Pemphigus vulgaris: Difference between revisions
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*Admit if evidence of: | *Admit if evidence of: | ||
**Significant fluid or electrolyte loss | **Significant fluid or electrolyte loss | ||
**Difficulty tolerating PO secondary to mucosal blisters | |||
**Decompensated disease | **Decompensated disease | ||
**Burn unit for large BSA involvement | **Burn unit for large BSA involvement | ||
Revision as of 03:22, 24 January 2016
Background
- Chronic autoimmune mucocutaneous disease against desmosomes in epidermis (bind keratinocytes)
Clinical Features
- 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
Bullous Rashes
- Bullous pemphigoid
- Cicatricial pemphigoid
- Dermatitis herpetiformis
- Drug-Induced Bullous Disorders
- Erythema multiforme
- Pemphigus Vulgaris
Diagnosis
- 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
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.