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Treatment of Recalcitrant Leg Ulcers in Cryoglobulinemia Types I and II With Plasmapheresis

Treatment of Recalcitrant Leg Ulcers in Cryoglobulinemia Types I and II With Plasmapheresis Abstract REPORT OF CASES CASE 1 In November 1989, a 60-year-old white man with a 2-year history of chronic lymphocytic leukemia presented with a 1-week history of pain in both feet accompanied by progressive purpura and petechiae in the lower extremities. Purpura over both lateral malleoli and the right medial tibia ulcerated during the next 2 weeks (Figure 1, top). A skin biopsy specimen of a purpuric lesion revealed vascular occlusion with amorphous, eosinophilic material that was periodic acid Schiff—positive, diastase resistant, and phosphotungistic acid—hematoxylin negative. A mild perivascular infiltrate of lymphocytes and eosinophils was present. Special stains for organisms were negative.The patient's cryoglobulin screen demonstrated a cryocrit of 0.3, 0.2, and 0.5 at temperatures of 4°C, 22°C, and 37°C, respectively. Cryoglobulin analysis revealed a monoclonal IgG-K type I cryoglobulinemia (CG). Despite two courses of chlorambucil (30 mg/d for 5 days every 4 weeks) and prednisone (20 to 60 mg/d), References 1. Boom BW, Brand A, Bouwes-Bavinck J, Eernisse JG, Daha MR, Vermeer B. Severe leukocytoclastic vasculitis of the skin in a patient with essential mixed cryoglobulinemia treated with high-dose γ-globulin intravenously . Arch Dermatol. 1988;124:1550-1553.Crossref 2. Sidi Y, Weinberger A, Berliner S, et al. Relapsing polychondritis associated with mixed cryoglobulinemia . Dermalologica. 1982;165:66-70.Crossref 3. Levo Y. Nature of cryoglobulinemia . Lancet. 1980;1:285-287.Crossref 4. Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M. Biologic and clinical significance of cryoglobulins: a report of 86 cases . Am J Med. 1974;57:775-788.Crossref 5. Levo Y, Gorevic PD, Kassab HJ, Zucker-Franklin D, Franklin EC. Association between hepatitis B virus and essential mixed cryoglobulinemia . N Engl J Med. 1977;296:1501-1504.Crossref 6. Munoz-Fernandez S, Barbado FJ, Mola EM, et al. Evidence of hepatitis C virus antibodies in the cryoprecipitate of patients with mixed cryoglobulinemia . J Rheumatol. 1994;21:229-233. 7. Verdickt W, Dequeker J. Plasmapheresis combined with corticosteroids and cyclophosphamide in mixed monoclonal cryoglobulinemia . Acta Clin Belg. 1982; 37:237-240. 8. Cohen SJ, Pittelkow MR, Su WP. Cutaneous manifestations of cryoglobulinemia . J Am Acad Dermatol. 1991;25(pt (1) ):21-27.Crossref 9. Finn AF, Gorevic PD. Pathogenic paraproteins: gammopathies and cryoglobulinemia . Curr Opin Rheumatol. 1990;2:652-660.Crossref 10. Gorevic PD, Kassab HJ, Levo Y, et al. Mixed cryoglobulinemia . Am J Med. 1980;69:287-308.Crossref 11. Baughman RD, Sommer RG. Cryoglobulinemia presenting as 'factitial ulceration.' Arch Dermatol. 1966;94:725-731.Crossref 12. Placik OJ, Zukowski ML, Lewis Jr VL. Cryoglobulinemia: dilemma for the reconstructive surgeon . Plast Reconstr Surg. 1993;91:348-351.Crossref 13. Geltner D, Kohn RW, Gorevic P, Franklin EC. The effect of combination therapy (steroids, immunosuppressives, and plasmapheresis) on 5 mixed cryoglobulinemia patients with renal, neurologic, and vascular involvement . Arthritis Rheum. 1981;24:1121-1127.Crossref 14. Riu R, Ruth LD, Snyder CC. Skin necrosis secondary to cryoglobulinemia . Plast Reconstr Surg. 1970;46:510-512.Crossref 15. Shaw M, Van de Pette J, Fenton D, McGibbon DH. Mutilating cryoglobulinaemia rapidly improved by plasmapheresis: diagnostic features on blood film . J R Soc Med. 1985;78( (suppl 11) ):37-39. 16. Geltner D. Therapeutic approaches in mixed cryoglobulinemia . Springer Semin Immunopathol . 1988;10:103-113.Crossref 17. Ralston SH, Boyle IT. Essential mixed cryoglobulinaemia presenting with chronic perianal skin ulceration . Scot Med J. 1988;33:311-312. 18. Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis J Am Acad Dermatol. 1985;13:193-200.Crossref 19. Petersen J. Essential cryoglobulinaemia . Scand J Haematol. 1985;35:501-504.Crossref 20. Narita H, Ogata K, Kikuchi I, Inoue S. A case of cryoglobulinemic gangrene in myeloma with fatal outcome despite successful skin grafting . Dermatologica. 1980;160:125-130.Crossref 21. Berkman EM, Orlin JB. Use of plasmapheresis and partial plasma exchange in the management of patients with cryoglobulinemia . Transfusion. 1980;20:171-178.Crossref 22. Council on Scientific Affairs, American Medical Association. Current status of therapeutic plasmapheresis and related techniques . JAMA. 1985;253:819-825.Crossref 23. Karmochkine M, Bussel A, Leon A, Jarrousse B, Baudelot J, Guillevin L. Longterm plasma exchange: analysis of indications, outcome and side effects . Ann Med Interne (Paris) . 1994;145:373-375. 24. Leitman SF, Ciavarella D, McLeod B, et al. Guidelines for Therapeutic Hemapheresis . Bethesda, Md. American Association of Blood Banks; 1994. 25. Pusey CD. Plasma exchange in immunological disease . Prog Clin Biol Res. 1990;337:419-424. 26. Valbonesi M, Montani F, Mosconi L, Florio G, Vecchi C. Plasmapheresis and cytotoxic drugs for mixed cryoglobulinemia . Haematologia. 1984;17:341-351. 27. Pineda AA. Applications of therapeutic apheresis . Mayo Clin Proc. 1994;69: 893-894.Crossref 28. Nusbacher J. Therapeutic hemapheresis . Clin Lab Med. 1982:2:87-106. 29. McLeod BC, Sassetti RJ. Plasmapheresis with return of cryoglobulin-depleted autologous plasma (cryoglobulinpheresis) in cryoglobulinemia . Blood. 1980; 55:866-870. 30. Evans TW, Nicholls AJ, Shortland JR, Ward AM, Brown CB. Acute renal failure in essential mixed cryoglobulinemia: precipitation and reversal by plasma exchange . Clin Nephrol. 1984;21:287-293. 31. Jones JV, Clough JD, Klinenberg JR, Davis P. The role of therapeutic plasmapheresis in the rheumatic diseases . J Lab Clin Med. 1981;97:589-598. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Treatment of Recalcitrant Leg Ulcers in Cryoglobulinemia Types I and II With Plasmapheresis

