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]

Causes

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. p. 464-465. ISBN 978-0-323-54753-6. Archived from the original on 21 March 2022. Retrieved 2022-03-15.
  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. Archived from the original on 2022-03-21. Retrieved 2022-03-21.
  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