Autoimmune Blistering Diseases

  • Dermatitis herpetiformis
  • Linear IgA Bullous Disease
  • Pemphigoid
  • Pemphigus


  

Dermatitis Herpetiformis (DH) is a very itchy blistering disease that especially on the elbows, knees, shoulder blades and tailbone areas. It is related to other forms of celiac disease as it is caused by IgA antibody against gluten (gliadin). Diagnosis is by skin biopsy, direct immunofluorescence and lab work. Treatment is gluten avoidance completely and/or dapsone. There is a risk of GI lymphoma if untreated and continued gluten ingestion in this and other forms of celiac disease. 
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Linear IgA Bullous Dermatosis (LABD)is an autoimmune blistering disease with IgA deposition in the epidermal-dermal junction. Most commonly seen as rings or groups of smaller blisters. Itching and burning can be associated with the eruption. Treatment can be dapsone or any of a variety of systemic immune suppressor drugs.
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Pemphigoid Bullous Pemphigoid, Cicatricial Pemphigoid, Mucous Membrane Pemphigoid (BP, CP, MMP, other variants) is a group of autoimmune blistering disease that can affect the skin and/or mucous membrane of the eyes, nose mouth, throat or genitals. Therapy ranges from intermittent topical steroids to various systemic immunosuppressant drugs. Causes are not clearly known but some individuals may have disease triggered by medications or underlying other diseases.
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  Pemphigus Vulgaris, Pemphigus Foliaceous (PV, PF, other variants) is an autoimmune blistering disease that can affect the skin and/or mucous membrane of the eyes, nose mouth, throat or genitals. Therapy ranges from intermittent topical steroids to various systemic immunosuppressant drugs. Causes are not clearly known, some cases can be drug-induced or related to underlying diseases. PF is a variant with more superficial skin blisters and crusts. All forms can cause scalp lesions. 
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