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56 Cards in this Set
- Front
- Back
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What are the immunobullous skin disorders?
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Pemphigus foliaceus
Pemphigus vulgaris Bullous pemphigoid Epidermolysis bullosa acquisita |
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Where does pemphigus foliaceous occur?
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Within the granular layer
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Where does pemphigus vulgaris occur?
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Spinous layer
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Where does bullous pemphigoid occur?
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Below the basal layer but above the basement membrane
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Where does epidermolysis bullosa occur?
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Below the basal layer; high dermis
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What is the function of the deesmosomes?
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Hold the cells together in the epidermis
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What is the function of the hemidesmosome?
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Hold the epidermis to the dermis, like an anchor
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What proteins are in the desmosomes?
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DSG 1
DSG 3 |
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What are the proteins in the hemidesmosome?
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Bpag1/2
Laminin 5 Alpha6/Beta4 integrin |
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What are tests that you should order up if you suspect an autoimmune blistering disease?
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Direct immunofluoresence (in the skin)
Biopsy Indirect immunoflouresence (in the blood) |
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What is the target for pemphigus foliaceus?
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DSG1
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What do the lesions look like in pemphigus foliaceus?
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Skin lesions:
Scaly Superficial Crusted erosions Looks like crusty corn flakes |
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Where does pemphigus foliaceus localize?
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Trunk, extremities
The V's |
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What does p. foliaceous look like under the scope?
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LIght microscopy: a splitting under the epidermis; immune deposits in the granular layer
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Where is desmoglein 1 expression the highest?
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Inside the granular layer, decreases as you go down into the skin
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What is the treatment for foliaceus?
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Corticosteroids
-Locally if in one place -Systemically if you have serious disease |
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Where do blisters take place in pemphigus vulgaris?
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Within the spinous layer
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What kinds of autoantibodies are there in p. vulgaris?
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DSG 3/DSG 1 autoantibodies
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What's the visual difference between pemphigus vulgaris and foliaceus?
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IN vulgaris you can actually see the blisters
Also, in foliaceous you don't have blisters in the mouth, in vulgaris you do. |
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What do the lesions look like in pemphigus vulgaris?
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Flaccid blisters on normal looking skin
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What do you see in light microscopy in pemphigus vulgaris?
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Tombstones.
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What's the difference between p. vulgaris and foilaceous on direct IF?
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You can't tell the difference!
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What proteins are mutated in pemphigus vulgaris?
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Desmoglein 3, 1
Desmoglein 3 in the oral mucosa |
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What's the treatment for pemphigus vulgaris?
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Corticosteroids
Plasmapheresis-->cyclophosphamide Rituxan |
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What are the proteins mutated in bullous pemphigoid?
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BPag1/BPag2
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What do the lesions look like in bullous pemphigoid?
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Large, tense bullae on an erythematous base; very puritic
YOU SEE TSNES BLISTERS!!! |
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What's the distribution of bullous pemphigoid?
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Trunk
Extremities Mucosal involvementis rare |
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When do people get bullous pemphigoid?
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When they're old
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What does a light microscopy slide look likein bullous pemphigoid?
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You see a split below the epidermis that's just above the BM
Eosinophils (IT"S WHAT CAUSES THE ITCH!) |
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What kinds of proteins deposit in bullous pemphigoid?
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IgG
C3 |
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What's the prognosis for bullous pemphigoid?
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Self-limited; may last 3 years
Some do last longer, though |
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What's the treatment for bullous pemphigoid?
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Mild disease: topical steroids
Extensive disease: prednisone, tetracyclines, |
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What is cictrical pemphigoid?
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Disease of the mucous membranes
You have ulcers and erosions |
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Where does cicatrical pemphigoid distribute?
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Mucus membranes:
Eyes (especially concerning!) Oropharynx Nasal cavity Larynx Esophagus |
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What are the antibodies that are mutated in cicatrical pemphigoid?
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Laminin 5
Alpha6/beta4 integrin BP antigen 2 |
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What are the major concerns with cicatrical pemphigoid?
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Eye pain/problems
Laryngeal/esophagical strictures, scarring |
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What's the treatment for cicatrical pemphigoid?
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Cortisteroids
Immunosuppressives |
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What protein is mutated in epidermolysis bullosa?
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Collagen 7
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Where does epidermolysis bullosa happen?
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Within the dermis
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What are the kinds of skin lesions that happen with epidermiolysis bullosa?
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Blisters on non-erythematous skin that heals with formation of milia
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What does a light slide of epidermolysis bullos look like?
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Complete shearing of the basement membrane
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What's the course for epidermolysis?
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Frequently self-limited
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What's the treatment for epidermolysis?
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We can't treat this.
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What are the different kinds of inherited mechanobullous diseases?
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Epidermolysis bullosa:
-Simplex -Junctional -Dystrophic |
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What proteins are mutated in epidermolysis bullosa simplex?
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Keratins 5, 14
Right near the basal cell. |
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What proteins are mutated in junctional EB?
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Laminin 5
Above lamina densa. Cell is sheared off, lamina densa is still intact |
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What proteins are mutated in dystrophic EB?
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Collagen VIII
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What's the inheritance of epidermolysis bullosa simplex?
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Autosomal dominant
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What do the lesions look like in epidermolysis bullosa simplex?
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Mild: blisters in traumatized areas in adults
Severe: generalized blisters in neonates |
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What's the treatment for epidermolysis bullosa?
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Can't.
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What's the inheritance of junctional epidermolysis bullosa?
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AR
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What do the lesions look like in junctional epidermolysis bullosa?
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Blisters and erosions at birth
MUCOSA ALL OVER THE PLACE! |
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What disease is this?
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This is a perioral granulation tissue
Junctional epidermolysis bullosa |
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What's the gene mutated in junctional epidermolysis bullosa?
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Laminin 5
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Where does dystrophic epidermolysis bullosa distribute?
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Generalized to all mucosla surfaces?
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What's a common cancer in people with dystrophic epidermolysis bullosa?
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SCC
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