Porphyria Cutanea Tarda
Epidemiology &
Etiology:
- Group
of disorders with an inborn or acquired disturbance of porphyrin metabolism
- Association
with systemic disorders including:
- Hepatic
insufficiency
- Hepatitis
C
- alcoholism
Common sites:
- Dorsal
aspects of hands and fingers
Gross features:
- urticaria
- vesicles
- heal
with scarring
- exacerbated
by sunlight
- hirsuitism esp. lateral sides of
face commonly
Histologic
features:
- subepidermal vesicle
- extensive
solar elastosis
- marked
thickening of walls of superficial dermal vessels
- festooning
of papillary dermal tips
- minimal
inflammatory response in early lesions
Immunophenotype
& Special Stains:
|
Marker:
|
Sensitivity:
|
Specificity:
|
|
PAS
(thickened superficial vessels)
|
|
|
|
IgG (linear, along
the dermal-epdiermal junction and surrouding blood vessels)
|
|
|
|
IgM
|
|
|
|
C3
|
|
|
Molecular features:
Other features:
- Exacerbated
by sunlight
- hirsuitism esp. lateral sides of
face commonly
-
References: