Disease |
Epidemiology / prognosis |
Causes |
( antibodies ) |
Pemphigus vulgaris 3
|
Onset between 50 – 60 years of age , without sex predominance . Incidence between 0.76 and 32 cases per million people-year and prevalence of 95 per million people . Fatal without treatment in 2 – 5 years |
IgG anti-DSG-3 |
Very fragile flaccid blisters , on normal skin , painful erosions that bleed easily , partially covered with scabs , in seborrhoeic areas . Difficulty healing ( often with hypopigmented macules , without scarring ). Positive Nikolsky sign |
It usually precedes the cutaneous manifestations |
Pemphigus foliaceous 3
|
Not usually serious . It can remain localised for years or progress rapidly |
IgG anti-DSG-1 |
Very superficial and fragile vesicles . They often present only crusted and scaly erosions on an erythematous base . Seborrhoeic distribution |
Not present |
Paraneoplastic pemphigus 4
|
Rare ; about 5 % of all pemphigus cases . It predominates in men between 45 and 70 years of age Serious . Possible development of bronchiolitis obliterans . |
IgG anti-DSG ( 1 and 3 ) and antiplakin . Humoral and cellular immunity |
Polymorphous : erythematous macules , flaccid bullae , erosions , tense bullae , erythema multiforme-like , and lichenoid eruptions |
Painful and severe oral ( stomatitis ) and conjunctival erosions ( pseudomembranous conjunctivitis ) |
Bullous pemphigoid 13
|
The most common AI subepidermal BD in the elderly Incidence between 6 and 13 cases per million-year . Predominantly affecting elderly people . Significantly associated with mortality ( rates 6 – 41 % within the first year after diagnosis ) |
IgG against BP180 and BP230 |
From only pruritus , urticarial lesions , papules , and eczematous plaques to large , generalised blisters and erosions |
Possible involvement |
Mucus membrane pemphigoid 14
|
Incidence : 1.3 – 2.0 cases per million-year . Mainly in the elderly . Unpredictable prognosis with common relapses and progression |
BP180 , BP230 , laminin 332 , integrin α6 and β4 , and type VII collagen |
Erythematous plaques and subsequent vesicles in the head , neck , and upper trunk |
Erosions and fibrosis / scarring |
Dermatitis Prevalence up to 75 herpetiformis 15 cases / 100.000 people . It affects mainly adults with a slight predominance in men Excellent long-term prognosis if complete adherence to gluten-free diet |
IgA against epidermal transglutaminase |
Symmetric itchy polymorphic rash with erythema , papules and tense subepidermal blisters ( predominantly on elbows , knees , and buttocks ), often with erosions , crusts , and post – inflammatory hyperpigmentation secondary to scratching |
Rarely affected |
Epidermolysis bullosa acquisita 16
|
No predominance for either sex . Incidence range between 0.08 and 0.5 cases per million-year Complete remission usually in children . Worse prognosis in adults with mucosal involvement |
Autoantibodies against collagen VII ( mainly IgG ) |
Mucocutaneous blistering with numerous phenotypes |
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