linear e homogênea em ZMB de C3 (7,(11)(12)(13) .…”

Linear IgA bullous dermatosis in a patient with sclerosing cholangitis

“…Linear IgA bullous dermatosis (LABD) is a rare, blistering disease characterized by pruritic, subepidermal blisters. This entity was once considered a subset of dermatitis herpetiformis (DH) but has since been reclassified as a distinct entity based on the distinct linear deposition of IgA at the epidermal basement membrane and the presence of gluten‐sensitive enteropathy and IgA endomysial antibodies in DH and their absence in LABD 1,2 . Primary sclerosing cholangitis is an autoimmune disease characterized by a progressive, inflammatory sclerosing, and obliterative process affecting the bile ducts.…”