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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