Pemphigus Vegetans

Aka: Pemphigus Vegetans, Familial Benign Pemphigus, Hailey-Hailey Disease

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II. Epidemiology

  1. Autosomal Dominant inherited condition

III. Pathophysiology

  1. Benign variant of Pemphigus Vulgaris
  2. Suprabasal cell Acantholysis
  3. Exacerbating factors
    1. Superficial Bacterial Infection
    2. Warm weather

IV. 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

V. Labs

  1. Immunofluorescence shows no antibodies on biopsy

VI. 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

VII. Course

  1. Much better prognosis than Pemphigus Vulgaris

VIII. References

  1. Parker in Goldman (2000) Cecil Medicine, p. 2284
  2. Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]

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