Description, Causes and Risk Factors:
Bullous pemphigoid is a chronic, autoimmune, subepidermal, blistering disease of the skin. It rarely involves mucous membranes. Bullous pemphigoid is characterized by the presence of (1) immunoglobulin IgG and complement deposits in the skin of patients and (2) of the presence in the serum of immunoglobulin G (IgG) autoantibodies specific for the basement membrane zone antigens such as to BP230 ( BPAg1) and BP180 (BPAg2).
While the cause is unknown, it is felt by some that an aging immune system may become activated in certain individuals with some genetic predisposition to develop bullous pemphigoid. Bullous pemphigoid can be chronic, mild and not affect the general health or it can be severe and compromise the health of the sufferer. Clinically bullous pemphigoid may present with several distinct clinical presentations such as generalized bullous, vesicular, vegetative, generalized erythroderma, urticarial, and nodular. It can affect any part of the skin surface, with a predilection on the flexural areas of the skin. Oral and ocular mucosa involvement rarely occurs and, when seen, is of minor clinical significance.
Some patients with bullous pemphigoid have other autoimmune diseases such diabetes and rheumatoid arthritis. Various other factors have been reported to play a role in triggering bullous pemphigoid. These include drugs, mechanical trauma, and physical traumas (burns from radiation, sun or heat).
Its frequency is unknown. No racial predilection is apparent. Bullous pemphigoid primarily affects elderly individuals in the fifth through seventh decades of life, with an average age at onset of 65 years. A few cases of bullous pemphigoid of childhood onset have been reported in the literature.
Symptoms of bullous pemphigoid include intense itching andburning sensation of the skin. When the mucous membranes of themouth are affected, it can cause pain, burning, and sensitivity toacidic foods. Eating can be difficult, and involvement in the deeperareas of the throat can cause coughing. Involvement of the innernose can cause nosebleeds.
Bullous pemphigoid diagnosis is by biopsy and serumanalysis of the presence of autoantibodiesto the basement membrane zone (BMZ).Biopsy studies include H&E (hematoxylin and eosin (stain))and directimmunofluorescence (IF). Direct IF studies show the presence of immunoglobulinsand complement in the BMZ with a linearpattern. Serum studies detect the presenceof autoantibodies to BMZ antigens byindirect IF using primate esophagusand/or split skin. For H&E biopsy shouldbe taken from the edge of a blister and fordirect IF studies biopsy should be fromnormal-appearing perilesional skin.
The H&E examination demonstratessubepidermal blister. The inflammatoryinfiltrate is typically polymorphous, withan eosinophil predominance. Direct IFstudies demonstrate in vivo deposits ofIgG (70-90% of patients) and complement C3 deposition (90-100% of patients)in a linear band at the BMZ. This patternof immunoreactants is also observed inother sub-epidermal bullous diseases suchas mucous membrane pemphigoid(MMP) and epidermolysis bullosa acquisita (EBA).
In summary, a combination ofclinical, histological, and IF studies are themost reliable in diagnosis of bullous pemphigoid
Topical steroids are effective however their use in extensive disease may be limited by side effects and practical factors. The effectiveness of the addition of plasma exchange or azathioprine to corticosteroids has not been fully established. Combination treatment with tetracycline and nicotinamide may be useful.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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