Abstract
Systemic lupus erythematosus (SLE) is a disease of unknown cause that may involve one or many organ or systems. Skin involvement is a major feature in this disease, and a wide variety of skin conditions may be present. Lupus erythematosus panniculitis (LEP) constitutes a rare form of cutaneous lupus characterized by recurrent nodular or plaque lesions that can vary from a benign and mild course to a more disfiguring disease. Initial therapy includes corticosteroids, antimalarials, and azathioprine and, in refractory cases, two antimalarials in association, mycophenolate mofetil, or other immunomodulators. Intravenous immuglobulin (IVIG) is used in many autoimmune disorders, like in SLE, although clinical trials have not yet taken place. In this report, we review skin manifestations of SLE and their treatment, IVIG, and finally a case of LEP successfully treated with IVIG when other therapy modalities failed.
Similar content being viewed by others
References
Tan EM et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277
Ullrich SE (2000) Sun exposure and the lupus patient. Lupus Awareness 22:6–7
Vila LM, Mayor AM, Valentin AH et al (1999) Association of sunlight exposure and photoprotection measures with clinical outcome in systemic lupus erythematosus. Puerto Rico Health Sci J 18:89–94
Doria A et al (1996) Photosensitivity in systemic lupus erythematosus: laboratory testing of ARA/ACR definition. Lupus 5:263–268
Hochberg MC et al (1985) Systemic lupus erythematosus: a review of clinico-laboratory features and immunogenetic markers in 150 patients with emphasis on demographic subsets. Medicine 64:285–295
Mikle MF, Barton EN, Morgan OS, Deceulaer K (1996) Photosensitivity and antinuclear antibodies in black patients with systemic lupus erythematosus. J Assoc Acad Minor Phys 7:53–55
Sontheimer RD et al (1982) Serologic and HLA associations in subacute cutaneous lupus erythematosus, a clinical subset of lupus erythematosus. Ann Intern Med 97:664–671
Callen JP, Klein J (1988) Subacute cutaneous lupus erythematosus. Clinical, serologic, immunogenetic, and therapeutic considerations in seventy two patients. Arthritis Rheum 31:1007–1013
Herrero C et al (1988) Subacute cutaneous lupus erythematosus: clinicopathologic findings in thirteen cases. J Am Acad Dermatol 19:1057–1062
Sontheimer RD, Provost TT (1996) Lupus erythematosus. In: Sontheimer RD, Provost TT (eds) Cutaneous manifestations of rheumatic diseases. Williams and Wilkins, Baltimore, pp 1–71
Tuffanelli DL (1971) Lupus erythematosus panniculitis (profundus). Arch Dermatol 103:231–242
Provost TT (1979) Lupus band test. In: Beutner EH, Chorzelski TP, Bean SF (eds) Immunopathology of the skin, vol 22. Wiley, New York, pp 399–410
Burnham TK, Fine G (1971) The immunofluorescent “band” test for lupus erythematosus. III. Employing clinically normal skin. Arch Derm 103:24–32
Levy Y, Sherer Y, Ahmed A, Langevitz P, George J, Fabbrizzi F, Terryberry J, Meissner M, Lorber M, Peter JB, Shoenfeld Y (1999) A study of 20 SLE patients with intravenous immunoglobulin clinical and serologic response. Lupus 8:705
