Autoimmune bullous diseases during pregnancy: insight into pathogenetic mechanisms and clinical features.
G Ital Dermatol Venereol
; 154(3): 256-262, 2019 Jun.
Article
in En
| MEDLINE
| ID: mdl-30375213
ABSTRACT
Pemphigoid gestationis (PG), also known as herpes gestationis, is the prototypic pregnancy-associated autoimmune bullous disease (AIBD), but also the other AIBDs, notably pemphigus vulgaris, may begin or exacerbate during pregnancy. Although the increase in concentration of T and B regulatory cells makes pregnancy a state of increased immunologic tolerance toward the semiallogeneic fetal antigens, a prevalent T helper (Th) 2 profile, that is reported to be associated with pregnancy, may cause exacerbation of pemphigus and AIBDs in general during this period. Active disease may lead to stillbirth, spontaneous abortion, preterm pregnancy, low birthweight, and neonatal pemphigus. PG is a rare AIBD usually starting during the third trimester of pregnancy and healing in the postpartum. It is due to the formation of autoantibodies directed against different epitopes of bullous pemphigoid (BP) 180 as a consequence of the aberrant expression of BP180 in the placental tissue of genetically predisposed women. PG is characterized by vesicles with herpetiform distribution, blisters and urticarial elements typically involving the periumbilical area and the distal portion of the upper limbs. Diagnosis is based on 1) physical examination; 2) histopathological pattern consisting of a dermal inflammatory infiltrate rich in eosinophils; 3) direct immunofluorescence test demonstrating linear deposits of complement fraction 3 and immunoglobulin G along the basement membrane zone; 4) detection of circulating autoantibodies by means of indirect immunofluorescence or enzyme linked immunosorbent assay. Here, we provide an updated overview on the pathophysiologic mechanisms of pregnancy-associated or pregnancy-exacerbated AIBDs, focusing also on peculiar clinical features of these disorders.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pregnancy Complications
/
Skin Diseases, Vesiculobullous
/
Pemphigoid Gestationis
Type of study:
Diagnostic_studies
Limits:
Female
/
Humans
/
Pregnancy
Language:
En
Journal:
G Ital Dermatol Venereol
Year:
2019
Document type:
Article
Affiliation country:
Italia
Publication country:
IT
/
ITALIA
/
ITALY
/
ITÁLIA