- skin biology
- signs and symptoms
- investigations
- conditions and diseases
Section Links
Pemphigus
- epidemiology
- signs and symptoms
- diagnosis
- pathophysiology
- causes and risk factors
- treatments
- course
- patient education
- community resources
- references
group of autoimmune disorders characterized by intraepidermal blister formation
- uncommon but important
- tends to be body-wide and very painful
- also mucosal involvment in the marority of patients
- Nikolsy's sign is positive, with large blisters that easily shear
- can be paraneoplastic
- IgG directed against keratinocyte cell surface protein
- occurs just above basal layer
- affects men and women equally
- age of onset 50-60
- more common in Jewish/Mediterranean
- can be found using indirect immunofluorescence or skin biopsy staining
Epidemiology
Signs and Symptoms
Diagnosis
take edge of blister for histology, and para-blister region for immunofluorescence
Pathophysiology
Causes and Risk Factors
Treatments
Glucocorticoid treatment is tarted early, with good control
Course
Beofre glucocorticoids, disease was uniformly fatal.
Still signigicant morbidity and mortality
Patient Education
Community Resources