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Combination Oral Prednisone and Intravenous Immunoglobulin in the Treatment of Scleromyxedema

  • Basic/Clinical Science
  • Published:
Journal of Cutaneous Medicine and Surgery

Abstract

Background

Scleromyxedema is a clinical variant of the rare disease papular mucinosis that has both cutaneous and systemic manifestations. Treatment options are numerous and tend to be associated with serious potential side effects and frequent relapse.

Objective

We report a case of scleromyxedema treated with low-dose oral prednisone and intravenous immunoglobulin (IVIg). This is followed by a review of the literature.

Conclusion

IVIg is being used for a growing number of inflammatory and immune disorders. It is being increasingly reported as a successful treatment for scleromyxedema. Although our patient succumbed to the disease, combination therapy with prednisone and IVIg provided temporary symptomatic, laboratory, and clinical improvement of the condition. Optimization of this therapeutic strategy is thus indicated for the management of scleromyxedema.

Sommaire Antè cédents

Le scléromyxoedème est une variante clinique de la maladie rare mucinose papuleuse dont les manifestations sont à la fois cutanées et systémiques. Les options de traitement sont nombreuses et souvent associées é des effets secondaires graves et à des récidives fréquentes.

Objectif

Nous rapportons un cas de scléromyxoedème traité avec de faibles doses de prednisone oral et d’immunoglobuline intraveineuse. Par la suite, nous passons en revue les publications scientifiques.

Conclusion

L’immunoglobuline intraveineuse est utilisée dans le traitement d’un. nombre de plus en plus élevé de maladies inflammatoires et auto-immunes. Les rapports sont de plus en plus fréquents sur son efficacité dans les cas de scléromyxoedème. Bien que patiente n’ait pas survécu à sa maladie, la polythérapie au prednisone et àl’immunoglobuline intraveineuse a permis une amélioration temporaire au niveau des symptômes ainsi que des résultats cliniques et de laboratoire. Ainsi, l’optimisation de cette thérapie est recommandée dans la gestion du scléromyxoedème.

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References

  1. HA Gottron (1954) ArticleTitleSkleromyxodem (Eine eigenartige Erscheinungsform von Myxothesaurodermie) Arch Dermatol Syphilol 199 71 Occurrence Handle1:STN:280:DyaG2M%2FktFWntg%3D%3D Occurrence Handle10.1007/BF00362184

    Article  CAS  Google Scholar 

  2. W Dubreuilh (1906) ArticleTitleFibromes miliaires folliculaires: sclerodermie consecutive Ann Dermatol Syph 37 569–572

    Google Scholar 

  3. K Reitmann (1908) ArticleTitleUber eine eigenartige, der sklerodermie nahestehende affektion Arch Dermatol Syph 92 417–424 Occurrence Handle10.1007/BF01949029

    Article  Google Scholar 

  4. S Caradonna H Jacobe (2004) ArticleTitleThalidomide as a potential treatment for scleromyxedema Arch Dermatol 140 277–280 Occurrence Handle10.1001/archderm.140.3.277 Occurrence Handle15023770

    Article  PubMed  Google Scholar 

  5. JJ Pomann EJ Rudner (2003) ArticleTitleScleromyxedema revisited Int J Dermatol 42 31–35 Occurrence Handle10.1046/j.1365-4362.2003.01565.x Occurrence Handle12581140

    Article  PubMed  Google Scholar 

  6. AM Dinneen CH Dicken (1995) ArticleTitleScleromyxedema J Am Acad Dermatol 33 37–43 Occurrence Handle10.1016/0190-9622(95)90007-1 Occurrence Handle1:STN:280:DyaK2MzisFChuw%3D%3D Occurrence Handle7601944

    Article  CAS  PubMed  Google Scholar 

  7. RK Lister S Jolles S Whittaker et al. (2000) ArticleTitleScleromyxedema: Response to high-dose intravenous immunoglobulin (hdIVIg) J Am Acad Dermatol 43 403–408 Occurrence Handle1:STN:280:DC%2BD3cvgsVyquw%3D%3D Occurrence Handle10901735 Occurrence Handle10.1016/S0190-9622(00)70305-7

