Derma Flashcards
_____ is an autoimmune disease of unclear etiology in which the body essentially becomes allergic to its own skin
Pemphigus vulgaris
Pathology of Pemphigus vulgaris
Antibodies are produced against antigens in the intercellular spaces of the epidermal cells. They attack the “glue” that holds the epidermal
cells together
Pemphigus vulgaris is most often idiopathic, but _____
can occasionally cause
ACE inhibitors or penicillamine
____occurs in patients age 30s and 40s, whereas ____
occurs in those age 70s and 80s.
pemphigoid Vulgaris
bullous pemphigoid
Which is more serious?
pemphigoid Vulgaris
bullous pemphigoid
Pemphigus vulgaris is a much more serious and potentially life-threatening disease than pemphigoid
The presence of the _____ (the easy removal of skin by just a little pressure from the examiner’s finger, pulling the skin off like a sheet) is seen in pemphigus vulgaris,
staphylococcal scalded skin syndrome, and toxic epidermal necrolysis
Nikolsky sign
The lesions of ____ are painful, not pruritic.
pemphigus vulgaris
pemphigus vulgaris
The most accurate diagnostic test is to biopsy the skin and to use immunofluorescent stains. These stains will detect _____
intercellular deposits of IgG and C3 in the epidermis
Tx of pemphigus vulgaris
Treatment is with systemic glucocorticoids, such as prednisone. Topical steroids will not be sufficiently strong
Tx of PV
For those in whom steroids are ineffective or not tolerated, you can use
azathioprine, mycophenolate, or cyclophosphamide.
Rituximab and IVIG are also effective.
_____2× as common as pemphigus vulgaris and occurs in elderly persons age 70s and 80s. It can also be drug induced with sulfa drugs, including furosemide, penicillamine, and others.
Bullous Pemphigoid
Pathology of Bullous Pemphigoid
The defect occurs at the dermo-epidermal junction, so the layer of skin that separates off is much thicker
DX of Bullous Pemphigoid
The most accurate diagnostic test is a biopsy with immunofluorescent antibodies at the dermo-epidermal junction (basement membrane).
TX of Bullous Pemphigoid
Systemic steroids, such as prednisone, are the standard means of treatment.
Alternative TX of Bullous Pemphigoid
Tetracycline or erythromycin combined with nicotinamide is the alternative to steroids. Use topical steroids only if no oral lesions are present
___ is a disorder of porphyrin metabolism. Deficiency
of the enzyme uroporphyrinogen decarboxylase results in an abnormally high accumulation
of porphyrins, which then leads to a photosensitivity reaction
Porphyria cutanea tarda
Asscn of Porphyria cutanea tarda
porphyria cutanea tarda is associated with increased liver iron stores. Diabetes is found in 25% of patients.
HIV, alcoholism, liver disease, chronic hepatitis C, or a woman taking oral contraceptives
SSx of Porphyria cutanea tarda
Fragile, nonhealing blisters are seen on the sun-exposed parts of the body, such as the backs of the hands and the face.
This leads to hyperpigmentation of the skin in general and hypertrichosis of the face
Dx of Porphyria cutanea tarda
The diagnostic test is a level of urinary uroporphyrins. Uroporphyrins are elevated 2–5× above the coproporphyrins in this disease
Tx of Porphyria cutanea tarda
The best initial step in management is to stop drinking alcohol (although it is unlikely to be effective) and to discontinue all estrogen use
PCT
The most effective therapy to use if this is insufficient
is _______
phlebotomy to remove iron.