Pemphigus vulgaris
An autoimmune blistering disorder
| Pemphigus vulgaris | |
|---|---|
| |
| Synonyms | PV |
| Pronounce | /ˈpɛmfɪɡəs vʌlˈɡɛərɪs/ |
| Field | Dermatology, Immunology |
| Symptoms | Painful blisters on the skin and mucous membranes, especially in the mouth |
| Complications | Secondary infections, dehydration, sepsis |
| Onset | Typically middle-aged or older adults |
| Duration | Chronic, potentially life-threatening if untreated |
| Types | Mucosal-dominant, mucocutaneous |
| Causes | Autoimmune response against desmoglein proteins (DSG1 and DSG3) |
| Risks | Genetic predisposition, certain medications, other autoimmune disorders |
| Diagnosis | Skin biopsy, direct immunofluorescence, ELISA for desmoglein antibodies |
| Differential diagnosis | Bullous pemphigoid, mucous membrane pemphigoid, lichen planus, Stevens–Johnson syndrome |
| Prevention | No known prevention; avoid triggering medications |
| Treatment | Immunosuppressive therapy, corticosteroids, biologic therapy (e.g., rituximab) |
| Medication | Prednisone, azathioprine, mycophenolate mofetil, rituximab |
| Prognosis | Improved with treatment; risk of relapse or complications persists |
| Frequency | Rare (1–5 cases per million per year) |
| Deaths | Reduced with modern therapies; previously high mortality without treatment |
Pemphigus vulgaris is a rare, chronic autoimmune disease characterized by the formation of blisters and erosions on the skin and mucous membranes. It is the most common form of pemphigus, a group of autoimmune blistering disorders.
Pathophysiology[edit | edit source]
Pemphigus vulgaris is caused by the production of autoantibodies against desmoglein, a protein that is critical for the adhesion of keratinocytes in the epidermis. Desmoglein is a component of desmosomes, which are structures that hold cells together. The autoantibodies disrupt the function of desmoglein, leading to the loss of cell adhesion, a process known as acantholysis. This results in the formation of intraepidermal blisters.
Clinical Presentation[edit | edit source]
Patients with pemphigus vulgaris typically present with painful blisters and erosions on the skin and mucous membranes. The oral cavity is often the first site of involvement, with painful erosions that can make eating and drinking difficult. Skin lesions may appear as flaccid blisters that easily rupture, leaving raw, painful areas.
Diagnosis[edit | edit source]
The diagnosis of pemphigus vulgaris is based on clinical examination, histopathological findings, and immunofluorescence studies. A skin biopsy from the edge of a blister can show acantholysis and intraepidermal blister formation. Direct immunofluorescence of perilesional skin typically reveals IgG and C3 deposits in the intercellular spaces of the epidermis. Indirect immunofluorescence can detect circulating autoantibodies in the patient's serum.
Treatment[edit | edit source]
The mainstay of treatment for pemphigus vulgaris is systemic corticosteroids, which help to reduce inflammation and autoantibody production. Immunosuppressive agents such as azathioprine, mycophenolate mofetil, and rituximab are often used as steroid-sparing agents. Plasmapheresis and intravenous immunoglobulin (IVIG) may be considered in severe cases.
Prognosis[edit | edit source]
With appropriate treatment, the prognosis of pemphigus vulgaris has improved significantly. However, it remains a potentially life-threatening condition due to the risk of infection and complications from long-term immunosuppressive therapy. Early diagnosis and aggressive treatment are crucial to improving outcomes.
Related pages[edit | edit source]
External links[edit | edit source]
| Classification | |
|---|---|
| External resources |
|
Search WikiMD
Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available.
Advertise on WikiMD
|
WikiMD's Wellness Encyclopedia |
| Let Food Be Thy Medicine Medicine Thy Food - Hippocrates |
Translate this page: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
தமிழ்,
తెలుగు,
Urdu,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
বাংলা
European
español,
Deutsch,
français,
Greek,
português do Brasil,
polski,
română,
русский,
Nederlands,
norsk,
svenska,
suomi,
Italian
Middle Eastern & African
عربى,
Turkish,
Persian,
Hebrew,
Afrikaans,
isiZulu,
Kiswahili,
Other
Bulgarian,
Hungarian,
Czech,
Swedish,
മലയാളം,
मराठी,
ਪੰਜਾਬੀ,
ગુજરાતી,
Portuguese,
Ukrainian
Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates, categories Wikipedia, licensed under CC BY SA or similar.
Contributors: Kondreddy Naveen, Prab R. Tumpati, MD
