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Pemphigus of the eyelids
M Seishima1, Z Oyama, H Shimizu
1Department of Dermatology, Ogaki Municipal Hospital, Minaminokawa-cho 4-86, Ogaki, 503-8502, Japan. seimarik@gifu-u.ac.jp
European Journal of Dermatology : EJD
|March 29, 2001
Summary
This case study describes an unusual presentation of pemphigus vulgaris, a rare autoimmune blistering disease, affecting primarily the oral mucosa. Early diagnosis and treatment with corticosteroids led to significant improvement.
Area of Science:
- Dermatology
- Immunology
- Autoimmune Diseases
Background:
- Pemphigus vulgaris is a chronic autoimmune blistering disease characterized by IgG autoantibodies against desmogleins.
- Mucosal-dominant pemphigus vulgaris presents with oral erosions and blisters, often preceding skin lesions.
Observation:
- A 56-year-old woman presented with widespread oral erosion and eyelid papules.
- Histopathology revealed suprabasal acantholysis in oral and eyelid lesions.
- Direct and indirect immunofluorescence showed intercellular IgG and C3 deposits in the epidermis.
Findings:
- Enzyme-linked immunosorbent assay confirmed high titers of antibodies against desmoglein 3 (118) and desmoglein 1 (25.9).
- The patient was diagnosed with pemphigus vulgaris, likely a mucosal-dominant type, based on clinical, histopathological, and serological findings.
Implications:
- This case highlights the importance of considering pemphigus vulgaris in patients with unexplained oral erosions, even with atypical presentations.
- Prompt diagnosis and appropriate immunosuppressive therapy, such as corticosteroids, are crucial for managing pemphigus vulgaris and improving patient outcomes.