Section 1: Bullous and Blistering Diseases Flashcards
Pemphigus Vulgaris:
This is an autoimmune disease of unclear etiology in which the body becomes, essentially, allergic to its own skin. Antibodies are produced against antigens in the intercellular spaces of the epidermal cells
List the causes of pemphigus vulagaris
- Idiopathic
- ACE inhibitors
- Penicillamine
Pemphigus vulgaris acts like a burn, because the bullae occur from destruction within the epidermis and so are relatively thin and fragile
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8594-8602). Kaplan Publishing. Kindle Edition.
Nikolsky’s sign
Nikolsky’s sign is the easy removal of skin by just a little pressure, with the examiner’s finger pulling it off like a sheet.
List the conditions in which Nikolsky’s sign is seen
- Pemphigus vulgaris
- Staphylococcus scalded skin (SSS) syndrome
- Toxic epidermal necrolysis
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8594-8602). Kaplan Publishing. Kindle Edition.
True or False:
Pemphigus vulgaris is pruritic
False. The lesions are painful; not pruritic
Pemphigus vulagaris
- Most accurate diagnostic test
- Best initial Rx
- Rx when (2) fails
- Skin biopsy
- Glucocorticoids (prednisone)
- When steroids are ineffective:
- Azathiaprine
- Mecophenolate
- Cyclophosphamide
Bullous Pemphigoid
** Pemphigoid can be drug induced by sulfa drugs and others**. The fracture of the skin causing the blisters is relatively deep, and the bullae are thicker walled and much less likely to rupture than the bullae of pemphigus vulgaris. Oral lesions are rare. Because the bullae are tense and intact, the skin is better protected. There is no dressing for skin as good as the skin. Hence, there is much less fluid loss and infection is much less likely as compared with pemphigus vulgaris.
- Diagnostic test
- Best initial Rx
- Alternative to (2) above
- Biopsy with immunofluorescent antibodies
- Systemic steroids, such as prednisone
- Alternatives to steroids
- Tetracycline
- Erythromycin with nicotinamide
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8614-8627). Kaplan Publishing. Kindle Edition.
True or False:
- Oral lesions are common in Bullous Pemphigoid
- Mortality is much less likely in bullous pemphigoid
- Pemphigoid can be drug induced by sulfa drugs and others
- False, they are rare
- True
- True
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8614-8627). Kaplan Publishing. Kindle Edition.
Compare Pemphigus vulagris (PV) with Bullous Pemphigus (BP)
- Age range for PV is 30s anf 40s WHILE age range for BP is 70s and 80s
- PV is lifethreatening but BP resolves
- Bullae are thin and fragile in PV BUT are thick and intact in BP
- The mouth is involved in PV BUT not so in BP
- Nikolsky’s sign is positive in PV; it is not present in BP
Pemphigus Foliaceus
- What is it associated with?
- Diagnostic tests?
- Rx?
- This blistering disease is associated with
- Other autoimmune diseases
- Drug induced by ACE inhibitors or NSAIDs.
- Biopsy
- Treated with steroids in the same fashion as pemphigus vulgaris.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8651-8659). Kaplan Publishing. Kindle Edition.
True or False (Pemphigus Foliaceus):
- Foliaceus is much more superficial than pemphigus vulgaris and bullous pemphigoid, and intact bullae are not seen because they break so easily
- There are no oral lesions.
- True
- True
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8651-8659). Kaplan Publishing. Kindle Edition.
Porphyria Cutanea Tarda (PCT)
This is a disorder of porphyrin metabolism resulting in a photosensitivity reaction to an abnormally high accumulation of porphyrins
List the conditions associated with PCT
- Alcoholism
- Liver disease
- Chronic hepatitis C
- Oral contraceptives
- Liver disease, such as chronic hepatitis or hemochromatosis PCT, is associated with increased liver iron stores
- Diabetes is found in 25 percent of patients
Use the mnemonic “CH LOAD”
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8661-8665). Kaplan Publishing. Kindle Edition.
CF of PCT
- Nonhealing blisters on the sun-exposed parts of the body, such as the backs of the hands and the face
- Hyperpigmentation of the skin
- Hypertrichosis of the face
Hypertrichosis is excess hair
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8661-8665). Kaplan Publishing. Kindle Edition.
PCT
- Diagnostic test
- Rx
- Urinary uroporphyrins
- Manage as follows:
- Stop drinking alcohol
- Stop all estrogen use
- Use barrier sun protection
- Use phlebotomy to remove iron. Deferoxamine is used to remove iron if phlebotomy is not possible
- Chloroquine increases the excretion of porphyrins
Urticaria
Acute urticaria is a hypersensitivity reaction, most often mediated by IgE and mast cell activation, which results in evanescent wheals and hives. It is a type of localized, cutaneous anaphylaxis but without hypotension and hemodynamic instability. The onset of the wheals and hives is usually within 30 minutes and lasts for < 24 hours. Itching is prominent
List the causes of acute urticaria
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8676-8681). Kaplan Publishing. Kindle Edition.
- Medications (aspirin, NSAIDs, morphine, codeine, penicillins, phenytoin, and quinolones)
- Insect bites
- Foods (peanuts, shellfish, tomatoes, and strawberries)
- Emotions (occasionally)
- Contact with latex (in any form)
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8682-8692). Kaplan Publishing. Kindle Edition.
List the factors associated with chronic urticaria
Pressure on the skin: Pressure on the skin resulting in localized urticaria is also known as dermatographism
Cold
Vibration
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8682-8692). Kaplan Publishing. Kindle Edition.
Rx of severe acute urticaria
Rx of life threatening reactions
H1 antihistamines: Severe, acute urticaria is treated with older medications
- Diphenhydramine (Benadryl)
- Hydroxyzine (atarax)
- Cyproheptadine
For lifethreatening reactions
- Add systemic steroids to above Rx
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 8682-8692). Kaplan Publishing. Kindle Edition.