MTB 3 - Dermatology Flashcards
How does pemphigus vulgaris present?
Painful bullae that are easily ruptured
Involvement of the mouth
Nikolsky’s sign
What’s the pathophysiology of pemphigus vulgaris?
IgG Antibodies against desmosomes.
Desmosomes allow cell-to-cell adhesion
What causes pemphigus vulgaris?
Idiopathic
ACE-I
Penicillamine
What 3 things can give Nikolsky’s sign?
Pemphigus vulgaris
SSSS
Toxic epidermal necrolysis
How to diagnose pemphigus vulgaris?
Biopsy of skin showing autoantibodies on immunofluorescent studies in a netlike pattern
Treatment of pemphigus vulgaris?
Prednisone
If inneffective, use azathioprine
Without treatment, this condition is fatal
Describe bullous pemphigoid:
Much milder disease than pemphigus vulgaris
Bullae stay intact so less fluid loss and infection
Mouth involvement is uncommon
Pathophysiology of bullous pemphigoid?
IgG antibodies against hemidesmosomes (basement membrane). Linear immunofluorescence.
How to diagnose bullous pemphigoid?
Skin biopsy showing linear immunofluorescence of IgG against the hemidesmosomes of the basement membrane
Treatment for bullous pemphigoid?
Prednisone
What ages does pemphigus vulgaris and bullous pemphigoid present?
Pemphigus vulgaris: 30’s and 40’s
- Life-threatening
- Thin and fragile bullae
- Mouth involved
- Nikolsky’s
Bullous pemphigoid: 70’s and 80’s
- Thick and intact bullae
- No mouth involvement
What is porphyria cutanea tarda (PCT)?
Disorder of porphyrin metabolism –>
High accumulation of porphyrins –>
Causes photosensitivity reaction
List 5 associations with PCT:
Alcoholism Liver disease -Chronic Hep C -Hemochromatosis OCP Diabetes
How does PCT present?
Nonhealing blisters on sun-exposed part of body
Hyperpigmentation of skin
Hypertrichosis of face (excess hair)
How to test for PCT?
Urinary uroporphyrins will be 2-5x higher than coproporphyrins
How to manage PCT?
Stop alcohol
Stop estrogen use
Use sun protection
Phlebotomy to remove iron or deferoxamine
Chloroquine increases excretion of porphyrins
What is urticaria?
A localized, cutaneous anaphylaxis without hypotension or hemodynamic instability.
Caused by a hypersensitivity reaction by IgE and mast cell activation
Results in wheals and hives very pruritic
When is the onset of wheals and hives in urticaria? How long does it last?
Within 30 minutes of exposure. Lasts for
Most common medication causes of urticaria:
Aspirin NSAIDs Morphine Codeine Penicillin Phenytoin Quinolones
Other common causes of urticaria besides medications:
Insect bites
Foods (peanuts, shellfish, tomatoes, strawberries)
Latex
Emotions (occasionally)
Chronic urticaria is associated with:
Pressure on skin
Cold
Vibration
How to treat urticaria?
Always: Antihistamines (diphenhydramine, hydroxyzine, cyproheptadine)
Life-threatening: Systemic steroids
Chronic therapy: Nonsedating antihistamines (loratadine, cetirizine)
When do you do desensitization for someone with urticaria?
When the trigger can’t be avoided (bee stings in a farmer).
What must you stop taking prior to desensitization?
Beta-blockers (cause you might need to use epinephrine which uses beta receptors)