Pemphigus vulgaris: Difference between revisions
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==Background== | ==Background== | ||
{{Skin anatomy background images}} | |||
*Chronic autoimmune mucocutaneous disease against desmosomes in epidermis (bind keratinocytes) | *Chronic autoimmune mucocutaneous disease against desmosomes in epidermis (bind keratinocytes) | ||
*Mortality 5-15% sec to complications of long-term steroid therapy. | *Mortality 5-15% sec to complications of long-term steroid therapy. | ||
==Clinical Features== | ==Clinical Features== | ||
[[File:Pemphgoid vulgaris.jpg|thumb|Pemphigus vulgaris of torso.]] | |||
[[File:PMC5418673 gr1.png|thumb|Painful, shallow oral erosions in a patient with pemphigus vulgaris.]] | |||
[[File:pemphigus.JPG|thumbnail|Pemphigus vulgaris]] | [[File:pemphigus.JPG|thumbnail|Pemphigus vulgaris]] | ||
*Painful but rarely pruritic | *Painful but rarely [[pruritus|pruritic]] | ||
*Mucosal involvement common | *Mucosal involvement common | ||
**Presenting complaint in 50% of cases | **Presenting complaint in 50% of cases | ||
*Primary lesions | *Primary [[vesiculobullous rashes|lesions]] | ||
**Tense and clear vesicles/bullae on head, trunk, mucosa | **Tense and clear vesicles/bullae on head, trunk, mucosa | ||
**Become flaccid and turbid 2-3 days later | **Become flaccid and turbid 2-3 days later | ||
**Rupture and leave | **Rupture and leave sensitive denuded area of skin | ||
***Slow to heal and prone to secondary infection | ***Slow to heal and prone to secondary infection | ||
*Nikolsky's sign: Sliding pressure applied to normal skin adjacent to blister causes further ulceration | *Nikolsky's sign: Sliding pressure applied to normal skin adjacent to blister causes further ulceration | ||
**Intraepidermal | **Intraepidermal acantholysis: Keratinocytes separated at the basal layer from one another | ||
**Gives appearance of lesion expanding into adjacent tissue | **Gives appearance of lesion expanding into adjacent tissue | ||
**Transudate accumulate between keratinocyte and basement membrane which gives rise to new blisters | **Transudate accumulate between keratinocyte and basement membrane which gives rise to new blisters | ||
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==Management== | ==Management== | ||
[[File:PMC3760958 abd-88-0676-g01.png|thumb|Pemphigus vulgaris before (A) and after (B) treatment.]] | |||
*[[IVF]] and electrolyte resuscitation | *[[IVF]] and electrolyte resuscitation | ||
*Systemic [[corticosteroids]] | *Systemic [[corticosteroids]] | ||
Latest revision as of 17:27, 11 December 2024
Background
- Chronic autoimmune mucocutaneous disease against desmosomes in epidermis (bind keratinocytes)
- Mortality 5-15% sec to complications of long-term steroid therapy.
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 sensitive 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 acantholysis: 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 (chickenpox)
- Smallpox
- Monkeypox
- Disseminated gonococcal disease
- DIC
- Purpural fulminans
- Localized distribution
Afebrile
- Diffuse distribution
- Bullous pemphigoid
- Drug-Induced bullous disorders
- Pemphigus vulgaris
- Phytophotodermatitis
- Erythema multiforme major
- Bullous impetigo
- Localized distribution
- Contact dermatitis
- Herpes zoster (shingles)
- Dyshidrotic eczema
- Burn
- Dermatitis herpetiformis
- Erythema multiforme minor
- Poison Oak, Ivy, Sumac dermatitis
- Bullosis diabeticorum
- Bullous impetigo
- Folliculitis
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
- Plasmapheresis 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.