    Article  CAS  PubMed  Google Scholar 

  8. A Karim MM Black F Lawlor (2003) ArticleTitleCase 5: extensive skin eruption causing skin to become tight, sore, and itchy. Diagnosis: scleromyxoedema Clin Exp Dermatol 28 343–344 Occurrence Handle1:STN:280:DC%2BD3s3ls1Cqsw%3D%3D Occurrence Handle12780738 Occurrence Handle10.1046/j.1365-2230.2003.01289.x

    Article  CAS  PubMed  Google Scholar 

  9. M Penmetcha AS Highet JM Hopkinson (1987) ArticleTitleFailure of PUVA in lichen myxedematosus: acceleration of associated multiple keratoacanthomas with development of squamous cell carcinoma Clin Exp Dermatol 12 220–223 Occurrence Handle1:STN:280:DyaL1c%2FotFSmtg%3D%3D Occurrence Handle3690891 Occurrence Handle10.1111/j.1365-2230.1987.tb01902.x

    Article  CAS  PubMed  Google Scholar 

  10. RA Harper J Rispler (1978) ArticleTitleLichen myxedematous serum stimulates human skin fibroblast proliferation science 188 545 Occurrence Handle10.1126/science.622555

    Article  Google Scholar 

  11. M Yaron I Yaron I Yust et al. (1985) ArticleTitleLichen myxedematosus (scleromyxedema) serum stimulates hyaluronic acid and prostaglandin E production by human fibroblasts J Rheumatol 12 171–175 Occurrence Handle1:STN:280:DyaL2M7mtVGqtA%3D%3D Occurrence Handle3981505

    CAS  PubMed  Google Scholar 

  12. SM Howsden JH Herndon RG Freeman (1975) ArticleTitleLichen myxedematosus: Dermal infiltrative disorder response to cyclophosphamide therapy Arch Dermatol 111 1325–1330 Occurrence Handle10.1001/archderm.111.10.1325 Occurrence Handle1:STN:280:DyaE28%2FmtVCltQ%3D%3D Occurrence Handle53033

    Article  CAS  PubMed  Google Scholar 

  13. A Lai RFM Fat D Suurmond et al. (1973) ArticleTitleScleromyxedema (lichen myxedematous) associated with a paraprotein IgG of type Kappa Br J Dermatol 88 107–116

    Google Scholar 

  14. S Jablonska (Eds) (1975) Scleroderma and Pseudosclerderma EditionNumber2 Polish Medical Publishers Warsaw 407–411

    Google Scholar 

  15. R Fleischmajer (2003) Papular mucinosis IM Freedberg AZ Eisen K Wolff (Eds) Fitzpatrick’s Dermatology in General Medicine EditionNumber6 McGraw–Hill New York 1803–1805

    Google Scholar 

  16. A Kulczycki M Nelson A Eisen et al. (2003) ArticleTitleScleromyxoedema: treatment of cutaneous and systemic manifestations with high-dose intravenous immunoglobulin Br J Dermatol 149 1276–1281 Occurrence Handle10.1111/j.1365-2133.2003.05682.x Occurrence Handle1:STN:280:DC%2BD3srptlykug%3D%3D Occurrence Handle14674909

    Article  CAS  PubMed  Google Scholar 

  17. S Brenner I. Yust (1984) ArticleTitleTreatment of scleromyxedema with etretinate J Am Acad Dermatol 10 295–296 Occurrence Handle1:STN:280:DyaL2c7os1aksQ%3D%3D Occurrence Handle6715602 Occurrence Handle10.1016/S0190-9622(84)80230-3

    Article  CAS  PubMed  Google Scholar 

  18. CP Milam LE Cohen NA Fenske et al. (1988) ArticleTitleScleromyxedema: therapeutic response to isotretinoin in three patients J Am Acad Dermatol 19 469–477 Occurrence Handle1:STN:280:DyaL1M%2FgvFSiug%3D%3D Occurrence Handle3170809 Occurrence Handle10.1016/S0190-9622(88)70199-1

