Dermatology Flashcards
Pathophysiology of pemphigus vulgaris?
LIFE-THREATENING autoimmune disease of unclear etiology in which the body produces antibodies against antigens in the intercellular spaces of the epidermal cells
Causes of pemphigus vulgaris?
- idiopathic
- ACE inhibitors
- penicillamine
Classic sign seen in pemphigus vulgaris?
Nikolsky’s sign - easy removal of the skin by just a little pressure (also seen in SSSS, TEN)
Dx of pemphigus vulgaris?
Bx
Tx of pemphigus vulgaris?
- glucocorticoids (e.g. Prednisone)
- when steroids ineffective –> Azathioprine, Mycophenolate, Cyclophosphamide
Causes of bullous pemphigoid?
can be drug-induced (e.g. sulfa drugs and others)
How are bullous pemphigoid blisters different from pemphigous vulgaris?
- BP: fracture of skin is deeper, bullae thicker walled, much less likely to rupture; skin better protected
- PV: fracture of skin more superficial, much easier to remove skin
Dx of bullous pemphigoid?
bx w/ immunofluorescent antibodies
Tx of bullous pemphigoid?
- systemic steroids (e.g. prednisone)
- OR tetracyline OR erythromycin with nicotinamide
Age differences between bullous pemphigoid and pemphigus vulgaris?
BP: 70s and 80s
PV: 30s and 40s
Mouth involvement in bullous pemphigoid vs. pemphigus vulgaris?
BP: No
PV: Yes
Cause of pemphigus foliaceous?
- in association w/ other AI diseases
- drug-induced (ACE inhibitors, NSAIDs)
How is pemphigus foliaceous different from BP and PV?
-foliaceous is much more superficial, intact bullae not seen because they break so easily, no oral lesions
Dx of pemphigus foliaceous?
Bx
Tx of pemphigus foliaceous?
steroids (e.g. Prednisone)
What is porphyria cutanea tarda?
Disorder of porphyrin metabolism resulting in a photosensitivity reaction to an abnormally high accumulation of porphyrins
What diseases/medications are a/w porphyria cutanea tarda?
- alcoholism
- liver disease (e.g. due to chronic Hep C or hemochromatosis)
- OCPs
- DM
How does PCT present?
- nonhealing blisters on sun-exposed parts of the body (e.g. backs of hands & face)
- hyperpigmentation of face
- hypertrichosis of face
Dx of PCT?
test for urinary uroporphyrins (elevated 2-5X higher than coproporphyrins)
Tx of PCT?
- stop drinking alcohol
- stop all estrogen use
- use barrier sun protection
- use phlebotomy to remove iron (add deferoxamine if phlebotomy not possible)
- chlorquine (increases excretion of porphyrins)
Pathophysiology of urticaria?
HSR, most often mediated by IgE and mast cell activation –> evanescent wheals and hives. A type of localized cutaneous anaphylaxis but w/o hypotension and hemodynamic instability
Presentation of urticaria?
wheals and hives w/i 30 min of inciting factor, lasts < 24 hrs. Itching prominent
Most common causes of urticaria?
- meds (ASA, NSAIDs, morphine, codeine, PCNs, phenytoin, quinolones)
- insect bites
- foods (peanuts, shellfish, tomatoes, strawberries)
- emotions (occasionally)
- latex contact
Tx of urticaria?
- H1 antihistamines (Diphenhydramine, hydroxyzine, cyproheptadine)
- Life threatening reactions: add systemic steroids
- chronic Tx: newer nonsedating antihistamines (e.g. loratadine, desloratadine, fexofenadine, cetirizine)
- when trigger cannot be avoided –> desensitization