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

Afebrile

Evaluation

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

Management

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

External Links

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.