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Bullous pemphigoid
Classification and external resources
ICD-10 L12.0
ICD-9 694.5
eMedicine derm/64
MeSH D010391

Bullous pemphigoid, also referred to as BP, is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the skin layers epidermis and dermis. The bullae are formed by an immune reaction, initiated by the formation of autoantibodies targeting the type XVII collagen component of hemidesmosomes. It can also rarely involve the mucous membranes. Following antibody targeting, a cascade of immunomodulators results in a variable surge of neutrophils, lymphocytes and eosinophils coming to the affected area. Unclear events subsequently result in a separation along the dermal-epidermal junction and eventually stretch bullae.

Clinically the earliest lesions may appear urticarial (like hives). Tense bullae eventually erupt, most commonly at the inner thighs and upper arms but the trunk and extremities are frequently both involved. Any part of the skin surface can be involved. Milia are more common with epidermolysis bullosa acquisita (EBA), because of the deeper antigenic targets. A more ring-like configuration, with a central depression or centrally collapsed bullae may indicate linear IgA disease. The disease may be acute, but typically will wax and wane.

Diagnosis is based on two biopsies of the skin, one submitted for routine H&E staining and one for immunofluorescence studies.

Treatments include Class I topical steroids (clobetasol, halobetasol, etc.) which in some studies have proven to be equally as effective as systemic, or pill, therapy and somewhat safer. However, in difficult to manage or widespread cases, systemic prednisone and powerful steroid-free immunosuppressant medications such as methotrexate, azathioprine or mycophenolate mofetil may be appropriate.

Many mammals can be afflicted, including dogs, cats, pigs, and horses, as well as humans. Very rarely seen in children, it usually occurs in people 70 years of age and older.

At least one study indicates the incidence might be increasing in the United Kingdom.[1]

See also

References

Guidelines for the management of bullous pemphigoid, British Journal of Dermatology 2002; 147: 214–221. http://bad.org.uk/Portals/_Bad/Guidelines/Clinical%20Guidelines/Bullous%20Pemphigoid.pdf

External links

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