    Article  CAS  PubMed  Google Scholar 

  19. BM Hisler LB Savoy K Hashimoto (1988) ArticleTitleImprovement of scleromyxedema associated with isotretinoin therapy J Am Acad Dermatol 19 854–857

    Google Scholar 

  20. KB Horn MA Horn J Swan et al. (2004) ArticleTitleA complete and durable clinical response to high-dose dexamethasone in a patient with Scleromyxedema J Am Acad Dermatol 51 S120–S123 Occurrence Handle10.1016/j.jaad.2004.03.023 Occurrence Handle15280830

    Article  PubMed  Google Scholar 

  21. .

  22. JA Tschen JR Chang (1999) ArticleTitleScleromyxedema: treatment with interferon alfa J Am Acad Dermatol 40 303–307 Occurrence Handle10.1016/S0190-9622(99)70471-8 Occurrence Handle1:STN:280:DyaK1M7kvVOhsw%3D%3D Occurrence Handle10025854

    Article  CAS  PubMed  Google Scholar 

  23. LS Davis S Sanal OP Sangueza (1996) ArticleTitleTreatment of Scleromyxedema with 2-chlorodeoxyadenosine J Am Acad Dermatol 35 288–290 Occurrence Handle1:STN:280:DyaK28zhvFGntw%3D%3D Occurrence Handle8698907 Occurrence Handle10.1016/S0190-9622(96)90650-7

    Article  CAS  PubMed  Google Scholar 

  24. AM Cohen E Hodar M David et al. (1996) ArticleTitleBeneficial effect of granulocyte-colony stimulating factor in Scleromyxedema associated with severe idiopathic neutropenia Br J Dermatol 135 626–629 Occurrence Handle10.1111/j.1365-2133.1996.tb03846.x Occurrence Handle1:STN:280:DyaK2s%2Fnt1eqtw%3D%3D Occurrence Handle8915161

    Article  CAS  PubMed  Google Scholar 

  25. AW MacFarlane A Davenport JL Verbov et al. (1987) ArticleTitleScleromyxoedema: successful treatment with plasma exchange and immunosuppression Br J Dermatol 117 653–657 Occurrence Handle1:STN:280:DyaL1c%2FnslOjtg%3D%3D Occurrence Handle3689684 Occurrence Handle10.1111/j.1365-2133.1987.tb07499.x

    Article  CAS  PubMed  Google Scholar 

  26. AI Westheim DP Lookingbill (1987) ArticleTitlePlasmapheresis in a patient with Scleromyxedema Arch Dermatol 123 786–789 Occurrence Handle10.1001/archderm.123.6.786 Occurrence Handle1:STN:280:DyaL2s3ht1Whug%3D%3D Occurrence Handle3107482

    Article  CAS  PubMed  Google Scholar 

  27. D Kaufman AP Truha HH Roenigk SuffixJr (1987) ArticleTitleScleromyxedema: systemic manifestations and cosmetic improvement with dermabrasion Cutis 39 321–324 Occurrence Handle1:STN:280:DyaL2s3hslCruw%3D%3D Occurrence Handle3581903

    CAS  PubMed  Google Scholar 

  28. NJ Lowe PA Dufton RD Hunter et al. (1982) ArticleTitleElectron-beam treatment of scleromyxoedema Br J Dermatol 106 449–453 Occurrence Handle1:STN:280:DyaL387ot1Oitw%3D%3D Occurrence Handle6803824 Occurrence Handle10.1111/j.1365-2133.1982.tb04539.x

    Article  CAS  PubMed  Google Scholar 

  29. M Berkson GS Lazarus M Uberti–Benz et al. (1991) ArticleTitleExtracorporeal photochemotherapy: a potentially useful treatment for Scleromyxedema J Am Acad Dermatol 25 724 Occurrence Handle1:STN:280:DyaK387ms1SnsA%3D%3D Occurrence Handle1791229 Occurrence Handle10.1016/S0190-9622(08)80676-7

