autoimmune bullous disease (... pemphigus, bullous pemphigoid)
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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]
More general terms
More specific terms
- epidermolysis bullosa acquisita (EBA)
- linear IgA bullous dermatosis (IgA pemphigoid)
- pemphigoid
- pemphigus
References
- ↑ 1.0 1.1 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: https://www.ncbi.nlm.nih.gov/pubmed/21146735
- ↑ Schmidt E, Zillikens D. Modern diagnosis of autoimmune blistering skin diseases. Autoimmun Rev. 2010 Dec;10(2):84-9 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20713186
- ↑ 4.0 4.1 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
- ↑ 5.0 5.1 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: https://www.ncbi.nlm.nih.gov/pubmed/18270859