Pemphigoid gestationis

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Pemphigoid gestationis
Other names: Gestational pemphigoid, herpes gestationis[1]
Pemphigoid gestationis (DermNet NZ immune-pemgest1).jpg
Pemphigoid gestationis
SymptomsBlisters, itch, hives[1]
ComplicationsPremature delivery of a small baby, a few who may be born with blisters and urticaria,[1] bacterial infection[2]
Usual onsetMiddle of pregnancy or shortly after[1]
DurationAround 6-months[1]
CausesAutoimmune[1]
Risk factorsPregnancy, molar pregnancy, choriocarcinoma, oral contraceptive pill[1]
Diagnostic methodAppearance, skin biopsy, immunofluorescence[1]
Differential diagnosisPruritic urticarial papules and plaques of pregnancy, erythema multiforme, drug reactions, blistering scabies[1]
TreatmentCorticosteroid by application to skin or by mouth[1]
MedicationPrednisolone 40mg/day by mouth[1]
FrequencyRare, 1 in 20,000 to 50,000 pregnancies[1]

Pemphigoid gestationis (PG), also known as gestational pemphigoid, is an autoimmune variant of the skin disease bullous pemphigoid, and first appears in pregnancy.[3] It presents with tense blisters, small bumps, hives and intense itching, usually starting around the belly button before spreading to the chest and limbs in mid-pregnancy or shortly after delivery.[1] The head, face and mouth are not usually affected.[4]

PG is caused by antibodies acting against the mother's own skin which results in the skin layers splitting and forming blisters, possibly triggered by some placenta cells entering into the mother's blood.[4] It typically lasts around six months and can be triggered by subsequent pregnancies, menstrual periods and oral contraceptive pill.[1] A molar pregnancy and choriocarcinoma can provoke it.[1] In some people, it persists long-term.[1] It is associated with premature delivery of a small baby, a few who may be born with blisters and urticaria, which generally resolves within six weeks.[4] There is a risk that the blisters can become infected with bacteria.[2] It does not spread from one person to another, and does not run in families.[4] Around 10% of affected people develop Grave's disease.[1]

Diagnosis is by its appearance and behaviour, blood test, biopsy and immunofluorescence.[5] It can resemble pruritic urticarial papules and plaques of pregnancy (PUPP), erythema multiforme, drug reactions and blistering scabies.[1] Treatment is generally with corticosteroids, either by application to skin or by taking prednisolone 40mg/day mouth.[1] Other medicines that have been tried include pyridoxine, and tetracycline with nicotinamide, among others.[1]

It is rare.[1] Around 1 in 20,000 to 50,000 pregnancies are affected.[1] It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus.[4]

Definition

Pemphigoid gestationis is an autoimmune variant of the skin disease bullous pemphigoid, and first appears in pregnancy.[3]

Signs and symptoms

It presents with tense blisters, small bumps, hives and intense itching, usually starting around the belly button before spreading to chest and limbs in mid-pregnancy or shortly after delivery.[1] The head, face and mouth are not usually affected.[4]

  • Pemphigoid gestationis

    Pemphigoid gestationis

  • Pemphigoid gestationis

    Pemphigoid gestationis

  • Pemphigoid gestationis

    Pemphigoid gestationis

  • Pemphigoid gestationis

    Pemphigoid gestationis

  • Pemphigoid gestationis

    Pemphigoid gestationis

  • Pemphigoid gestationis

    Pemphigoid gestationis

  • Pemphigoid gestationis

    Pemphigoid gestationis

Causes

See also: List of human leukocyte antigen alleles associated with cutaneous conditions

Circulating complement-fixing IgG antibodies attach to the lamina lucida resulting in the skin layers splitting and forming blisters, possibly triggered by some placenta cells entering into the mother's blood.[1] The triggering antigen, transmembrane collagen XVII, is part of the baby's membrane cells that encourage the movement of placental cytotrophoblastic cells.[1] The disease appears to be triggered by female hormones.[1] It can recur in subsequent pregnancies, menstrual periods and oral contraceptive pill.[1] A molar pregnancy and choriocarcinoma can provoke it.[1]

Complications

In some people, it persists long-term.[1] It is associated with premature delivery of a small baby, a few who may be born with blisters and urticaria, which generally resolves within six weeks.[4] It does not spread from one person to another, and does not run in families.[4] Around 10% of affected people develop Grave's disease.[1]

Diagnosis

Micrograph of gestational pemphigoid showing the characteristic subepidermal blisters and abundant eosinophils. HPS stain.

Diagnosis is by its appearance and behaviour, blood test, biopsy and immunofluorescence.[5]

Differential diagnosis

Early in the disease, PG may appear similar to several other skin diseases.[4] Conditions that may appear similar include pruritic urticarial papules and plaques of pregnancy (PUPPP), erythema multiforme, drug reactions and blistering scabies.[1]

Treatment

The main aim of treatment is to relieve the itch, prevent further blistering, and treat any overlying infection.[4] PG is generally managed with the use of corticosteroids; prednisolone 40mg/day reducing to the lowest dose required to ease symptoms.[1] Milder cases may be managed with applying steroid creams to the skin.[1]

After the baby is born, if necessary, a more extensive range of immunosuppressive treatment may be administered for those unresponsive to corticosteroid treatments; pyridoxine, tetracycline with nicotinamide, cyclophosphamide, dapsone, rituximab, methotrexate, or intravenous immunoglobulin.[1]

Epidemiology

It is rare.[1] Around 1 in 20,000 to 50,000 pregnancies are affected.[1]

History

It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus.[4]

See also

References

  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 1.18 1.19 1.20 1.21 1.22 1.23 1.24 1.25 1.26 1.27 1.28 1.29 1.30 1.31 1.32 1.33 1.34 1.35 James, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac (2020). "21. Chronic blistering dermatoses". Andrews' Diseases of the Skin: Clinical Dermatology (13th ed.). Edinburgh: Elsevier. pp. 464–465. ISBN 978-0-323-54753-6.
  2. ↑ 2.0 2.1 "Pemphigoid gestationis | DermNet NZ". dermnetnz.org. Archived from the original on 9 March 2022. Retrieved 23 March 2022.
  3. ↑ 3.0 3.1 Wakelin, Sarah H. (2020). "22. Dermatology". In Feather, Adam; Randall, David; Waterhouse, Mona (eds.). Kumar and Clark's Clinical Medicine (10th ed.). Elsevier. pp. 686–687. ISBN 978-0-7020-7870-5.
  4. ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 "Pemphigoid (herpes) gestationis". www.BAD.org.uk. British Association of Dermatologists. November 2020. Archived from the original on 2 February 2020. Retrieved 21 March 2022.
  5. ↑ 5.0 5.1 Johnstone, Ronald B. (2017). "6. Vesiculobullous reaction pattern". Weedon's Skin Pathology Essentials (2nd ed.). Elsevier. p. 123. ISBN 978-0-7020-6830-0. Archived from the original on 2021-05-25. Retrieved 2022-03-21.

External links

Classification
External resources
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