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Section 3<br />

Dermatology<br />

Bullous and Blistering Diseases<br />

Pemphigus Vulgaris<br />

This is an autoimmune disease of unclear etiology in which <strong>the</strong> body becomes,<br />

essentially, allergic to its own skin. Antibodies are produced against antigens<br />

in <strong>the</strong> intercellular spaces of <strong>the</strong> epidermal cells. Its causes are as follows:<br />

··<br />

Idiopathic<br />

··<br />

ACE inhibitors<br />

··<br />

Penicillamine<br />

Pemphigus vulgaris acts like a burn, because <strong>the</strong> bullae occur from destruction<br />

within <strong>the</strong> epidermis and so are relatively thin and fragile.<br />

Nikolsky’s sign is present: Nikolsky’s sign is <strong>the</strong> easy removal of skin by just a<br />

little pressure, with <strong>the</strong> examiner’s finger pulling it off like a sheet. Note that<br />

Nikolsky’s sign is present in pemphigus vulgaris, staphylococcal scalded skin<br />

syndrome, and toxic epidermal necrolysis.<br />

The lesions of pemphigus vulgaris are painful, not pruritic.<br />

Diagnostic Testing<br />

The most accurate diagnostic test is a biopsy of <strong>the</strong> skin.<br />

Treatment<br />

Treatment is as follows:<br />

··<br />

Use glucocorticoids, such as prednisone.<br />

··<br />

When steroids are ineffective, use <strong>the</strong> following:<br />

--<br />

Azathioprine<br />

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