Grading & Level of Importance: C
Bullous pemphigoid is the most common type: typically present in older adults (>60 -70 years). Annual incidence: 6-30 new cases per million population. M = F.
Autoimmune diseases with sub-epidermal blister formation.
Subepidermal separation caused by circulating auto-antibodies directed against hemi-desmosomal adhesion proteins of the cutaneous basement membrane (bullous pemphigoid antigens, BP180 and BP230). Triggered in some cases by drugs (gliptins, diuretics).
Skin, and sometimes mucous membranes.
Direct and indirect immunofluorescence: demonstration of antibodies directed against basement membrane zone (linear deposits of C3 and IgG). Hypereosinophilia is common, ELISA for detection of BP180 and BP230 specific auto-antibodies in serum.
Subepidermal blister with tissue eosinophilia.
Skin-related sepsis, fluid and protein loss. Mortality among elderly patients. Permanent mucosal scars in mucous membrane pemphigoid.
Tzanck test negative. Histology (sub-epidermal blister formation, tissue eosinophilia). Direct immnunofluorescence: linear deposits of C3 and IgG along the dermo-epidermal junction. Indirect inmmunofluorescence: circulating IgG which binds to the dermo-epidermal junction of normal skin and to the roof of human sodium chloride split skin. ELISA for detection of BP180 and BP230 specific auto-antibodies in serum.
With other autoimmune bullous dermatoses, especially epidermolysis bullosa acquisita, which is caused by antibodies against collagen VII. Linear IgA dermatosis is characterised by linear deposits of IgA rather than IgG along the basement membrane zone (this is the most common autoimmune bullous disease in childhood). Bullous arthropod bites, allergic contact dermatitis, Steven-Johnson syndrome, erythema multiforme, porphyria cutanea tarda/pseudoporphyria.
Topical treatments: High potency topical corticosteroids (e.g. clobetasol propionate), anti-septics, general wound care. Systemic: corticosteroids (first-line therapy), azathioprine, mycophenolate mofetil, dapsone, methotrexate, nicotinamide/tetracycline, rituximab and high dose intravenous immunoglobulins.
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