2. EPIDERMOLYSIS BULLOSA
The term epidermolysis bullosa describes a heterogeneous group of inherited blistering
mucocutaneous disorders.
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3. DEPENDING ON THE DEFECTIVE MECHANISM OF CELLULAR
COHESION, THERE ARE FOUR BROAD CATEGORIES:
• 1. Simplex
• 2. Junctional
• 3. Dystrophic
• 4. Kindler syndrome
Each category consists of several forms of the disorder.
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12. PEMPHIGUS
• The condition known as pemphigus represents four related
diseases of an autoimmune origin:
• 1. Pemphigus vulgaris
• 2. Pemphigus vegetans
• 3. Pemphigus erythematosus & …
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13. Pemphigus vulgaris is the most common of these disorders (vulgarisis Latin for
common).Even so, it is not seen very often.
The estimated incidence is one to five cases per million people diagnosed each
year in the general population.
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14. for unknown reasons, of autoantibodies that are directed against the epidermal cell
surface glycoproteins, desmoglein 3 and desmoglein 1.
These desmogleins are components of desmosomes
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29. BULLOUS PEMPHIGOIDIS
the most common of the autoimmune blistering conditions, occurring at an
estimated rate of ten cases per million population per year.
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One significant difference
is that the clinical course in patients with bullous pemphigoid is usually
characterized by periods of remission followed by relapse, whereas the course in
patients with
mucous membrane pemphigoid is usually protracted and progressive.
38. MINOR
MAJOR
TEN?
The difference between Stevens-Johnson syndrome and toxic epidermal
necrolysis
is the degree of skin involvement, with Stevens-Johnson
syndrome having less than 10% of the body surface affected by lesions,
and toxic epidermal necrolysis having more than 30% involvement.
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39. LICHEN PLANUS
Careful examination of the surface of the skin papules reveals a fine, lacelike
network of white lines (Wickham striae)
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41. If the erosive component is severe, epithelial separation from the
underlying connective tissue may occur.
This results in the relatively rare presentation of bullous lichen
planus.
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