68. Pemphigoid/Pemphigus
Definition
Pemphoigoid and pemphigus are a group of chronic, autoimmune blistering diseases that are classified depending on the tissue predominately involved. Cicatricial pemphigoid (CP) predominately affects the mucous membranes, such as the conjunctiva, buccal membrates, and oropharynx, whereas bullous pemphigoid (BP) predominately affects the skin.
Pemphigoid/Pemphigus Variants
Pemphigoid
• Bullous pemphigoid
• Cicatricial pemphigoid
Pemphigus
• Erythematous pemphigus
• Pemphigus foliaceous
• Pemphigus vegetans
• Pemphigus vulgaris
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| Pemphigoid/Pemphigus. Bullous pemphigoid; close-up of tense bullae. |
Incidence
The exact incidence of pemphigoid/pemphigus is not known.
Etiology
In CP, basement membrane zone target antigens, bullous pemphigoid antigen Z (BPAGZ), and epiligrin are attacked by autoantibodies.
In BP, the true cause is unknown; however, several possible contributing factors have been proposed, including immunogenetics, age, epitope spreading, complement activation, and chemokines.
Signs and Symptoms
Cicatricial Pemphigoid
• Alopecia from scalp erosions
• Ankyloblepharon
• Conjunctivitis
• Corneal epithelium keratinization
• Corneal sulcus shortening
• Dysphagia
• Epistaxis
• Erosions of the genitelia (clitoris, labia, glans, penile shaft)
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