Pemphigus

Pemphigus 

Updated: 02/09/2021

© Jun Wang, MD, PhD

General features
  • Rare, nonhereditary, chronic, autoimmune disease
  • Ages 30-59; no gender preference
  • May be associated with internal malignancies, including thymoma
  • Intraepidermal blisters with acantholysis
  • Benign, but may be fatal if untreated in extreme cases
Pathogenesis
  • Autoantibody against desmoglein 1 and 3 (desmosome components)
Pemphigus vulgaris
  • Most common type
  • May involve both skin and oral mucosa
  • Superficial vesicles, easily ruptured with painful erosion
  • Healing usually accompanied by post-inflammatory hyperpigmentation
  • Deaths may occur due to staphylococcal infection, or pulmonary embolism
  • Suprabasilar bullae with single row of basal cells resides on basement membrane
Pemphigus foliaceus
Pemphigus erythematosus
  • Senear–Usher syndrome
  • Usually middle age adults
  • Localized area, resemble lupus malar rash on face
  • May be considered as a localized less severe form of pemphigus foliaceus
Pemphigus vegetans
  • Rare
  • Considered a localized form of pemphigus vulgaris
  • Verrucous plaques in affected regions
  • Oral cavity commonly affected
  • Subtle supra basal acantholysis with epidermal hyperplasia, intraepidermal eosinophilic and neutrophilic microabscesses
Pemphigus paraneoplastic
  • Associated with internal tumor
  • Involving both oral mucosa and skin
Pathological features
  • Intraepidermal vesicles
  • Acantholysis
  • May have eosinophilic infiltrate
Immunofluorescence feature
Management
  • Corticosteroid
  • Immunosuppressant
  • Wound care, etc


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Contents

Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4.  Benign blood cell disorders Anemia Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas M
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Anemia

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Lymphoid neoplasms

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