Pemphigus encompasses a group of potentially life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin.
Pemphigus vulgaris (PV) is the most common variant and affects skin and mucosa.
Pemphigus foliaceus (PF) is confined to the skin. Paraneoplastic pemphigus (PNP) is rare; it can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and esophagus.
Diagnosis is based on clinical findings of erosions (epidermal loss) and blisters of the skin, mucosa, or both.
Direct immunofluorescence staining of biopsied skin shows immunoglobulin and C3 deposits on the keratinocyte surface, and histology reveals keratinocyte acantholysis.
Treatment aims to reduce or eliminate pathologic autoantibodies and consists of long-term immunosuppressive therapy.
Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are three broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP).
Underlying intraepithelial blister formation is caused by IgG autoantibodies against desmoglein 3 (Dsg3) and/or desmoglein 1 (Dsg1) on the cell surface of epidermal keratinocytes.
Pemphigus is associated with pain and impaired sleep. Swallowing difficulties are present if there is esophageal involvement. It is associated with increased rates of depression.
History and exam
Key diagnostic factors
- chronic erosive blistering of the skin, mucosa, or both
- chronic mouth erosions (PV, PNP)
- painful lips (PNP)
- shortness of breath (PNP)
Other diagnostic factors
- pruritic scalp (PV, PF)
- bloody nose (PV, PNP)
- painful skin (PV, PF, PNP)
- dysphagia (PV)
- pruritic skin (PV, PF)
- conjunctivitis (PV, PNP)
- increasing age
- HLA DR4 (PV)
- HLA DQ1 (PV)
- HLA DRB1 (PNP)
- associated malignancy (PNP)
- ACE inhibitors
- interleukin 2
- exposure to hematophagous insects
1st investigations to order
- skin biopsy, hematoxylin and eosin stain
- skin biopsy, direct immunofluorescence
Investigations to consider
- indirect immunofluorescence on serum
- serum ELISA
- upper gastrointestinal endoscopy
- chest CT scan
- serum immunoblot (Western blot)
mild pemphigus vulgaris
mild pemphigus foliaceus
moderate to severe pemphigus vulgaris or pemphigus foliaceus
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: with disease control/complete remission
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: without complete remission
moderate to severe pemphigus vulgaris or pemphigus foliaceus 12 to 18 months after initial therapy: complete remission
- Familial benign pemphigus (Hailey-Hailey disease)
- Bullous pemphigoid (BP)
- Linear IgA bullous dermatosis
- Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV)
- Diagnosis and management of pemphigus: recommendations by an international panel of experts
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