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Effectiveness of Intravenous Immunoglobulin (IVIG) Therapy in Dermatology


Intravenous Immune Globulin (IVIG) is a new rising star in dermatalogical therapeutics. Most of IVIG applications are "off-label"(non-FDA approved) indications due to the lack of randomised controlled studies for most dermatologic entities. The common agreement is that it should be used as a second-line treatment. It is recommended:
  • When conventional treatments are unsuccessful and symptoms continue to worsen.
  • When there are critical side effects developed from standard therapies.
  • Contraindications to the extensive use of immunosuppresive drugs.

Indications of IVIG Use in Dermatology

In dermatology, there is a broad indication for IVIG use. This includes:

  • Graft vs Host Disease: This is an FDA-approved indication, but is not as widely used as immunosuppressive drugs.[1]
  • Kawasaki Syndrome: This is another FDA-approved indication, IVIG is the first line treatment for this disease.
  • Dermatomyositis, scleroderma and overlap connective tissue diseases: IVIG can be used as adjuvant therapy or a second-line treatment agent after the failure of an immunosuppressive drug.[2,3]
  • Leukocytoclastic vasculitis (Both systemic and cutaneous): IVIG can be used as adjuvant therapy or as a second line therapy.[4]
  • Scleromyxedema and chronic autoimmune urticaria: Case reports of its use in recalcitrant cases.[5,6]
  • The bulk of the dermatologic diseases in which IVIG has been used are the immunobullous dermatosis like bullous pemphigoid, pemphigus valgaris, and pemphigus foliaceus[7-14]. Rapid remission and rapid tapering off steroids with the use of IVIG has been reported. In general, this treatment appears to have greater efficacy as an adjunctive therapy than monotherapy.[4]
  • Severe drug reactions including toxic epidermal necrolysis, erythema multiforme, and Stevens- Johnson syndrome. Many studies have appeared supporting the use of IVIG as first line agent for TEN and SJS. [15]

Further Readings

Effectiveness of Intravenous Immune Globulin (IVIG) Therapy in Treatment of Dermatomyositis >>>
Effectiveness of Intravenous Immune Globulin (IVIG) Therapy in Treatment of Pemphigus >>>

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References

1. Winston DJ, Antin JH, Wolff SN, Bierer BE, Small T, Miller KB, Linker C, Kaizer H, Lazarus HM, Petersen FB, Cowan MJ, Ho WG, Wingard JR, Schiller GJ, Territo MC, Jiao J, Petrarca MA, Tonetta SA. Multicenter, randomized, double-blind comparison of different doses of intravenous immunoglobulin for prevention of graft-versus-host disease and infection after allogencic bone marrow transplantation. Bone Marrow Transplant. 28:187-96,2001.
2. 1228-1231. Hamacher KL, el-Azhary RA, Pittelkow MR, Davis.MD: Severe Recalcitrant and Ulcerative Mixed. Connective Tissue Disease responding to. Intravenous Immunoglobulin (IVIG) therapy. Soc. Investlg Derm (abst.), 2003
3. Al-Mayouf SM, Laxer RM, Schneider R, Silverman ED, Feldman BM: Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety. J Rheumatol, 27:2498-503.2000
4. Ahmed AR, Spigelman Z, Cavacini LA, et al. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med. 2006; 355(17):1772-9.
5. Kulczycki A, Nelson M, Eisen A, Hefferman M: Scleromyxedema: treatment of cutaneous and systemic manifestations with high-dose intravenous immunoglobulin. Br J Dermatol 149:1276-1281, 2003
6. Autoimmune urticaria response to high-dose intravenous immunoglobulin. Ann Allergy Asthma Immunol94:307-8, 2005. 7. Ahmed AR,; Sami N . Intravenous immunoglobulin therapy for patients with pemphigus vulgaris unresponsive to immunosuppressive treatment. J Am Acad Dermatol. 45:679-690, 2001.
8. Sami N, Bhol KC, Ahmed RA. Influence of intravenous immunoglobulin therapy on autoantibody titers to desmoglein 3 and desmoglein 1 in pemphigus vulgaris. Eur J Dermatol 13:377-381,2003.
9. Sami N, Qureshi A, Ahmed AR. Steroid sparing effect of intravenous immunoglobulin therapy in patients with pemphigus foliaceus. Eur J Dermatol 12:174-178,2002.
10. Engineer, L, Bhol, KC, Ahmed, AR: Analysis of current data on the use of intravenous immunoglobulin in management of pemphigus vulagaris. J M Acad Dermatol 43:1049-1057, 2000.
11. Goebeler M, Seitz C, Rose C, Sitaru C, Jeschke R, Marx A, Bröcker EB, Zillikens D: Successful treatment of linear IgA disease with salazosulphapyridine and intravenous immunoglobulins. Br J Dermatol 149:912-914, 2003.
12. Bystryn JC, Jiao D, Natow S. Treatment of pemphigus with intravenous immunoglobulin. J Am Acad Dermatol 47(3):358-363, 2002.
13. Ahmed AR,; Sami N . Intravenous immunoglobulin therapy for patients with pemphigus foliaceus unresponsive to conventional therapy. J Am Acad Dermatol 46:42-49, 2002.
14. Jolles S: High-dose intravenous immunoglobulin (hdIVIg) in the treatment of autoimmune blistering disorders. Clin Exp Immunol 129:385-389, 2002.
15. Rokea el-Azhary, PhD, M.D: Intravenous Immunoglobulin in Dermatology. Mayo Clinic and Mayo Foundation. Rochester, Minnesota.

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