Pemphigus vulgaris

Background

• Chronic autoimmune mucocutaneous disease against desmosomes in epidermis (bind keratinocytes)
• Mortality 5-15% sec to complications of long-term steroid therapy.

Clinical Features

Pemphigus vulgaris

• Painful but rarely pruritic
• Mucosal involvement common
  • Presenting complaint in 50% of cases
• Primary lesions
  • Tense and clear vesicles/bullae on head, trunk, mucosa
  • Become flaccid and turbid 2-3 days later
  • Rupture and leave sensistive denuded area of skin
    • Slow to heal and prone to secondary infection
• Nikolsky's sign: Sliding pressure applied to normal skin adjacent to blister causes further ulceration
  • Intraepidermal acantholyis: Keratinocytes separated at the basal layer from one another
  • Gives appearance of lesion expanding into adjacent tissue
  • Transudate accumulate between keratinocyte and basement membrane which gives rise to new blisters

Differential Diagnosis

Vesiculobullous rashes

Febrile

• Diffuse distribution
  • Varicella
  • Smallpox
  • Disseminated gonococcal disease
  • DIC
  • Purpural fulminans
• Localized distribution
  • Necrotizing fasciitis
  • Hand-foot-and-mouth disease

Afebrile

• Diffuse distribution
  • Bullous pemphigoid
  • Drug-Induced bullous disorders
  • Pemphigus vulgaris
  • Phytophotodermatitis
  • Erythema multiforme major
• Localized distribution
  • Contact dermatitis
  • Herpes zoster
  • Dyshidrotic eczema
  • Burn
  • Dermatitis herpetiformis
  • Erythema multiforme minor
  • Poison Oak, Ivy, Sumac dermatitis
  • Bullosis diabeticorum

Evaluation

• Clinical diagnosis. Nikolsky's sign may be helpful to differentiate from other bullous diseases.
  • Gold standard: punch biopsy

Management

• IVF and electrolyte resuscitation
• Systemic Corticosteroids
• IV Antibiotics for signs of secondary infection.
• Consider need for Rheumatology evaluation
  • Plasmapharesis and IVIG in severe cases.

Disposition

• Admit if evidence of:
  • Significant fluid or electrolyte loss
  • Difficulty tolerating PO secondary to mucosal blisters
  • Decompensated disease
  • Burn unit for large BSA involvement

See Also

• Bullous rashes

References

• Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
• Thomas J, Perron AD, Brady WJ. Serious Generalized Skin Disorders. In: Tintinalli JE, Stapczynski J, Ma O, Cline DM, Cydulka RK, Meckler GD, T. eds. Tintinalli's Emergency Medicine: A Comprehensive Study Guide. New York, NY: McGraw-Hill; 2011.
• Pitzele HZ, Kessler CS. Life-Threatening Dermatoses. In: Sherman SC, Weber JM, Schindlbeck MA, Rahul G. P. eds. Clinical Emergency Medicine, 1e. New York, NY: McGraw-Hill; 2014.
• Ahmed AR, Moy R. Death in pemphigus. J Am Acad Dermatol. 1982 Aug. 7(2):221-8.

Authors

Amr Badawy, Ross Donaldson, Kevin Lu, Daniel Ostermayer, Claire, Neil Young