Strober BE (2001) Lupus panniculitis (lupus profundus). Dermatology Online Journal 7(2):20 New York University, Department of Dermatology
Wimmershoff MB, Hohenleutner U, Landthaler M (2003) Discoid lupus erythematosus and lupus profundus in childhood: a report of two cases. Pediatr Dermatol 20:140–145
Bacanli A, Uzun S, Ciftcioglu MA, Alpsoy E (2005) A case of lupus erythematosus profundus with unusual manifestations. Lupus 14:403
Chen MT, Chen KS, Chen MJ et al (1999) Lupus profundus (panniculitis) in a chronic haemodialysis patient. Nephrol Dial Transplant 14:966–968
Martens PB, Moder KG, Ahmed I (1999) Lupus panniculitis: clinical perspectives from a case series. J Rheumatol 26:68–72
Kundig TM, Trueb RM, Krasovec M (1997) Lupus profundus=panniculitis. Dermatology 195:99–101
Watanabe T, Tsuchida T (1996) Lupus erythematosus profundus: a cutaneous marker for a distinct clinical subset? Br J Dermatol 134:123–125
Ng PP, Tan SH, Tan T (2002) Lupus erythematosus panniculitis: a clinicopathologic study. Int J Dermatol 41:488–490
Grossberg E, Scherschun L, Fiven DP (2001) Lupus profundus: not a benign disease. Lupus 10:514
Cernea SS, Kihara SM, Sotto MN et al (1993) Lupus mastitis. J Am Acad Dermatol 29:343–346
Holland NW, McKnight K, Challa VR et al (1995) Lupus panniculitis (profundus) involving the breast: report of 2 cases and review of the literature. J Rheumatol 22:344–346
White WL, Sherertz EF, Berg D et al (1993) Periparotid lupus erythematosus panniculitis: clinicopathologic correlation of two cases presenting as primary parotid disease. Arch Pathol Lab Med 117:535–539
Ogura N, Fujisaku A, Jodo S et al (1997) Lupus erythematosus profundus around the salivary glands: a case resembling submandibular gland disease. Lupus 6:477–479
Peters MS, Su WP (1989) Lupus erythematosus panniculitis. Med Clin North Am 73:1113–1126
Laman SD, Provost TT (1994) Cutaneous manifestations of lupus erythematosus. Rheum Dis Clin North Am 13:641–644
Tada J, Arata J, Katayama H (1991) Linear lupus erythematosus profundus in a child. J Am Acad Dermatol 24:871–874
Heid E (1998) A 17-year old Italian boy with a linear lupus erythematosus profundus. Eur J Dermatol 8:69
Stork J, Vosmik F (1994) Lupus erythematosus panniculitis with morphea-like lesions. Clin Exp Dermatol 19:79–82
Hytiroglou P, Phelps RG, Wattenberg DJ, Strauchen JA (1992) Cytophagic histiocytic panniculitis; molecular evidence for a clonal T cell disorder. J Am Acad Dermatol 27:333–336
Saeki Y, Ohshima S, Kurimoto I, Miura H, Suemura M (2000) Maintaining remission of lupus erythematosus profundus (LEP) with cyclosporin A. Lupus 9:390
Sanchez NP, Peters MS, Winkelmann RK (1981) The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol 5:673–680
Lonceint J, Sassolas B, Lefur JM, Guillet G, Leroy JP (2001) Panniculitis and macrophage activation syndrome in a child with lupus erythematosus. Ann Dermatol Venereol 128:1339–1342 in French
D'Cruz D (2001) Antimalarial therapy: a panacea for mild lupus? Lupus 10:148
Petri M (1996) Hydroxychloroquine use in the Baltimore Lupus Cohort: effects on lipids, glucose and thrombosis. Lupus 5(Suppl 1):S16–S22
Khamashta MA, Wallace DJ (eds) (1996) Antimalarials in rheumatology. Lupus 5(Suppl 1):S1–S73.
