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The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

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The authors present the in vivo investigation options in drug allergy. In suspicion of drug induced hypersensitivity reaction prick testing, intradermal testing and patch testing are recommended according to the assumed immuno-mechanism. If these examinations are negative, the next step is the drug provocation test, which is the gold standard in the diagnosis of drug allergy. We summarize methods, indications and contraindications and the evaluationof each test, focusing on issues concerning antibiotics, perioperative medication, local anesthetics and biological agents. There are increasing number of patients presenting hypersensitivity reactions who require proper identification of the culprit drug.

Mucous membrane pemphigoid (MMP) is an autoimmune subepithelial blistering disease. Oral and ocular mucosae are the most frequently afected areas, but the nasopharynx, esophagus, larynx and anogenital region can also be involved. It is characterized by linear deposition of IgG, IgA, or C3 along the basement membrane zone. In low-risk cases topical, intralesional corticosteroids are administered, together with anti-infammatory, immunomodulatory (dapsone) drugs or antibiotics (doxycycline). In severe, high-risk cases we apply systemic corticosteroids and immunosuppresive agents, or biological therapies (rituximab and anti-tumour necrosis factor drugs), or combination treatment of rituximab and intravenous immunoglobulins (IVIG)