Gestational pemphigoid
| Gestational pemphigoid | |
| ICD-10 | O26.4 |
|---|---|
| ICD-9 | 646.8 |
| MeSH | D006559 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Gestational Pemphigoid or Pemphigoid Gestationis (PG) is a rare autoimmune blistering skin disease that occurs during pregnancy, typically in the second or third trimester, and/or immediately following pregnancy. It was originally called Herpes Gestationis because of the blistering appearance. Gestational Pemphigoid is not associated with the herpes virus.
Presentation
PG typically starts as a blistering rash in the naval area and then spreads over the entire body. It is sometimes accompanied by raised, hot, painful welts called plaques. There have been cases where only plaques occurred. PG creates a histamine response that causes extreme relentless itching (pruritus).
Causes
The cause of PG is not known, although many researchers believe that the antibodies that protect the placenta become confused and begin to increase in number. These antibodies are directed at the skin and attack the protein the binds the outer layer of skin to the body. This action causes the skin to lift up, creating blisters.
Differential diagnosis
PG often is confused with PUPPP (pruritic urticarial papules and plaques of pregnancy), especially if it occurs in a first pregnancy. PUPPP typically begins in stretch mark areas of the abdomen and usually ends within 2 weeks after delivery. PUPPP is not an autoimmune disease.
Diagnosing PG is done by biopsy using direct immunofluorescence (DIF); appearance; and blood studies.[1]
Treatment
The most accepted way to treat PG is with the use of corticosteroids, i.e. prednisone; and/or topical steroids, i.e. clobetasol and betamethasone. Suppressing the immune system with corticosteroids helps by decreasing the number of antibodies that are attacking the skin. Treating PG can be difficult and can take several months. Some cases of PG persist for many years.
There is no cure for PG. Women who have PG are considered in remission if they are no longer blistering. Remission can last indefinitely, or until a subsequent pregnancy. PG usually occurs in subsequent pregnancies; however, PG often seems more manageable because it is anticipated.
Pregnant women with PG should be monitored for conditions that may affect the fetus, including, but not limited to, low or decreasing volume of amniotic fluid and preterm labor.
Support
Gestational Pemphigoid can be a frightening and devastating condition. Because of it's rarity, few studies have been conducted, the largest group studied included 87 women.[2] A Gestational Pemphigoid Support Group was created to help those diagnosed with PG.
References
- Ambros-Rudolph CM, Mullegger RR, Vaughan-Jones SA, et al: The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients. J Am Acad Dermatol 2006; 54: 395-404
- Boulinguez S, Bedane C, Prost C, et al: Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients. Dermatology 2003; 206(2): 113-9.
- Kroumpouzos G, Cohen LM: Dermatoses of pregnancy. J Am Acad Dermatol 2001 Jul; 45(1): 1-19; quiz 19-22
- Castro LA, Lundell RB, Krause PK, Gibson LE. Clinical experience in pemphigoid gestationis: Report of 10 cases. 2006;55:823-8
See also
- Bullous pemphigoid (BP)
- Cicatricial or Mucous membrane pemphigoid (CP)