Pemphigoid gestationis is an autoimmune blistering disease of pregnancy caused by autoantibodies directed against BP180 (type XVII collagen) in the basement membrane zone. This article discusses the current management of this rare disease.
Pemphigoid gestationis (PG) is a rare autoimmune bullous disease and has been reported as occurring in 1 in 20,000 to 50,000 pregnant women. Due to pregnancy being an exclusion criteria in most clinical trials and the extensive list of medications contraindicated during pregnancy, it is no surprise that there is a scarcity of literature when it comes to management of this condition. Breastfeeding also imposes limitations on recommended treatments. Most reports are small case series and retrospective reviews. It has been accepted that potent topical corticosteroids are helpful in milder cases, whereas more extensive disease requires systemic corticosteroids, which are considered the mainstay of therapy. There have been reports of the success of immunosuppressive treatment in recalcitrant, chronic, relapsing cases.
There is recent evidence that suggests that early-onset PG and blister formation is associated with adverse pregnancy outcomes in the form of decreased gestational age at delivery, perterm birth, and low-birth-weight babies. This finding suggests that in these more severe cases, early institution of treatment, mainly systemic corticosteroids, is essential to prevent these adverse outcomes.
This article focuses on the use of topical and systemic corticosteroids, as well as immunosuppressives, in the management of PG.
Management of pemphigoid gestationis
This section discusses the various treatments reported for PG. Table 1 summarizes the treatment options, while Table 2 shows the different pregnancy categories assigned to various medications by the United States Food and Drug Administration (US FDA). Table 3 shows the classification recommended for use by the Therapeutic Goods Authority (TGA) in Australia. The Australian categorization system is not hierarchical and differs from that of the US FDA. The subcategorization of the B category is based on animal data, as human data are lacking or inadequate. Furthermore, a B category does not imply greater safety than a C category. Some medications in the same drug class (ie, topical and systemic corticosteroids) are assigned different categories, as the risk of birth defects may be dependent on the dose, route of administration, and dosing regimen.
| Medication/Treatment | Pregnancy Category a | Type of Study/Author(s) | Dose | Use (Prepartum or Postpartum) |
|---|---|---|---|---|
| Topical corticosteroids | C A b | Multiple reports Mainstay of treatment | Potent CS applied twice a day (ie, clobetasol propionate 0.05% or betamethasone dipropionate 0.05%) | Both |
| Systemic corticosteroids | C A b | Multiple reports Mainstay of treatment | PSN 1 mg/kg/d or equivalent dose | Both |
| Tetracyclines and nicotinamide | D | Case reports Amato et al, 2002; Loo et al, 2001 | Doxycycline 200 mg/d + nicotinamide 500 mg/d Minocycline 100 mg/d + nicotinamide 1000 mg/d | Postpartum |
| Cyclophosphamide | D | Case report Castle et al, 1996 | 0.75 g/m 2 slow IV infusion | Postpartum |
| Cyclosporin | C | Case report Hern et al, 1998 | 100 mg/d + PSN 10–20 mg/d IVIG 0.4 g/kg/d × 5 d | Postpartum |
| Azathioprine | D | Case reports Kreuter et al, 2004 Sereno et al, 2005 Cianchini et al, 2007 Amato et al, 2003 | 50–100 mg/d (+ IVIG 0.5 g/kg/d × 2 d + PSN 15 mg) 150 mg/g ineffective followed by IVIG 0.4 g/kg/d × 6 cycles 150 mg/d (+ dapsone 125 mg/d + PSN 100 mg/d + IVIG 0.5 g/kg/d × 3 d) ineffective followed by rituximab 375 mg/m 2 weekly for 4 wk 100 mg/d (+ PSN 40–150 mg/d + dapsone 50 mg/d + 5 plasmaphereses) | Postpartum |
| Dapsone | C B2 b | Case reports Cianchini et al, 2007 Amato et al, 2003 | Dapsone 125 mg/d (+ PSN 100 mg/d + azathioprine 150 mg/d + IVIG 0.5 g/kg/d × 3 d) ineffective followed by rituximab 375 mg/m 2 weekly for 4 wk 50 mg/d (+ PSN 40–150 mg/d + azathioprine 100 mg/d + 5 plasmaphereses) | Postpartum |
| IVIG | C | Case reports Doiron and Pratt, 2010 Hern et al, 1998 Kreuter et al, 2004 Sereno et al, 2005 | 40 g IV × 3 d monthly during pregnancy 0.4 g/kg/d × 5 d (+ cyclosporin 100 mg/d + PSN 10–20 mg/d) 0.5 g/kg/d × 2 d (+ PSN 15 mg imuran 50–100 mg/d) IVIG 0.4 g/kg/d × 6 cycles | Both Prepartum Postpartum Postpartum |
| Rituximab | C | Case report Cianchini et al, 2007 | Rituximab 375 mg/m 2 weekly for 4 wk | Postpartum |
| Plasmapheresis | Unclassified | Case reports Amato et al, 2003 Van de Wiel et al, 1980 Marker et al, 2011 | 5 plasmaphereses + (PSN 40–150 mg/d + azathioprine 100 mg/d + dapsone 50 mg/d) 3 plasma exchanges during 26 wk, 5 during delivery, and 2 postpartum 15 immunoapheresis sessions (14 pre- and 1 postpartum) in addition to methylprednisolone 60 mg/d tapered | Both Postpartum Both Both |
| Goserelin | X D b | Case report Garvey et al, 1992 | 6-Month course of goserelin (in addition to PSN 20–80 mg/d) | Postpartum |
a Labeling of medications in pregnancy and lactation vary in different countries.
| Pregnancy Category | Details | Examples |
|---|---|---|
| A | Adequate and well-controlled studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters) | |
| B | Animal reproduction studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women | |
| C | Animal reproduction studies have shown an adverse effect on the fetus and there are no adequate and well-controlled studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks | Cyclosporin, rituximab, dapsone, IVIG Topical CS (betamethasone, mometasone, triamcinolone, hydrocortisone) Systemic CS (prednisone, prednisolone, methylprednisolone) |
| D | There is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience or studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks | Azathioprine, cyclophosphamide, doxycycline, minocycline |
| X | Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience, and the risk involved in use of the drug in pregnant women clearly outweighs potential benefits | Goserelin, systemic isotretinoin, thalidomide, methotrexate |
Related posts:
Autoimmune Blistering Diseases Part II—Diagnosis and Management
Rituximab and its Use in Autoimmune Bullous Disorders
Evidence-Based Management of Bullous Pemphigoid
Management of Linear IgA Disease
Management of Autoimmune Bullous Diseases in France: A Nationwide Network of 30 Centers
Treatment of Chronic Bullous Disease of Childhood
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
