Pemphigoid gestationis O26.4

Author: Prof. Dr. med. Peter Altmeyer

Our authors

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

Dermatitis multiformis gestationis; gestational pemphigoid; gravidarum pemphigus; Herpes gestationis; Hidroa gestationis; PG; Pregnancy pemphigoid

History
This section has been translated automatically.

Bunes, 1811; Milton 1872

Definition
This section has been translated automatically.

Rare, chronic, bullous, autoimmunological pregnancy dermatosis close to the bullous pemphigoid, occurring predominantly in the 2nd half of pregnancy or postpartum and characterized by the formation of large, usually considerably itchy inflammatory plaques and subepithelial blisters. Outside pregnancy the disease has been described in association with trophoblastic tumours (chorionic carcinoma, bladder mole).

Occurrence/Epidemiology
This section has been translated automatically.

Incidence: 1/2000-1/50.000 pregnancies/year. In Germany the incidence is about 2 new cases per 1 million inhabitants/year.

Etiopathogenesis
This section has been translated automatically.

Formation of complement fixing autoantibodies. The target antigen is the 180-kDa (BP 180) "bullous pemphigoid antigen = collagen XVII", in about 20% of cases the 230-kDa (BP 230), in the area of the hemidesmosomes of the dermoepidermal junction zone. This reactivity can be detected in the blood of patients and newborns. The cause of maternal autoantibody production is unclear. Furthermore, few patients react.

There is a correlation with the haplotypes HLA-DR3 and DR4.

It is possible that placental disease is the cause of the autoimmunological mechanism. This is especially supported by:

  • Disease occurs only during pregnancy as well as with chorionic carcinoma and bladder mole.
  • Circulating antibodies also bind to the basement membrane of the chorionic and amniotic epithelium.
  • Aberrant expression of MHC class II molecules in the area of the chorionic villi indicate an allogenic immune reaction against placental matrix antigens of paternal origin (Sadik 2016).
  • In the 2nd trimester BP180 can be detected in the amniotic epithelium.

Manifestation
This section has been translated automatically.

Mostly middle trimenon (on average in the 31st week for first gravids and 21.2 weeks for Multigravidae) or immediately post partum.

The average age of the first patient in larger studies is 30.5 years.

Localization
This section has been translated automatically.

Mainly located on the abdomen, periumbilical region and extremities. In 20% of the cases mucous membrane involvement.

Clinical features
This section has been translated automatically.

Usually the blistery exanthema is preceded by generalized itching for a few days or weeks. Skin changes preferably start periumbilical with grouped or disseminated, 0.2-4.0 cm large, intensely red, urticarial papules and plaques. Occasionally, cocardium-shaped formations are formed. Usually only after a few weeks formation of grouped (sometimes in herpetiform arrangement: see naming!) vesicles and bulging blisters (blistering is not obligatory). Associations with other blistering autoimmune diseases (pemphigus) have been reported.

Mucous membranes: The mucous membranes usually remain free.11 There is no association with striae distensae.

Newborns: In 10% of the cases, mostly mild skin changes are possible in the newborn (passive transplacental antibody transfer). These subside spontaneously within a few weeks.

Laboratory
This section has been translated automatically.

Bluteosinophilia.

Histology
This section has been translated automatically.

Subepidermal blistering. Cellular inflammatory reaction in the upper corium. Histoeosinophilia.

Direct Immunofluorescence
This section has been translated automatically.

Always linear complement deposits (C3) at the dermoepidermal junction zone. In about 1/3 of the cases analogous IgG, more rarely IgA and IgM deposits are detectable. With the salt separation method a 100% reactivity of C3 can be detected on the epidermal side of the salt-split preparation. The same antibodies are found in the infant's skin in the case of a co-infection.

Indirect immunofluorescence
This section has been translated automatically.

Detection of circulating antibodies (so-called herpes-gestationis factor) with complement fixing properties. In routine investigations, pemphigoid antibodies are found in only about 20% of patients.

Differential diagnosis
This section has been translated automatically.

PUPPP (almost always affects first-time mothers; no recurrence in further pregnancies; no antibody detection; periumbilical region is not clinically affected); erythema exsudativum multiforme; dermatitis herpetiformis; bullous pemphigoid; dermatosis, IgA linear.

Complication(s)
This section has been translated automatically.

Antibodies are diaplacentally transferable. 5-10% of infants may suffer from a more passable blister formation.

Therapy
This section has been translated automatically.

The aim of therapy is to suppress the often severe itching and blistering.

