Dermatology Book

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Pemphigus VegetansAka: Familial Benign Pemphigus, Hailey-Hailey Disease

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  1. Epidemiology
    1. Autosomal dominant inherited condition
  2. Pathophysiology
    1. Benign variant of Pemphigus Vulgaris
    2. Suprabasal cell Acantholysis
    3. Exacerbating factors
      1. Superficial bacterial infection
      2. Warm weather
  3. Signs
    1. Mouth mucosal lesions absent
    2. Large confluent vegetative Skin Erosions
      1. Center with moist granular tissue
      2. Border composed of Vesicles and Pustules
    3. Distribution
      1. Form in intertriginous areas (groin, axillae)
      2. Also affects neck folds and scalp or face
  4. Labs
    1. Immunofluorescence shows no antibodies on biopsy
  5. Management
    1. Treat local infections
      1. Consider topical and systemic antibiotics
      2. Consider Antifungal agents for candida infection
    2. Surgical excision of large vegetative growths
  6. Course
    1. Much better prognosis than Pemphigus Vulgaris
  7. References
    1. Parker in Goldman (2000) Cecil Medicine, p. 2284
    2. Cotell (2000) Am J Emerg Med 18(3):288

Pemphigus vegetans (C0263316)

ConceptsDisease or Syndrome (T047)
ICD9694.4
EnglishPemphigus vegetans
Spanishpénfigo vegetante, penfigo vegetante
CreditsDerived from the NIH UMLS (Unified Medical Language System)



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