Monthly Archives: May 2012

Bullous pemphigoid (BP) represents the most common autoimmune subepidermal blistering disease. BP typically affects the elderly and is associated with significant morbidity. It has usually a chronic course with spontaneous exacerbations. The cutaneous manifestations of BP can be extremely protean. While diagnosis of BP in the bullous stage is straightforward, in the non-bullous stage or in atypical variants of BP signs and symptoms are frequently non-specific with eg, only itchy excoriated, eczematous, papular and/or urticarial lesions that may persist for several weeks or months. Diagnosis of BP critically relies on immunopathologic examinations including direct immunofluorescence microscopy and detection of serum autoantibodies by indirect immunofluorescence microscopy or BP180-ELISA. (S…

from MedWorm Query: Pemphigoid http://www.medworm.com/index.php?rid=6018239&cid=u_0_3_f&fid=33229&url=http%3A%2F%2Fwww.immunology.theclinics.com%2Farticle%2FPIIS0889856112000173%2Fabstract%3Frss%3Dyes

ABSTRACT The study describes the case of a patient with a clinical and histopathological diagnosis of pemphigus vulgaris accompanied by severe side‐effects of combined immunosuppressive therapy, who achieved a remission of the disease with adalimumab. Pemphigus vulgaris is a chronic blistering disease of the skin and mucous membranes. Before corticosteroids were introduced, mortality was high. Corticosteroids are currently used as first‐line therapy. To reduce the dose of corticosteroids, therapeutic combinations with corticosteroid‐sparing immunosuppressive agents are used. The therapy brings a number of complications due to its side effects. To achieve a remission of the disease by treating our patient with combined immunosuppressives, we administered adalimumab and achieved a very …

from MedWorm: Pemphigus http://www.medworm.com/index.

  This article reviews the use of MMF for the treatment of several bullous conditions, and assesses the evidence gathered from clinical trials and case series. According to numerous case series, MMF could be of value in treating refractory disease. The few randomized clinical trials conducted to date of patients with pemphigus and bullous pemphigoid report a similar efficacy for MMF to other immunosuppressants. (Source: Immunology and Allergy Clinics of North America)

from MedWorm: Pemphigus http://www.medworm.com/index.php?rid=6018247&cid=c_297_3_f&fid=33229&url=http%3A%2F%2Fwww.immunology.theclinics.com%2Farticle%2FPIIS0889856112000252%2Fabstract%3Frss%3Dyes

Bullous pemphigoid, the most common autoimmune blistering disease, is induced by autoantibodies against type XVII collagen. Passive transfer of IgG or IgE antibodies against type XVII collagen into animals has revealed not only the pathogenicity of these antibodies but also the subsequent immune responses, including complement activation, mast cell degranulation, and infiltration of neutrophils and/or eosinophils. In vitro studies on ectodomain shedding of type XVII collagen have also provided basic knowledge on the development of bullous pemphigoid. The pathogenic role of autoreactive CD4+ T lymphocytes in the development of the pathogenic autoantibodies to type XVII collagen should also be noted. (Source: Immunology and Allergy Clinics of North America)
from MedWorm Query: Pemphigoid http://www.medworm.com/index.php?rid=6018238&cid=u_0_3_f&fid=33229&url=http%3A%2F%2Fwww.immunology.theclinics.com%2Farticle%2FPIIS0889856112000161%2Fabstract%3Frss%3Dyes
  Autoimmune bullous diseases are associated with autoimmunity against structural components that maintain cell-cell and cell-matrix adhesion in the skin and mucous membranes. They include those where the skin blisters at the basement membrane zone and those where the skin blisters within the epidermis (pemphigus vulgaris, pemphigus foliaceus, and other subtypes of pemphigus). The variants of pemphigus are determined according to the level of intraepidermal split formation. There are 5 main variants of pemphigus: pemphigus vulgaris, pemphigus foliaceus, pemphigus erythematosus, drug-induced pemphigus, and paraneoplastic pemphigus. This review focuses only on pemphigus vulgaris. (Source: Immunology and Allergy Clinics of North America)

from MedWorm: Pemphigus http://www.medworm.com/index.php?rid=6018240&cid=c_297_3_f&fid=33229&url=http%3A%2F%2Fwww.immunology.theclinics.com%2Farticle%2FPIIS0889856112000185%2Fabstract%3Frss%3Dyes

Although there are no standard guidelines for the treatment of autoimmune blistering diseases, azathioprine has shown good efficacy in acquired autoimmune blistering diseases, and is well tolerated. Side effects of azathioprine normally occur in mild variants. Severe reactions are due to reduced thiopurine S-methyltransferase (TPMT) or inosine triphosphate pyrophosphohydrolase (ITPA) activity. Therefore, screening for TPMT activity should be conducted in white patients and Africans, whereas Japanese should be screened for ITPA activity before therapy with azathioprine is started. Azathioprine is clinically meaningful for the treatment of pemphigus. (Source: Immunology and Allergy Clinics of North America)

http://www.immunology.theclinics.com/article/PIIS0889856112000240/abstract?rss=yes