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autoimmune bullous disease (... pemphigus, bullous pemphigoid)
Etiology:
- bullous pemphigoid
- cicatricial pemphigoid
- epidermolysis bullosa acquisita
- pemphigus
- paraneoplastic pemphigus
- linear IgA bullous dermatosis
Clinical manifestations:
- persistent pruritic to painful blisters &/or bullae
- variable mucosal & ocular involvement
- scarring is variable
Laboratory:
- skin biopsy
- direct immunofluorescence testing of perilesional skin
- histopathology of lesional skin
- see ARUP consult [4] (Algorithm)
Management:
- immunosuppressants [1]
- rituximab may be of benefit at a cost of serious adverse events [5]
Interactions
disease interactions
Specific
epidermolysis bullosa acquisita (EBA)
linear IgA bullous dermatosis (IgA pemphigoid)
pemphigoid
pemphigus
General
autoimmune disease
chronic skin disease (chronic dermatologic disorder, chronic dermatopathy, chronic dermatosis)
vesiculobullous dermatitis
References
- Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18.
American College of Physicians, Philadelphia 2012, 2015, 2018.
- Parker SR, MacKelfresh J.
Autoimmune blistering diseases in the elderly.
Clin Dermatol. 2011 Jan-Feb;29(1):69-79
PMID: 21146735
- Schmidt E, Zillikens D.
Modern diagnosis of autoimmune blistering skin diseases.
Autoimmun Rev. 2010 Dec;10(2):84-9
PMID: 20713186
- ARUP consult
Immunobullous Skin Diseases Testing - Cancer
https://arupconsult.com/algorithm/porphyrias-testing-algorithm
Immunobullous Skin Diseases Testing - Pregnancy
https://arupconsult.com/algorithm/immunobullous-disease-testing-algorithm-pregnancy
- Schmidt E, Brocker EB, Goebeler M.
Rituximab in treatment-resistant autoimmune blistering skin
disorders.
Clin Rev Allergy Immunol. 2008 Feb;34(1):56-64
PMID: 18270859