The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin and/or oral mucosa in combination with circulating and deposited autoantibodies reactive with (hemi)desmosomes. Koch's postulates, adapted for autoimmune diseases, were applied on these skin diseases. It appears that all adapted Koch's pos...

Linear IgA dermatosis or chronic bullous disease of childhood (CBDC) is a nonhereditary, autoimmune subepidermal bullous disease, characterized by the presence of continuous linear deposits of IgA autoantibodies along the basement membrane zone. Antigens mainly involved in the pathogenesis of CBDC are a 97-kDa and a 120 –kDa protein, which represent fragments of the extracellular domain of coll...

Since the mid-1970s, membrane modules became available and plasma separation techniques have gained importance. Therapeutic apheresis (TA) has been successfully introduced in a variety of autoantibody-mediated diseases. In dermatology, TA is increasingly applied as a support treatment for severe and/or refractory autoimmune bullous diseases. All autoimmune disease are characterized by autoantib...

Pemphigus vulgaris (PV) is a potentially fatal autoimmune mucocutaneous blistering disease. Although PV occurs predominantly in adulthood, in the 3rd to 5th decades of life, there have rarely been reports of childhood cases which are often misdiagnosed. It presents as oral blisters that rupture rapidly and progress to painful erosions. Most patients develop cutaneous flaccid blisters that ruptu...

Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or generalized bullous lesions. In up to 20% of affected patients, bullae may be completely absent, and only excoriations, prurigo-like lesions, eczematous lesions, urticated lesions and/or infiltrated ...

Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease and it primarily affects elderly population. It typically presents with tense bullae severe pruritus. Non-bullous a subtype of BP characterized by lacking typical formation different presentations including erythematous, eczematous, urticarial, polycyclic, targetoid, nodular, lichenoid, vesicular erythrodermic. Ai...

At present, there is no consensus about the clinical criteria that define disease severity of pemphigus. During recent years several scoring systems have been introduced which mainly compare inter-individual differences in disease activity. It thus remains a challenge to reflect the disease activity of individual patients by a standardised scoring system. Due to the remarkable clinical variabil...

IMPORTANCE Quality-of-life (QOL) evaluation is an increasingly important outcome measure in dermatology, with disease-specific QOL instruments being the most sensitive to changes in disease status. OBJECTIVE To develop a QOL instrument specific to autoimmune bullous disease (AIBD). DESIGN A comprehensive item generation process was used to build a 45-item pilot Autoimmune Bullous Disease Qu...

Bullous pemphigoid is the most common autoimmune blistering disease in industrialized countries and particularly affects elderly. In this patient population, comorbid diseases are frequent may complicate management treatment of bullous pemphigoid. A better understanding why distinct more patients lead to new pathophysiological insights - as a consequence result care. The association with neurol...