Pemphigoid gestationis

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Pemphigoid gestationis
Other names: Gestational pemphigoid, herpes gestationis[1]
Pemphigoid gestationis (DermNet NZ immune-pemgest1).jpg
Pemphigoid gestationis
SpecialtyDermatology. obstetrics
SymptomsBlisters, itch, hives[1]
ComplicationsBaby: Premature delivery, blisters and hives,[1] bacterial infection[2]
Mother: Grave's disease[1]
Usual onsetMid pregnancy to shortly after birth[1]
DurationAround 6-months[1]
CausesAutoimmune[1]
Risk factorsPregnancy, molar pregnancy, choriocarcinoma, birth control pill[1]
Diagnostic methodAppearance, skin biopsy with immunofluorescence[1]
Differential diagnosisPruritic urticarial papules and plaques of pregnancy, erythema multiforme, drug reactions, scabies[1]
TreatmentCorticosteroid applied to the skin or by mouth[1]
MedicationPrednisolone 40mg/day by mouth[1]
Frequency1 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, that first appears in pregnancy.[3] Symptoms include blisters, small bumps, hives, and intense itching.[1] It usually starting around the belly button before spreading to the chest and limbs; with the head, face, and mouth being spared.[1][4] Onset is usually in mid-pregnancy to shortly after delivery.[1][5]

It is caused by antibodies against the mother's own skin, resulting in skin layers splitting and forming blisters.[4] It is possibly triggered by some placenta cells entering the mother's blood.[4] Risks include molar pregnancy and choriocarcinoma.[1] It does not spread between people and does not run in families.[4] It typically lasts six months and can reoccur in future pregnancies, menstrual periods, and from birth control pill.[1] In some people, it persists long-term.[1] Diagnosis is based on progression of symptoms, blood tests, and skin biopsy with immunofluorescence.[6][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 applied to the skin or taking prednisolone 40mg/day by mouth.[1] Other medicines that have been tried include pyridoxine, and tetracycline with nicotinamide.[1] Complications can include premature delivery of a small baby; or a baby born with blisters and hives, which generally resolves within six weeks.[4] The blisters can subsequently become infected with bacteria.[2] Around 10% of affected women develop Grave's disease.[1]

Pemphigoid gestationis affects around 1 in 20,000 to 50,000 pregnancies.[1] The condition was first described by London physician John Laws Milton in 1872.[7] It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus.[4]

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]

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]

Causes

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

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]

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.[6]

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 1.36 1.37 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. Archived from the original on 2023-06-30. Retrieved 2023-05-15.
  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.
  4. 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 "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 Roth, MM (1 February 2011). "Pregnancy dermatoses: diagnosis, management, and controversies". American journal of clinical dermatology. 12 (1): 25–41. doi:10.2165/11532010-000000000-00000. PMID 21110524.
  6. 6.0 6.1 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.
  7. Murrell, Dédée F. (28 April 2011). AutoImmune Blistering Disease Part I, An Issue of Dermatologic Clinics. Elsevier Health Sciences. p. 447. ISBN 978-1-4557-1247-2. Archived from the original on 19 December 2023. Retrieved 16 December 2023.

External links

Classification
External resources