| • | AKA pemphigoid gestationis |
clinical:
| • | intensely pruritic, blistering disease of pregnancy |
| • | lesions usually begin around the umbilicus |
| • | 2nd or 3rd trimester or immediately post-partum |
| • | may clear spontaneously and then recur with delivery |
| • | mucosal disease nearly non-existent |
pathogenesis:
| • | IgG vs. 180kd component of hemidesmosome |
| • | mnemonic: “you have to be 18 to get pregnant” |
| • | thought to be due to HLA mismatch between fetus and mother |
histology:
| • | DIF = linear C3 at basement membrane zone |
| • | histology: intense dermal papillary edema (“tear drop sign”) is probably specific for this condition |
course:
| • | disappears 1 or 2 months after delivery (but may persist) |
| • | tends to recur with subsequent gestations |
| • | may also flare (recur) with oral contraceptives |
| • | newborn with mild cutaneous involvement approx. 10% of time (will resolve without treatment) |
treatment:
| • | systemic steroids (40mg/day prednisone) |
|
Incidence
|
Recurrence in future pregnancies
|
Newborn with cutaneous involvement
|
PUPPPS
|
1:160
|
unusual
|
none
|
Herpes Gestationis
|
1:50,000
|
yes
|
10%
|
|