High-dose intravenous immunoglobulin (hdIVIg) is being used increasingly for dermatological indications. Its mode of action is via a number of proposed mechanisms and it is not associated with the many side-effects of steroids and other immunosuppressive agents. The evidence for using hdIVIg in the treatment of autoimmune bullous disorders is based on uncontrolled trials and case reports. Howev...

BACKGROUND Epidermolysis bullosa acquisita (EBA) is a rare, chronic, autoimmune bullous dermatosis that is caused by autoantibodies against the noncollagenous terminus of the α chain of type VII collagen, resulting in decreased anchoring fibrils in the lamina densa. It classically presents with skin fragility and trauma-induced blisters that are particularly extensive over the distal aspect of ...

B ullous pemphigoid is an autoimmune disease, which means that the cells in the body that normally fight infection attack the body instead. The body’s immune system is confused and makes an antibody (type of protein used to fight infection) that targets a part of the skin that normally holds it together. The attack on the skin causes blisters (firm, fluid-filled bubbles on the skin) to form. Th...

An Bras Dermatol. 2017;92(4):584-95. Dear editor, Pemphigus is an autoimmune bullous disease that can affect the skin and mucous membranes, mediated by autoantibodies against desmosomal desmogleins, the main adhesion structures Marina Zoega Hayashida1 Jhonatan Rafael Siqueira Pinheiro1 Milvia Maria Simões e Silva Enokihara2 Mônica Ribeiro de Azevedo Vasconcellos1 t Biologic therapy-induced pemp...

Bullous pemphigoid is a subepidermal autoimmune blistering disease associated with autoantibodies to the hemidesmosomal bullous pemphigoid antigens 180 and 230. Most sera from bullous pemphigoid patients recognize epitopes within the N-terminal NC16A portion of the bullous pemphigoid 180 ectodomain. Using cryosections of human skin, patients' sera were shown to generate dermal-epidermal separat...

Autoimmune bullous diseases are relatively uncommon and their treatment -- although generally similar -- may vary depending on the dermatologist. Within this group of diseases, the most common are pemphigus vulgaris and pemphigus foliaceus, bullous and mucosal pemphigoid, linear immunoglobulin A disease, and dermatitis herpetiformis. In recent years, the therapeutic arsenal has been extended by...

Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple p...

Autoimmune bullous diseases are relatively uncommon and their treatment—although generally similar—may vary depending on the dermatologist. Within this group of diseases, the most common are pemphigus vulgaris and pemphigus foliaceus, bullous and mucosal pemphigoid, linear immunoglobulin A disease, and dermatitis herpetiformis. In recent years, the therapeutic arsenal has been extended by new d...

Lichen planus pemphigoides (LPP) is an autoimmune disease characterised by evolution of subepidermal blisters on normal and lichen planus affected skin. We describe a case of LPP in a 54-year-old Chinese woman. The patient presented with psoriasiform plaques and was diagnosed with guttate psoriasis. Narrowband ultraviolet B (NBUVB) therapy was commenced, and she experienced a generalised erupti...

Dermatitis herpetiformis is an autoimmune bullous disease that is associated with gluten sensitivity which typically presents as celiac disease. As both conditions are multifactorial disorders, it is not clear how specific pathogenetic mechanisms may lead to the dysregulation of immune responses in the skin and small bowel, respectively. Recent studies have demonstrated that IgA and antibodies ...