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</a><h1 class="with-also" itemprop="name">Pemphigus vulgaris</h1>
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</noscript></div><div class="main"><div id="ency_summary"><p>Pemphigus vulgaris (PV) is an <a test="test" href="./000816.htm">autoimmune disorder</a> of the skin. It involves blistering and sores (erosions) of the skin and <a test="test" href="./002264.htm">mucous membranes</a>.</p></div><section><div class="section"><div class="section-header"><div class="section-title"><h2>Causes</h2></div><div class="section-button"><button type="submit" aria-controls="section-1" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-1"><p>The immune system produces <a test="test" href="./002223.htm">antibodies</a> against specific proteins in the skin and mucous membranes. These antibodies break the bonds between skin cells. This leads to the formation of a blister. The exact cause is unknown.</p><p>In rare cases, PV is caused by some medicines, including:</p><ul><li>A medicine called penicillamine, which removes certain materials from the blood (chelating agent)</li><li>Blood pressure medicines called <a test="test" href="../patientinstructions/000087.htm">ACE inhibitors</a></li><li>Nonsteroidal anti-inflammatory drugs (NSAIDs)</li></ul><p>PV is uncommon. It most often occurs in middle-aged or older people.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Symptoms</h2></div><div class="section-button"><button type="submit" aria-controls="section-2" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-2"><p>About 50% of people with this condition first develop painful blisters and sores in the mouth. This is followed by skin blisters. Skin sores may come and go.</p><p>The skin sores may be described as:</p><ul><li>Draining</li><li>Oozing</li><li>Crusting</li><li>Peeling or easily detached</li></ul><p>They may be located:</p><ul><li>In the mouth and down the throat</li><li>On the scalp, trunk, or other skin areas</li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Exams and Tests</h2></div><div class="section-button"><button type="submit" aria-controls="section-3" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-3"><p>The skin separates easily when the surface of unaffected skin is rubbed sideways with a cotton swab or finger. This is called a positive <a test="test" href="./003285.htm">Nikolsky sign</a>.</p><p>A <a test="test" href="./003840.htm">skin biopsy</a> and blood tests are often done to confirm the diagnosis.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Treatment</h2></div><div class="section-button"><button type="submit" aria-controls="section-4" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-4"><p>Severe cases of PV may need wound management, similar to the treatment for severe <a test="test" href="./000030.htm">burns</a>. People with PV may need to stay in a hospital and receive care in a burn unit or intensive care unit.</p><p>Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections.</p><p>Treatment may involve:</p><ul><li>Antibiotics and antifungal medicines to control or prevent infections</li><li>Fluids and <a test="test" href="./002350.htm">electrolytes</a> given through a vein (<a test="test" href="./002383.htm">IV</a>) if there are severe mouth ulcers</li><li>IV feedings if there are severe mouth ulcers</li><li>Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain</li><li>Pain medicines if local pain relief is not enough </li></ul><p>Body-wide (systemic) therapy is needed to control PV and should be started as early as possible. Systemic treatment includes:</p><ul><li>An anti-inflammatory medicine called dapsone</li><li>Corticosteroids</li><li>Medicines containing gold</li><li>Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab) </li></ul><p>Antibiotics may be used to treat or prevent infection. Intravenous immunoglobulin (IVIg) is occasionally used.</p><p>Plasmapheresis may be used along with systemic medicines to reduce the amount of antibodies in the blood. Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma.</p><p>Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Outlook (Prognosis)</h2></div><div class="section-button"><button type="submit" aria-controls="section-5" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-5"><p>Without treatment, this condition can be life threatening. Severe infection is the most frequent cause of death.</p><p>With treatment, the disorder tends to be <a test="test" href="./002312.htm">chronic</a>. Side effects of treatment may be severe or disabling.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Possible Complications</h2></div><div class="section-button"><button type="submit" aria-controls="section-6" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-6"><p>Complications of PV include:</p><ul><li>Secondary skin infections</li><li>Severe <a test="test" href="./000982.htm">dehydration</a></li><li>Side effects of medicines</li><li>Spread of infection through the bloodstream (<a test="test" href="./000666.htm">sepsis</a>)</li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>When to Contact a Medical Professional</h2></div><div class="section-button"><button type="submit" aria-controls="section-7" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-7"><p>Your health care provider should examine any unexplained blisters.</p><p>Contact your provider if you have been treated for PV and you develop any of the following symptoms:</p><ul><li><a test="test" href="./003091.htm">Chills</a></li><li>Fever</li><li>
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General ill feeling
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</li><li>
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Joint aches
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</li><li>
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Muscle aches
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</li><li>New blisters or ulcers</li></ul></div></div></section><section><div class="section sec-mb"><div class="section-header"><div class="section-title"><h2>Images</h2></div><div class="section-button"><button type="submit" aria-controls="section-tnails" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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<li><img src="//medlineplus.gov/ency/images/ency/tnails/2459t.jpg" alt="Pemphigus vulgaris on the back" title="Pemphigus vulgaris on the back" class="side-img"/><a href="../imagepages/2459.htm">Pemphigus vulgaris on the back</a></li>
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<li><img src="//medlineplus.gov/ency/images/ency/tnails/2502t.jpg" alt="Pemphigus vulgaris - lesions in the mouth" title="Pemphigus vulgaris - lesions in the mouth" class="side-img"/><a href="../imagepages/2502.htm">Pemphigus vulgaris - lesions in the mouth</a></li>
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</ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>References</h2></div><div class="section-button"><button type="submit" aria-controls="section-Ref" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Ref"><p>Amagai M. Pemphigus. In: Bolognia JL, Schaffer JV, Cerroni L, eds. <em>Dermatology.</em> 5th ed. Philadelphia, PA: Elsevier; 2025:chap 29.</p><p>Dinulos JGH. Vesicular and bullous diseases. In: Dinulos JGH, ed. <em>Habif's Clinical Dermatology. </em>7th ed. Philadelphia, PA: Elsevier; 2021:chap 16.</p><p>James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM. Chronic blistering dermatoses. In: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. <em>Andrew's Diseases of the Skin.</em> 13th ed. Philadelphia, PA: Elsevier; 2020:chap 21.</p><p>Patterson JW. The vesiculobullous reaction pattern. In: Patterson JW, ed. <em>Weedon's Skin Pathology.</em> 5th ed. Philadelphia, PA: Elsevier; 2021:chap 7.</p></div></div></section>
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