Blistering Diseases? Flashcards
(19 cards)
What is Pemphigus Vulgaris commonly associated with?
ACE-I
Penicillamine
Phenobarbital
Penicillin
What is the cause of Pemphigus Vulgaris?
Autoab split the epidermis causing
-thin walled bullae: easily rupture
-Involvement of the mouth
-Fluid loss and infection: act like a burn
What is the most characteristic finding in Pemphigus Vulgaris?
Nikolsky sign
What is the most accurate test for Pemphigus Vulgaris?
Biopsy showing autoab on immunofluourescence
How do you treat Pemphigus Vulgaris?
Systemic Steroids
Azathioprine or Mycophenolate: wean pt off steroids
Rituximab (anti-CD20) or IVIG in refractory cases
What is the cause of Bullous Pemphigoid?
Ab to Hemidesmosomes
How does Bullous Pemphigoid present?
Bullae stay intact: less commonly involve the mouth
What is the most accurate test for Bullous Pemphigoid?
Biopsy with IF stains
What is the best initial therapy for Bullous Pemphigoid?
Prednisone
What does mild Bullous Pemphigoid respond to?
Erythromycin, dapsone and Nicotinamide
What is Porphyria Cutanea Tarda?
Hypersensitivity of the skin to abnormal Poryphyrins when exposed to light
How does Porphyria Cutanea Tarda (PCT) present?
Blistering disorder of skin in sun-exposed in areas with
-Liver disease; HCV, Alcoholism
-Estrogen Use
-Iron overload: Hemochromatosis
What is the underlying cause of PCT?
Deficiency of Uroporphyrin Decarboxylase activity
What is the most accurate test for PCT?
Increased Uroporphyrins in plasma or urine
How do you treat PCT?
Correct the underlying cause
What is Acute Intermittent Porrphyria (AIP)?
Altered heme biosynthesis due do a deficiency of HMBS “Porphobilinogen Deaminase”
How does AIP present?
Abdominal pain: Most common
Neuro: peripheral sensory or motor alterations
Psych: irritability, depression, anxiety, insomnia, hallucinations or paranoia
Urinary: retention, incontinence, dysuria
How do you test for AIP?
Elevated urinary Delta-aminolevulinic acid and porphobilinogen
How do you treat acute attacks of AIP?
10% glucose and hemin