    Article  CAS  PubMed  Google Scholar 

  30. PM Farr FA Ive (1984) ArticleTitlePUVA- treatment of scleromyxoedema Br J Dermatol 110 347–350 Occurrence Handle1:STN:280:DyaL2c7ivVClsA%3D%3D Occurrence Handle6696849 Occurrence Handle10.1111/j.1365-2133.1984.tb04642.x

    Article  CAS  PubMed  Google Scholar 

  31. P Feldman L Shapiro AI Pick et al. (1969) ArticleTitleScleromyxedema: a dramatic response to melphalan Arch Dermatol 99 51–56 Occurrence Handle10.1001/archderm.99.1.51 Occurrence Handle1:STN:280:DyaF1M%2Fns1WitA%3D%3D Occurrence Handle4178219

    Article  CAS  PubMed  Google Scholar 

  32. RB Harris HO Perry RA Kyle et al. (1979) ArticleTitleTreatment of scleromyxedema with melphalan Arch Dermatol 115 295–299 Occurrence Handle1:STN:280:DyaE1M7msVCntQ%3D%3D Occurrence Handle434843 Occurrence Handle10.1001/archderm.115.3.295

    Article  CAS  PubMed  Google Scholar 

  33. K Schubert A Stein M Meurer (2002) ArticleTitleInitial scleromyxedema. Successful treatment with melphalan Hautarzt 53 478–482 Occurrence Handle10.1007/s00105-001-0298-4 Occurrence Handle1:STN:280:DC%2BD38vlvVWltw%3D%3D Occurrence Handle12219271

    Article  CAS  PubMed  Google Scholar 

  34. RT Jessen M Straight LE Becker (1978) ArticleTitleLichen myxedematosus: treatment with cyclophosphamide Int J Dermatol 17 833–839 Occurrence Handle1:STN:280:DyaE1M%2FovVSmsQ%3D%3D Occurrence Handle730438 Occurrence Handle10.1111/j.1365-4362.1978.tb05989.x

    Article  CAS  PubMed  Google Scholar 

  35. I Krajnc (1997) ArticleTitleArndt–Gottron scleromyxedema: Summary of 2 years treatment Wien Klin Wochenschr 109 960–963 Occurrence Handle1:STN:280:DyaK1c7lt1ansg%3D%3D Occurrence Handle9491540

    CAS  PubMed  Google Scholar 

  36. Z Bata–Csorgo S Husz M Foldes et al. (1999) ArticleTitleScleromyxedema J Am Acad Dermatol 41 343–346 Occurrence Handle1:STN:280:DyaK1MzlsFyrsA%3D%3D Occurrence Handle10426928 Occurrence Handle10.1016/S0190-9622(99)70383-X

    Article  CAS  PubMed  Google Scholar 

  37. S Saigoh A Tashiro S Fujita et al. (2003) ArticleTitleSuccessful treatment of intractable scleromyxedema with cyclosporin A Dermatology 207 410–411 Occurrence Handle10.1159/000074127 Occurrence Handle1:STN:280:DC%2BD3sngtlOntQ%3D%3D Occurrence Handle14657639

    Article  CAS  PubMed  Google Scholar 

  38. MA Verity J Toop LP McAdam et al. (1978) ArticleTitleScleromyxedema myopathy: histochemical and electron microscopic observations Am J Clin Pathol 69 446–451 Occurrence Handle1:STN:280:DyaE1c7mtlaqtA%3D%3D Occurrence Handle645644

    CAS  PubMed  Google Scholar 

  39. LP McAdam CM Pearson WH Pitts et al. (1977) ArticleTitlePapular mucinosis with myopathy, arthritis, and eosinophilia: a histopathologic study Arthritis Rheum 20 989–996 Occurrence Handle1:STN:280:DyaE2s7otFKnsQ%3D%3D Occurrence Handle405019 Occurrence Handle10.1002/art.1780200412

