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Autoimmune Bullous Diseases

A group of autoimmune diseases, including pemphigus and pemphigoid, presenting with blister formation in the skin and mucosa.

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 Autoimmune Bullous Diseases?

Autoimmune bullous diseases are a group of diseases in which autoantibodies develop against keratinocyte-keratinocyte (desmoglein, desmocollin) or keratinocyte-basement membrane (BP180, BP230, Type VII collagen) connections in the skin and mucosa, leading to blister formation in these regions.

This group includes pemphigus vulgaris and foliaceus (intraepidermal blisters), bullous pemphigoid (subepidermal blisters), mucous membrane pemphigoid, dermatitis herpetiformis (celiac-related), and epidermolysis bullosa acquisita.

These diseases are common in older age. Diagnosis is made with clinical examination, histopathology, direct immunofluorescence, and ELISA tests. Treatment is generally conducted with systemic corticosteroids and immunosuppressants; multidisciplinary follow-up is required.

Symptoms

Easily tearing blisters in skin and mucosa
Painful erosions and ulcers in the mouth
Flaccid blisters in pemphigus vulgaris (positive Nikolsky sign)
Tense blisters and itching in bullous pemphigoid
Difficulty with mouth/swallowing disrupting quality of life
Weight loss, nutritional problems, and risk of infection
Pigmentation and scarring after blistering

Risk Factors

Advanced age (for bullous pemphigoid)
Jewish and Mediterranean ethnic groups (pemphigus vulgaris)
HLA-related genetic predisposition
Some medications (ACE inhibitors, furosemide, diuretics — drug-induced bullous pemphigoid)
Association with celiac disease (dermatitis herpetiformis)
Changes in immune system modulation
Malignancy association (paraneoplastic pemphigus)

When to See a Doctor?

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

  • Easily tearing skin blisters
  • Painful and non-healing sores in the mouth
  • Widespread and progressive blistering rash
  • If fever and systemic symptoms accompany
  • Exacerbation of existing disease and new medication use

Treatment Methods

01
Systemic corticosteroids (foundation of treatment)
02
Immunosuppressants (azathioprine, mycophenolate, methotrexate)
03
Rituximab (used as core therapy in pemphigus)
04
Wound care, infection prophylaxis, oral hygiene
05
Multidisciplinary follow-up (dermatology, ophthalmology, dentistry)
06
Discontinuation of the responsible drug in drug-related forms

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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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.