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Pemphigus

A serious autoimmune skin disease presenting with painful spontaneously occurring blisters on the skin and mucous membranes.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Pemphigus?

Pemphigus is a serious autoimmune disease in which autoantibodies against cell-cell junction proteins called desmoglein cause cell separation (acantholysis) within the epidermis, producing large, loose fluid-filled blisters.

The most common types are pemphigus vulgaris (deep epidermal and mucosal involvement) and pemphigus foliaceus (superficial epidermal, no mucosal involvement). In pemphigus vulgaris, oral mucosa is frequently and early involved.

Because the blisters are very loose, they easily rupture and leave extensive painful erosion areas. If untreated, serious infection and fluid loss can be life-threatening. Most patients achieve remission with immunosuppressive treatment.

Symptoms

Painful ulcers in the mouth, throat, or genital mucosa (often the first symptom)
Easily ruptured, loose large blisters on the skin
Extensive painful erosion areas when blisters burst
Erosion formation when pressure is applied to normal-appearing skin (Nikolsky sign +)
Difficulty swallowing (esophageal involvement)
Itching and burning sensation
Weight loss and weakness (due to feeding difficulty)

Risk Factors

Middle age and above (40-60 years common)
Individuals of Jewish and Mediterranean origin (genetic predisposition)
History of autoimmune disease
Some drugs like D-penicillamine and captopril (drug-induced pemphigus)
Presence of thymoma (paraneoplastic pemphigus)
Family history
HLA-DR4 and DR14 genetic markers

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • If there are long-standing non-healing ulcers in the mouth
  • When spontaneously occurring large blisters are noticed on the skin
  • If blisters burst and leave extensive erosions
  • If there is difficulty swallowing or severe pain
  • When fever and general condition deterioration are accompanied (EMERGENCY)

Treatment Methods

01
High-dose systemic corticosteroids (control of acute phase)
02
Rituximab (anti-CD20 biologic agent — current first-line)
03
Azathioprine, mycophenolate mofetil (steroid-sparing drugs)
04
IVIG and plasmapheresis (in resistant cases)
05
Wound care and infection control
06
Oral care and pain management (in mucosal involvement)

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.