    Article  CAS  PubMed  Google Scholar 

  40. JM Wieder KL Barton JM Baron et al. (1993) ArticleTitleLichen myxedematosus treated with chlorambucil J Dermatol Surg Oncol 19 475–476 Occurrence Handle1:STN:280:DyaK3s3ms12ktQ%3D%3D Occurrence Handle8496492

    CAS  PubMed  Google Scholar 

  41. D Rayson JA Lust A Duncan et al. (1999) ArticleTitleScleromyxedema: A complete response to prednisone Mayo Clin Proc 74 481–484 Occurrence Handle1:STN:280:DyaK1M3lt1Kgug%3D%3D Occurrence Handle10319080 Occurrence Handle10.4065/74.5.481

    Article  CAS  PubMed  Google Scholar 

  42. A Righi F Schiavon S Jablonska et al. (2002) ArticleTitleIntravenous immunoglobulins control scleromyxoedema (Concise report) Ann Rheum Dis 61 59–61 Occurrence Handle10.1136/ard.61.1.59 Occurrence Handle1:STN:280:DC%2BD38%2FltlGrsg%3D%3D Occurrence Handle11779761

    Article  CAS  PubMed  Google Scholar 

  43. A Karim F Lawlor MM Black (2004) ArticleTitleSuccessful treatment of scleromyxoedema with high-dose intravenous immunoglobulin Clin Exp Dermatol 29 317–318 Occurrence Handle10.1111/j.1365-2230.2004.01518.x Occurrence Handle1:STN:280:DC%2BD2c3hslKrsA%3D%3D Occurrence Handle15115524

    Article  CAS  PubMed  Google Scholar 

  44. WAC Sewell S Jolles (2002) ArticleTitleImmunomodulatory action of intravenous immunoglobulin Immunology 107 387–393 Occurrence Handle10.1046/j.1365-2567.2002.01545.x Occurrence Handle1:CAS:528:DC%2BD38Xps1yqt7k%3D Occurrence Handle12460182

    Article  CAS  PubMed  Google Scholar 

  45. C Ibanez JB Montoro–Rosano (2003) ArticleTitleIntravenous immunoglobulin preparations and autoimmune disorders: mechanism of action, Curr Pharm Biotechnol 4 239–247 Occurrence Handle10.2174/1389201033489775 Occurrence Handle1:CAS:528:DC%2BD3sXmt1Ghu78%3D Occurrence Handle14529426

    Article  CAS  PubMed  Google Scholar 

  46. HU Simon PJ Späth (2003) ArticleTitleIVIG — mechanisms of action Allergy 58 543–552 Occurrence Handle1:CAS:528:DC%2BD3sXmsFCnur4%3D Occurrence Handle12823109 Occurrence Handle10.1034/j.1398-9995.2003.00239.x

    Article  CAS  PubMed  Google Scholar 

  47. P Imbach A Morell (1989) ArticleTitleIdiopathic thrombocytopenic purpura (ITP): immunomodulation by intravenous immunoglobulin (IVIg) Int Rev Immunol 5 181–188 Occurrence Handle1:STN:280:DyaK283ptFCntQ%3D%3D Occurrence Handle8691051 Occurrence Handle10.3109/08830188909061985

    Article  CAS  PubMed  Google Scholar 

  48. JW Newburger M Takahashi JC Burns et al. (1986) ArticleTitleThe treatment of Kawasaki syndrome with intravenous gamma globulin N Engl J Med 315 341–347 Occurrence Handle1:STN:280:DyaL283osVyntQ%3D%3D Occurrence Handle2426590 Occurrence Handle10.1056/NEJM198608073150601

    Article  CAS  PubMed  Google Scholar 

  49. N Sato T Sugimura T Akagi et al. (1999) ArticleTitleSelective high dose gamma-globulin treatment in Kawasaki disease: assessment of clinical aspects and cost-effectiveness Pediatr Int 41 1–7 Occurrence Handle1:STN:280:DyaK1M3hsVOlsQ%3D%3D Occurrence Handle10200128