External therapy
This section has been translated automatically.

First of all, experiment with blanched, active substance-free shaking mixtures, emulsions or gels. Cool rinse off. If this is not sufficient, emulsions containing glucocorticoids such as 0.5-1% hydrocortisone emulsion (e.g. hydrogals, R123 ).

Internal therapy
This section has been translated automatically.

  • Use of oral antihistamines . Systemic antihistamines can be used for severe itching. The older sedative antihistamines (e.g. Dimetinden or Clemastin 1 tbl/day) are already considered safe during the 1st trimenon. Non-sedating antihistamines can be used in the 2nd/3rd trimenon or during lactation (e.g. loratadine, cetericine).
  • In the case of considerable eruption pressure with a considerable tendency to blister formation as well as intolerable itching with sleep disturbances, non-halogenated glucocorticoids e.g. prednisolone in a dosage of 0.5-1.0 mg/kg bw/day p.o. are indicated. See also " Pregnancy, Prescription drugs".
  • IVIG has been successfully used several times for therapy resistance.
  • In case of resistance to therapy, an immunapharesis may be necessary during pregnancy and postpartum an intensified immunosuppressive therapy.
  • Other alternatives, such as intravenous shock therapy with cyclophosphamide, plasmapheresis or dapsone are effective in cases of refractory therapy. However, they should be used postpartum.

Progression/forecast
This section has been translated automatically.

Usually no vital danger for mother and child. A flare-up of the disease at the time of birth is not unusual (short-term increase of the system therapy!). Spontaneous healing 2-3 weeks after delivery.

If the disease persists or manifests itself for the first time several weeks to months after delivery, a placental tumour should be excluded.

Recurrences are possible in the following gravidities, under hormonal contraception or with the beginning of menses. Caution with gestagen-containing contraceptives post partum.

The prognosis in children is favourable. In 20% of cases, increased rate of premature birth and the development of so-called "small -fordate-babies" (chronic placental insufficiency).

Note(s)
This section has been translated automatically.

The gestationis pemphigoid promotes the development of placental insufficiency and leads to underweight newborns (small-for-date babies). There is also an increased rate of premature births. Careful pregnancy monitoring is important. Outpatient gynaecological treatment and integration into a clinic with an infant intensive care unit are recommended. In about 10% of newborns, passive transplacental antibody transfers cause mild skin changes in the infants, which subside within a few days.

Literature
This section has been translated automatically.

  1. Al-Fouzan AW et al (2006) Herpes gestationis (Pemphigoid gestationis). Clin Dermatol 24: 109-112
  2. Ambros-Rudolph CM (2006) Dermatoses of pregnancy. J Dtsch Dermatol Ges 4: 748-759
  3. Ambros-Rudolph CM (2010) Specific pregnancy dermatoses. Dermatologist 61: 1014-1020
  4. Ambros-Rudolph CM (2017) Specific pregnancy dermatoses. Dermatologist 68: 87-94
  5. Boulinguez S et al (2003) Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients. Dermatology 206: 113-119
  6. Fabbri P et al (2003) The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis. Br J Dermatol 148: 1141-1148
  7. Huilaja L et al.(2014) Gestational pemphigoid. Orphanet J Rare Dis9:136.
  8. Loo WJ, Dean D, Wojnarowska F (2001) A severe persistent case of recurrent pemphigoid gestationis successfully treated with minocycline and nicotinamide. Clin Exp Dermatol 26: 726-727
  9. Ko BJ et al (2014) Intravenous Immunoglobulin Therapy for Persistent PemphigoidGestationis
    with Steroid Induced Iatrogenic Cushing's Syndrome. Ann Dermatol 26:661-663
  10. Sadik CD et al (2016) Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis.
    Clin Dermatol 34:378-82.
  11. Shimanovich I et al (2002) Pemphigoid gestationis with predominant involvement of oral mucous membranes and IgA autoantibodies targeting the C-terminus of BP180. J Am Acad Dermatol 47: 780-784
  12. Sitaru C et al (2003) Pemphigoid gestationis: maternal sera recognize epitopes restricted to the N-terminal portion of the extracellular domain of BP180 not present on its shed ectodomain. Br J Dermatol 149: 420-422
  13. Tani N et al (2015) Clinical and immunological profiles of 25 patients with pemphigoid gestationis. Br J Dermatol 172:120-129
  14. Vin H et al (2016) Concomitant pemphigus vulgaris andpemphigoid
    gestationis: a case report and review of the literature. Dermatol Online J 22.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020