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Autoimmune skin disorders

  • Pemphigus vulgaris
  • Bullous pemphigoid
  • Dermatitis herpetiformis
  • Pemphigoid gestationis
  • Epidermolysis bullosa acquisita
  • Bullous lupus erythematosus

Pemphigus vulgaris

IgG antibodies against intercellular attachment sites (desmosomes) between Keratinocytes.
Type II hypersensitivity reaction
Vesicles and bullae develop on skin and oral mucosa.
Intraepithelial vesicles are located above the basal layer (suprabasal)
(1) Basal cells resemble a row of tombstones.
(2) Acantholysis of keratinocytcs in the vesicle fluid

Clinical features

Age: 40-60 years
Usually the site of the blisters are the torso & head and there is always an involvement of the mucus membrane.
The blisters are flaccid and fragile & may have many erosions.

Treatment

Corticosteroids and other immunosuppressive agents (e,g., methotrexate, azathioprine


Pemphigus vulgaris: mucus membrane



Pemphigus vulgaris: Torso


Pemphigus vulgaris

Dermatitis herpetiformis


  • IgA-anti-lgA complexes deposit at the tips of the dermal papillae.
  • Produces subepidermal vesicles with neutrophils.
  • Strongly correlated with celiac disease
  • Increase in antireticulin and endomysial antibodies.



Clinical features

Age: young patients associated with coeliac disease.
Sites: Elbow, lower backs, buttocks.
Blisters are often excoriated and not present.

Treatment
(1) Gluten-free diet
(2) Dapsone or sulfapyridine
(3)Nikolsky sign positive




Bullous pemphigoid

IgG antibodies against the basement membrane.
Type II hypersensitivity reaction.
Vesicles are subepidermal 
(1) Develop on the skin and oral mucosa.
(2) No acantholytic cells in vesicle lluid
Disease usually subsides after months or years.


Clinical features

Age: 60 and above
Site: Trunk (especially flexures) and limbs with occassional involvement of the mucous membrene.
The blisters are tense.

Treatment

Disease usually subsides after months or years.
May requires systemic corticosteroid in resistant cases.