Pemphigus and pemphigoid (epidermal) antibodies
| Category | Immunology |
|---|---|
| Test background |
The severe blistering skin diseases (bullous dermatoses) are associated with a variety of pathogenic autoantibodies directed toward structural components of the epidermis. Desmoglein (DSG) 1 & 3 antibodies are associated with pemphigus, characterised by flaccid blisters and erosions. Pemphigus vulgaris, a predominantly mucosal disease is associated with DSG 3 and/or DSG1 antibodies and is the most common pemphigus variant. Pemphigus foliaceus is associated with DSG 1 antibodies and only affects the skin. Basement membrane antibodies BP180 and BP230 are associated with bullous pemphigoid, most commonly seen in men over 70 years of age. There is sub epithelial blister formation causing tense bullae, itchy erythematous and urticated plaques. Direct immunofluorescence on skin biopsy remains the gold standard method for diagnosis in these patients. This assay is performed by indirect immunofluorescence on monkey oesophagus tissue sections. |
| Clinical Indications |
Investigation of blistering skin disease |
| Reference range | Negative |
| Sample & container required | Serum (RST rust top) |
| Sample volume | 5-10 mL blood (1 mL serum) |
| Turnaround time | 28 days |
| Notes | Not currently performed in house; this test is referred to the Protein Reference Unit (PRU), Sheffield. |