Pemphigus vulgaris: Difference between revisions
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Created page with "== Background == * Blistering skin condition of unknown etiology * Associated with autoantibodies targeting cadherins === Drug-Induced === * Penicillamine and captopril * NSAIDs, penicillin, and cephalosporins * Possibly certain foods, though mostly anecdotal == Clinical Manifestations == * Initially presents in oral mucosa in 80% of cases * Followed by cutaneous lesions * Lesions are bullae that rupture leaving painful erosions * '''Nikolsky sign''' may be..." |
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== Background == |
== Background == |
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* Blistering skin condition of unknown etiology |
* Blistering skin condition of unknown etiology grouped with other forms of [[pemphigus]] |
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* Associated with autoantibodies targeting cadherins |
* Associated with autoantibodies targeting cadherins |
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== Clinical Manifestations == |
== Clinical Manifestations == |
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* Initially presents in oral mucosa in 80% of cases |
* Initially presents in oral mucosa in 80% of cases, and involves mucosa at some point in essentially all cases |
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** Erosions on buccal mucosa, palate, tongue, and inner lips |
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* Followed by cutaneous lesions |
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** Erosions and enanthema on gingiva |
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** Pain varies from mild to severe |
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* Can involve other parts of the upper respiratory tract, including nose (with crusting) and larynx (with hoarseness) |
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* Other mucosal surfaces can be involved as well |
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* Cutaneous lesions |
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* Lesions are bullae that rupture leaving painful erosions |
* Lesions are bullae that rupture leaving painful erosions |
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* '''Nikolsky sign''' may be present, where blisters form at sites of minor pressure or minor trauma |
* '''Nikolsky sign''' may be present, where blisters form at sites of minor pressure or minor trauma |
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== Management == |
== Management == |
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* Reviewed in [[CiteRef::schmidt2019pe]] |
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* Systemic corticosteroids |
* Systemic corticosteroids with prednisone 1 mg/kg daily |
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* Rituximab and other anti-CD20 monoclonal antibodies |
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* For more severe disease, add rituximab 2 x 1 g IV or azathrioprine or mycophenolate |
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* For treatment failure, increase prednisone dose, add rituximab (if not already using), add IVIG 2 g/kg over 2 to 5 days every 4 to 6 weeks, or try pulse steroids |
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[[Category:Dermatology]] |
[[Category:Dermatology]] |
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Latest revision as of 01:38, 7 January 2026
Background
- Blistering skin condition of unknown etiology grouped with other forms of pemphigus
- Associated with autoantibodies targeting cadherins
Drug-Induced
- Penicillamine and captopril
- NSAIDs, penicillin, and cephalosporins
- Possibly certain foods, though mostly anecdotal
Clinical Manifestations
- Initially presents in oral mucosa in 80% of cases, and involves mucosa at some point in essentially all cases
- Erosions on buccal mucosa, palate, tongue, and inner lips
- Erosions and enanthema on gingiva
- Pain varies from mild to severe
- Can involve other parts of the upper respiratory tract, including nose (with crusting) and larynx (with hoarseness)
- Other mucosal surfaces can be involved as well
- Cutaneous lesions
- Lesions are bullae that rupture leaving painful erosions
- Nikolsky sign may be present, where blisters form at sites of minor pressure or minor trauma
Diagnosis
- Based on clinical, histopathological, and laboratory evidence
- Skin biopsy is helpful
- Direct immunofluorescence can show net-like or chicken-wire pattern of IgG on the epidermis
- Tzanck smear shows acantholysis
Management
- Reviewed in 1
- Systemic corticosteroids with prednisone 1 mg/kg daily
- For more severe disease, add rituximab 2 x 1 g IV or azathrioprine or mycophenolate
- For treatment failure, increase prednisone dose, add rituximab (if not already using), add IVIG 2 g/kg over 2 to 5 days every 4 to 6 weeks, or try pulse steroids
References
- ^ Enno Schmidt, Michael Kasperkiewicz, Pascal Joly. Pemphigus. The Lancet. 2019;394(10201):882-894. doi:10.1016/s0140-6736(19)31778-7.