Easterbrook M (1990) Is corneal deposition of antimalarial any indication of retinal toxicity? Can J Ophthalmol 25:249–251
Mavrikakis M, Papazoglou S, Sifkakis PP, Vaiopoulos G, Rougas K (1996) Retinal toxicity in long term hydroxychloroquine treatment. Ann Rheum Dis 55:187–189
Bienfang D, Coblyn JS, Liang MH, Corzillius M (2000) Hydroxychloroquine retinopathy despite regular ophthalmologic evaluation: a consecutive series. J Rheumatol 27:2703–2706
Yamada Y, Dekio S, Jidoi J, Ozasa S (1989) Lupus erythematosus profundus: report of a case treated with dapsone. J Dermatol 16:379–382
Adu D, Cross J, Jayne DR (2001) Treatment of systemic lupus erythematosus with mycophenolate mofetil. Lupus 10:203–208
Gaubitz M, Schorat A, Schotte H, Kern P, Domschke W (1999) Mycophenolate mofetil for the treatment of systemic lupus erythematosus: an open pilot trial. Lupus 8:731–736
Schanz S, Ulmer A, Rassner G, Fierlbeck G (2002) Successful treatment of subacute cutaneous lupus erythematosus with mycophenolate mofetil. Br J Dermatol 147:174–178
Goyal S, Nousari HC (2001) Treatment of resistant discoid lupus erythematosus of the palms and soles with mycophenolate mofetil. J Am Acad Dermatol 45:142–144
Boehm I, Bieber T (2001) Chilblain lupus erythematosus Hutchinson: successful treatment with mycophenolate mofetil. Arch Dermatol 137:235–236
Knezevic-Maramica I, Kruskall MS (2003) Intravenous immune globulins: an update for clinicians. Transfusion 43:1460–1480
Kazatchkine MD, Morell A (eds) (1996) Intravenous immunoglobulin research and therapy. Parthenon, New York
Imbach P, Barandun S, d’Apuzzo V et al (1981) High-dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood. Lancet 1:1228–1231
Shoenfeld Y, Krause I (1996) Immunosuppression and immunomodulation of experimental models of systemic lupus erythematosus and antiphospholipid syndrome. Transplant Proc 28:3096–3098
Darabi K, Abdel-Wahab O, Dzik WH (2006) Current usage of intravenous immune globulin and the rationale behind it: the Massachusetts General Hospital data and a review of the literature. Transfusion 46:741
Onouchi Z, Yanagisawa M, Hirayama T et al (1995) Optimal dosage and differences in therapeutic efficacy of IVIg in Kawasaki disease. Acta Paediatr Jpn 37:40–46
Samuelsson A, Towers TL, Ravetch JV (2001) Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor. Science 291:484
Fehr J, Hoffman V, Kappeler U (1982) Transient reversal of thrombocytopenia in idiopathic thrombocytopenic purpura by high-dose intravenous gamma globulin. N Engl J Med 306:1254
Tsubakio T, Kurata Y, Katagiri S et al (1983) Alteration of T cell subsets and immunoglobulin synthesis in vitro during high dose gamma globulin therapy in patients with idiopathic thrombocytopenic purpura. Clin Exp Immunol 53:697
Dietrich G, Kaveri SV, Kazatchkine MD (1992) Modulation of autoimmunity by intravenous immune globulin through interaction with the function of the immune/idiotypic network. Clin Immunol Immunopathol 62:S73
Kaveri SV, Dietrich G, Hurez V, Kazatchkine MD (1991) Intravenous immunoglobulins (IVIG) in the treatment of autoimmune diseases. Clin Exp Immunol 86:192
Jayne DR, Davies MJ, Fox CJ et al (1991) Treatment of systemic vasculitis with pooled intravenous immunoglobulin. Lancet 337:1137
Palla R, Cirami C, Panichi V et al (1991) Intravenous immunoglobulin therapy of membranous nephropathy: Efficacy and safety. Clin Nephrol 35:98
Ashkenazi S, Cleary TG, Lopez E, Pickering LK (1988) Anticytotoxin neutralizing antibodies in immune globulin preparations: potential use in hemolytic–uremic syndrome. J Pediatr 113:1008
