eMedicine Specialties > Dermatology > Bullous Diseases
Pemphigoid Gestationis: Treatment & Medication
Updated: Dec 9, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
The goals of treatment are to relieve pruritus and to suppress extensive blister formation.
- To minimize the risk for the mother and fetus, use the lowest effective dose of medication to suppress disease activity.
- Even with optimum control, some blisters may continue to develop, and patients may have persistent pruritus.
- The risks and benefits of therapy must always be evaluated for the mother and the fetus.
Consultations
- A dermatologist and an obstetrician must coordinate care for patients with pemphigoid gestationis.
- The pediatrician also must be aware of the diagnosis and the medications the mother is receiving.
- Because pemphigoid gestationis is an uncommon disease, referral to immunodermatologists with expertise in the treatment of patients with severe or persistent disease is appropriate.
Medication
Tepid baths, compresses, and emollients may help alleviate pruritus. Patients with mild disease can be treated with antihistamines and midpotency topical or intralesional steroids, such as triamcinolone. However, these are usually ineffective in more severe cases, and systemic steroids remain the mainstay of therapy. Prednisone at 0.5 mg/kg/d is usually started, and the response to therapy is gauged by the abatement of pruritus and blister formation. Once blistering has ceased and lesions have begun to heal, the dose of prednisone is tapered to the minimum dose required to control the disease. Reported steroid-sparing agents used as the adjuvant therapy in the treatment of pemphigoid gestationis include azathioprine, dapsone, methotrexate, intravenous immunoglobulin, cyclosporine, pyridoxine, plasmapheresis, and minocycline/nicotinamide. Chemical oophorectomy with goserelin (a luteinizing hormone–releasing analog) also may hold promise.
Corticosteroids
Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, they modify the body's immune response to diverse stimuli.
Triamcinolone (Aristocort)
Treats inflammatory dermatosis that is responsive to steroids. Decreases inflammation by suppressing migration of PMN leukocytes and reversing capillary permeability. Apply water-based creams sparingly and rub into skin until they disappear. Ointments, which are in a petrolatum-based vehicle, are used to treat chronic dermatoses. Use 0.1% cream or ointment.
Adult
Apply thin film after bathing bid/tid until response is achieved
Pediatric
6-12 years: 0.03-0.2 mg/kg IM q1-7 d
>12 years: Administer as in adults
None reported
Documented hypersensitivity; fungal, viral, and bacterial skin infections; use in neonates; use in intertriginous areas (eg, groin, armpits) or on face; use on areolas in breastfeeding women; decreased skin circulation
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Prolonged use, applications over large areas, and use of potent steroids and occlusive dressings may result in systemic absorption, which may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria; at typically doses, topical steroids pose no significant risks for the fetus or breastfeeding infants
Prednisone (Deltasone)
Immunosuppressant used for the treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. In patients with severe blistering in whom topical or intralesional steroids fail to elicit a response, oral prednisone may be indicated.
Adult
20-60 mg PO qd; usually administered as single morning dose; if no response or if disease worsens after 4–7 d, increase dose by 50% q3-5d until response occurs.
Pediatric
4-5 mg/m2/d PO; alternatively, 0.05-2 mg/kg PO divided bid/qid.
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Absolute: Systemic fungal infection, herpes simplex keratitis, hypersensitivity (usually with corticotropin, occasionally with IV preparations)
Relative: HTN, active TB, CHF, prior psychosis, positive IPPD test result, glaucoma, severe depression, DM, active PUD, cataracts, osteoporosis, recent bowel anastomosis, pregnancy
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May unmask hypertension or gestational diabetes; frequently monitor blood sugar, blood pressure, and weight; at typically doses, amount secreted in breast milk is minimal and poses no significant risk to neonate; abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur
Use lower dose in hypothyroidism, liver disease, obesity (decrease cortisol-binding globulin and increase free fraction of steroid
Pregnancy, hyperthyroidism, and concurrent estrogen therapy may increase cortisol-binding globulin
Antihistamines
Antihistamines prevent histamine response in sensory nerve endings and blood vessels. They are more effective in preventing histamine response than in reversing it.
Diphenhydramine (Benadryl, Belix)
For symptomatic control of severe unremitting pruritus not controlled with previous therapeutic regimens.
