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Autoimmune Bullous Diseases: Pemphigus and Pemphigoid

Spectrum of autoimmune blistering disorders mediated by autoantibodies against epidermal adhesion molecules (desmogleins in pemphigus, hemidesmosomal BP180/BP230 in pemphigoid), causing intraepidermal or subepidermal blisters with distinctive clinical, immunopathological, and treatment features.

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: Pemphigus and Pemphigoid?

Autoimmune bullous diseases are a heterogeneous group of disorders characterized by autoantibody-mediated loss of cell-cell or cell-matrix adhesion in skin and mucous membranes, producing blisters. Two main groups exist by anatomic level of split: pemphigus (intraepidermal, due to acantholysis from anti-desmoglein autoantibodies) and pemphigoid (subepidermal, due to disruption of basement membrane zone).

Pemphigus subtypes include pemphigus vulgaris (anti-Dsg3 ± Dsg1, mucosal-dominant or mucocutaneous, suprabasal split, flaccid bullae, painful erosions, positive Nikolsky sign), pemphigus foliaceus (anti-Dsg1, superficial subcorneal split, scaly crusted erosions sparing mucosae), paraneoplastic pemphigus (associated with lymphoproliferative malignancy, severe mucositis), IgA pemphigus, and drug-induced pemphigus (penicillamine, captopril).

Pemphigoid subtypes include bullous pemphigoid (most common; elderly, anti-BP180/BP230, tense bullae on erythematous/urticarial base, intense pruritus), mucous membrane pemphigoid (predominant mucosal involvement with scarring, especially conjunctival), pemphigoid gestationis (pregnancy-associated), and linear IgA disease. Diagnosis: histopathology, direct immunofluorescence (intercellular IgG/C3 in pemphigus, linear IgG/C3 along BMZ in pemphigoid), indirect IF on monkey esophagus, ELISA for specific autoantibodies. Treatment: rituximab + tapering corticosteroids (first-line for moderate-severe pemphigus vulgaris per international guidelines), high-potency topical/systemic corticosteroids, doxycycline + nicotinamide (mild bullous pemphigoid), azathioprine, mycophenolate mofetil, dapsone, IVIG, and emerging therapies.

Symptoms

Pemphigus vulgaris: oral erosions (often initial), flaccid skin bullae rupturing into painful erosions, positive Nikolsky sign
Pemphigus foliaceus: superficial scaly crusted erosions on seborrheic areas (face, scalp, upper trunk), no mucosal involvement
Bullous pemphigoid: intense pruritus, urticarial plaques, tense bullae on erythematous base in elderly
Mucous membrane pemphigoid: oral erosions, scarring conjunctivitis (symblepharon), genital scarring
Pemphigoid gestationis: pregnancy-associated pruritic urticarial plaques and bullae
Paraneoplastic pemphigus: severe stomatitis with associated malignancy

Risk Factors

Age >60 years (bullous pemphigoid)
Middle age (pemphigus vulgaris, 40-60)
HLA associations (HLA-DR4, DR14 in pemphigus; DQB1*0301 in BP)
Mediterranean, Jewish, Indian descent (pemphigus vulgaris)
Drugs: penicillamine, captopril (pemphigus); furosemide, gliptins (BP)
Lymphoproliferative malignancy (paraneoplastic pemphigus)

When to See a Doctor?

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

  • Persistent painful oral erosions or blisters
  • New onset blisters in elderly with intense pruritus
  • Flaccid bullae easily rupturing on minor trauma
  • Painful skin erosions with sloughing
  • Pregnancy-associated blistering rash
  • Suspected drug-induced blistering disease

Treatment Methods

01
Skin biopsy from blister edge for histopathology + perilesional skin for direct immunofluorescence
02
Serum: indirect immunofluorescence on monkey esophagus, ELISA (anti-Dsg1/Dsg3, anti-BP180 NC16A, anti-BP230)
03
Pemphigus vulgaris first-line (per 2020 international consensus): rituximab + tapering oral corticosteroids
04
Pemphigus alternative: prednisone 1-1.5 mg/kg/day + steroid-sparing (azathioprine, mycophenolate mofetil)
05
Bullous pemphigoid mild-moderate: high-potency topical clobetasol (whole-body) is first-line per Cochrane evidence
06
Bullous pemphigoid moderate-severe: oral prednisone 0.5 mg/kg/day, doxycycline + nicotinamide for mild cases
07
Steroid-sparing for BP: methotrexate, azathioprine, mycophenolate mofetil
08
Refractory: rituximab, IVIG, omalizumab, dupilumab
09
Wound care, infection prevention, nutritional support, ophthalmology consultation for mucous membrane pemphigoid
10
Malignancy workup for paraneoplastic pemphigus

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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.