Bleeker WK, Teeling JL, Hack CE (2001) Accelerated autoantibody clearance by intravenous immunoglobulin therapy: studies in experimental models to determine the magnitude and time course of the effect. Blood 98:3136
Basta M, Van Goor F, Luccioli S et al (2003) F(ab)(2)-mediated neutralization of C3a and C5a anaphylatoxins: a novel effector function of immunoglobulins. Nat Med 9:431
Sherer Y, Levy Y, Langevitz P, Rauova L, Fabrizzi F, Shoenfeld Y (2001) Adverse effects of intravenous immunoglobulin therapy in 56 patients with autoimmune diseases. Pharmacolgy 62:133–137
Rauova L, Lukac J, Levy Y, Rovensky J, Shoenfeld Y (2001) High-dose intravenous immunoglobulins for lupus nephritis—a salvage immunomodulation. Lupus 10:209–213
Orbach H, Tishler M, Shoenfeld Y (2004) Intravenous immunoglobulin and the kidney—a two-edged sword. Semin Arthritis Rheum 34:593–601
Pasatiempo AMG, Kroser JA, Rudnick M, Hoffmnann BI (1994) Acute renal failure after intravenous immunoglobulin therapy. J Rheumatol 21:347–349
Jordan SC (1989) Intravenous y-globulin therapy in systemic lupus erythematosus and immune complex disease. Ctin Immunol Immunopathol 53:164–169
Hashkes PJ, Lovell DJ (1996) Vasculitis in systemic lupus erythematosus following intravenous immunoglobulin therapy. Clin Exp Rheumatol 14:673–675
Reinhart WH, Berchtold PE (1992) Effect of high-dose intravenous immunoglobulin therapy on blood rheology. Lancet 339:662–644
Dalakas MC (1994) High-dose intravenous immunoglobulin and serum viscosity: risk of precipitating thromboembolic events. Neurology 44:223–226
Nishikawa M, Ichiyama T, Hasegawa M, Kawasaki K, Matsubara T, Furukawa S (2003) Safety from thromboembolism using intravenous immunoglobulin therapy in Kawasaki disease: study of whole-blood viscosity. Pediatr Int 45:156–158
Woodruff RK, Grigg AP, Firkin FC, Smith IL (1986) Fatal thrombotic events during treatment of autoimmune thrombocytopenia with intravenous immunoglobulin in elderly patients. Lancet 2:217–218
Voltz R, Rosen FV, Yousry T, Beck J, Hohlfeld R (1996) Reversible encephalopathy with cerebral vasospasm in a Guillain–Barre syndrome patient treated with intravenous immunoglobulin. Neurology 46:250–251
Sztajzel R, Le Floch-Rohr J, Eggimann P (1999) High-dose intravenous immunoglobulin treatment and cerebral vasospasm: a possible mechanism of ischemic encephalopathy? Eur Neurol 41:153–158
Katz U, Shoenfeld Y (2005) Review: intravenous immunoglobulin therapy and thromboembolic complications. Lupus 14:802
Go RS, Call TG (2000) Deep venous thrombosis of the arm after intravenous immunoglobulin infusion: case report and literature review of intravenous immunoglobulin-related thrombotic complications. Mayo Clin Proc 75:83–85
Turner B, Wills AJ (2000) Cerebral infarction complicating intravenous immunoglobulin therapy in a patient with Miller Fisher syndrome. J Neurol Neurosurg Psychiatry 68:790–791
Shoenfeld Y, Krause I (2004) IVIG for autoimmune, fibrosis, and malignant conditions: our experience with 200 patients. J Clin Immunol 24:107–114
Sherer Y, Wu R, Krause I, Peter JB, Shoenfeld Y (2001) Antiphospholipid antibody levels in intravenous immunoglobulin (IVIg) preparations. Lupus 10:568. doi:10.1191/096120301701549705
Krause I, Blank M, Shoenfeld Y (1998) Anti-DNA and antiphospholipid antibodies in IVIg preparations: in vivo study in naive mice. J Clin Immunol 18:52–60
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Espírito Santo, J., Gomes, M.F., Gomes, M.J. et al. Intravenous Immunoglobulin in Lupus Panniculitis. Clinic Rev Allerg Immunol 38, 307–318 (2010). https://doi.org/10.1007/s12016-009-8162-x
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12016-009-8162-x