Adult
25-50 mg PO bid/qid
Pediatric
12.5-25 mg PO tid/qid; alternatively, 5 mg/kg/d or 150 mg/m2/d divided tid/qid; not to exceed 300 mg/d
5 mg/kg/d IV/IM or 150 mg/m2/d divided qid; not to exceed 300 mg/d
Potentiates effect of CNS depressants; do not give syrup with medications that can cause disulfiramlike reactions (due to alcohol content)
Documented hypersensitivity; MAOI use; cardiovascular compromise in neonates
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May exacerbate angle-closure glaucoma, hyperthyroidism, peptic ulcer, and urinary tract obstruction; may decrease milk production secondary to anticholinergic effect of antihistamines and decrease cardiovascular response to stress in breastfeeding neonates
More on Pemphigoid Gestationis |
| Overview: Pemphigoid Gestationis |
| Differential Diagnoses & Workup: Pemphigoid Gestationis |
 Treatment & Medication: Pemphigoid Gestationis |
| Follow-up: Pemphigoid Gestationis |
| Multimedia: Pemphigoid Gestationis |
| References |
| « Previous Page | Next Page » |
References
Chimanovitch I, Schmidt E, Messer G, Dopp R, Partscht K, Brocker EB, et al. IgG1 and IgG3 are the major immunoglobulin subclasses targeting epitopes within the NC16A domain of BP180 in pemphigoid gestationis. J Invest Dermatol. Jul 1999;113(1):140-2. [Medline].
Jenkins RE, Hern S, Black MM. Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol. Jul 1999;24(4):255-9. [Medline].
Sitaru C, Powell J, Messer G, Brocker EB, Wojnarowska F, Zillikens D. Immunoblotting and enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis. Obstet Gynecol. Apr 2004;103(4):757-63. [Medline].
Amato L, Mei S, Gallerani I, Moretti S, Fabbri P. A case of chronic herpes gestationis: persistent disease or conversion to bullous pemphigoid?. J Am Acad Dermatol. Aug 2003;49(2):302-7. [Medline].
Ambros-Rudolph CM, Mullegger RR, Vaughan-Jones SA, Kerl H, Black MM. The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients. J Am Acad Dermatol. Mar 2006;54(3):395-404. [Medline].
Bedocs PM, Kumar V, Mahon MJ. Pemphigoid gestationis: a rare case and review. Arch Gynecol Obstet. May 28 2008;[Medline].
Boulinguez S, Bedane C, Prost C, Bernard P, Labbe L, Bonnetblanc JM. Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients. Dermatology. 2003;206(2):113-9. [Medline].
Fabbri P, Caproni M, Berti S, Bianchi B, Amato L, De Pita O, et al. The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis. Br J Dermatol. Jun 2003;148(6):1141-8. [Medline].
Kreuter A, Harati A, Breuckmann F, Appelhans C, Altmeyer P. Intravenous immune globulin in the treatment of persistent pemphigoid gestationis. J Am Acad Dermatol. Dec 2004;51(6):1027-8. [Medline].
Kroumpouzos G, Cohen LM. Dermatoses of pregnancy. J Am Acad Dermatol. Jul 2001;45(1):1-19; quiz 19-22. [Medline].
Lin MS, Arteaga LA, Diaz LA. Herpes gestationis. Clin Dermatol. Nov-Dec 2001;19(6):697-702. [Medline].
Lin MS, Gharia MA, Swartz SJ, Diaz LA, Giudice GJ. Identification and characterization of epitopes recognized by T lymphocytes and autoantibodies from patients with herpes gestationis. J Immunol. Apr 15 1999;162(8):4991-7. [Medline].
Powell AM, Sakuma-Oyama Y, Oyama N, Albert S, Bhogal B, Kaneko F, et al. Usefulness of BP180 NC16a enzyme-linked immunosorbent assay in the serodiagnosis of pemphigoid gestationis and in differentiating between pemphigoid gestationis and pruritic urticarial papules and plaques of pregnancy. Arch Dermatol. 2005;141:705-10. [Medline].
Satoh S, Seishima M, Sawada Y, Izumi T, Yoneda K, Kitajima Y. The time course of the change in antibody titres in herpes gestationis. Br J Dermatol. Jan 1999;140(1):119-23. [Medline].
Shimanovich I, Brocker EB, Zillikens D. Pemphigoid gestationis: new insights into the pathogenesis lead to novel diagnostic tools. BJOG. Sep 2002;109(9):970-6. [Medline].
Tunzi M, Gray GR. Common skin conditions during pregnancy. Am Fam Physician. Jan 15 2007;75(2):211-8. [Medline].
Further Reading
Keywords
herpes gestationis, PG, autoimmune dermatosis of pregnancy, pregnancy-associated autoimmune disease, bullous pemphigoid antigen 2, BPAG2, herpetiform blisters, herpes gestationis factor, pruritic urticarial papules, pruritic urticarial blisters