    CAS  PubMed  Google Scholar 

  50. RA Sater A Rostami (1998) ArticleTitleTreatment of Guillain–Barre syndrome with intravenous immunoglobulin Neurology 51 S9–15 Occurrence Handle1:CAS:528:DyaK1MXhtV2nuw%3D%3D Occurrence Handle9851724

    CAS  PubMed  Google Scholar 

  51. AF Hahn (1998) ArticleTitleTreatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin Neurology 51 S16–21 Occurrence Handle1:CAS:528:DyaK1MXhtV2msg%3D%3D Occurrence Handle9851725

    CAS  PubMed  Google Scholar 

  52. JP Azulay O Blin J Pouget et al. (1994) ArticleTitleIntravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study Neurology 44 429–432 Occurrence Handle1:STN:280:DyaK2c7psFCqsQ%3D%3D Occurrence Handle8145910

    CAS  PubMed  Google Scholar 

  53. S Jolles J Hughes S Whittaker (1998) ArticleTitleDermatological uses of high-dose intravenous immunoglobulin Arch Dermatol 134 80–86 Occurrence Handle10.1001/archderm.134.1.80 Occurrence Handle1:STN:280:DyaK1c7htFGltQ%3D%3D Occurrence Handle9449914

    Article  CAS  PubMed  Google Scholar 

  54. S Jolles (2002) ArticleTitleHigh-dose intravenous immunoglobulin (hdIVIg) in the treatment of autoimmune blistering disorders Clin Exp Immunol 129 385–389 Occurrence Handle1:CAS:528:DC%2BD38Xnsl2hu78%3D Occurrence Handle12197877 Occurrence Handle10.1046/j.1365-2249.2002.01967.x

    Article  CAS  PubMed  Google Scholar 

  55. C Prins C Vittorio RS Padilla et al. (2003) ArticleTitleEffect of high-dose intravenous immunoglobulin therapy in Stevens–Johnson syndrome: a retrospective, multicenter study Dermatology 207 96–99 Occurrence Handle10.1159/000070957 Occurrence Handle1:CAS:528:DC%2BD3sXltlChtLk%3D Occurrence Handle12835566

    Article  CAS  PubMed  Google Scholar 

  56. MM Kozel RA Sabroe (2004) ArticleTitleChronic urticaria: aetiology, management and current and future treatment options Drugs 64 2515–2536 Occurrence Handle10.2165/00003495-200464220-00003 Occurrence Handle1:CAS:528:DC%2BD2MXhs1Omug%3D%3D Occurrence Handle15516152

    Article  CAS  PubMed  Google Scholar 

  57. N Kumar M Rodriguez (2004) ArticleTitleScleromyxedema in a patient with multiple sclerosis and monoclonal gammopathy on interferon beta-la Mult Scler 10 85–86 Occurrence Handle10.1191/1352458504ms987cr Occurrence Handle14760958

    Article  PubMed  Google Scholar 

  58. K Amemiya C Semino–Mora RP Granger et al. (2000) ArticleTitleDownregulation of TGF-betal mRNA and protein in the muscles of patients with inflammatory myopathies after treatment with high-dose intravenous immunoglobulin Clin Immunol 94 99–104 Occurrence Handle10.1006/clim.1999.4823 Occurrence Handle1:CAS:528:DC%2BD3cXhtFOktb4%3D Occurrence Handle10637094

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. University Dermatology Centre, University of Alberta, Edmonton, AB, Canada

    Candace Majeski, Muba Taher, Parbeer Grewal, Marlene Dytoc & Gilles Lauzon

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  1. Candace Majeski
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  2. Muba Taher
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  5. Gilles Lauzon
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Correspondence to Gilles Lauzon.

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Majeski, C., Taher, M., Grewal, P. et al. Combination Oral Prednisone and Intravenous Immunoglobulin in the Treatment of Scleromyxedema. J Cutan Med Surg 9, 99–104 (2005). https://doi.org/10.1007/s10227-005-0137-9

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