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Publisher
American Medical Association
Copyright
Copyright © 1996 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1996.03890290024003
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Abstract

Abstract REPORT OF CASES CASE 1 In November 1989, a 60-year-old white man with a 2-year history of chronic lymphocytic leukemia presented with a 1-week history of pain in both feet accompanied by progressive purpura and petechiae in the lower extremities. Purpura over both lateral malleoli and the right medial tibia ulcerated during the next 2 weeks (Figure 1, top). A skin biopsy specimen of a purpuric lesion revealed vascular occlusion with amorphous, eosinophilic material that was periodic acid Schiff—positive, diastase resistant, and phosphotungistic acid—hematoxylin negative. A mild perivascular infiltrate of lymphocytes and eosinophils was present. Special stains for organisms were negative.The patient's cryoglobulin screen demonstrated a cryocrit of 0.3, 0.2, and 0.5 at temperatures of 4°C, 22°C, and 37°C, respectively. Cryoglobulin analysis revealed a monoclonal IgG-K type I cryoglobulinemia (CG). Despite two courses of chlorambucil (30 mg/d for 5 days every 4 weeks) and prednisone (20 to 60 mg/d), References 1. Boom BW, Brand A, Bouwes-Bavinck J, Eernisse JG, Daha MR, Vermeer B. Severe leukocytoclastic vasculitis of the skin in a patient with essential mixed cryoglobulinemia treated with high-dose γ-globulin intravenously . Arch Dermatol. 1988;124:1550-1553.Crossref 2. Sidi Y, Weinberger A, Berliner S, et al. Relapsing polychondritis associated with mixed cryoglobulinemia . Dermalologica. 1982;165:66-70.Crossref 3. Levo Y. Nature of cryoglobulinemia . Lancet. 1980;1:285-287.Crossref 4. Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M. Biologic and clinical significance of cryoglobulins: a report of 86 cases . Am J Med. 1974;57:775-788.Crossref 5. Levo Y, Gorevic PD, Kassab HJ, Zucker-Franklin D, Franklin EC. Association between hepatitis B virus and essential mixed cryoglobulinemia . N Engl J Med. 1977;296:1501-1504.Crossref 6. Munoz-Fernandez S, Barbado FJ, Mola EM, et al. Evidence of hepatitis C virus antibodies in the cryoprecipitate of patients with mixed cryoglobulinemia . J Rheumatol. 1994;21:229-233. 7. Verdickt W, Dequeker J. Plasmapheresis combined with corticosteroids and cyclophosphamide in mixed monoclonal cryoglobulinemia . Acta Clin Belg. 1982; 37:237-240. 8. Cohen SJ, Pittelkow MR, Su WP. Cutaneous manifestations of cryoglobulinemia . J Am Acad Dermatol. 1991;25(pt (1) ):21-27.Crossref 9. Finn AF, Gorevic PD. Pathogenic paraproteins: gammopathies and cryoglobulinemia . Curr Opin Rheumatol. 1990;2:652-660.Crossref 10. Gorevic PD, Kassab HJ, Levo Y, et al. Mixed cryoglobulinemia . Am J Med. 1980;69:287-308.Crossref 11. Baughman RD, Sommer RG. Cryoglobulinemia presenting as 'factitial ulceration.' Arch Dermatol. 1966;94:725-731.Crossref 12. Placik OJ, Zukowski ML, Lewis Jr VL. Cryoglobulinemia: dilemma for the reconstructive surgeon . Plast Reconstr Surg. 1993;91:348-351.Crossref 13. Geltner D, Kohn RW, Gorevic P, Franklin EC. The effect of combination therapy (steroids, immunosuppressives, and plasmapheresis) on 5 mixed cryoglobulinemia patients with renal, neurologic, and vascular involvement . Arthritis Rheum. 1981;24:1121-1127.Crossref 14. Riu R, Ruth LD, Snyder CC. Skin necrosis secondary to cryoglobulinemia . Plast Reconstr Surg. 1970;46:510-512.Crossref 15. Shaw M, Van de Pette J, Fenton D, McGibbon DH. Mutilating cryoglobulinaemia rapidly improved by plasmapheresis: diagnostic features on blood film . J R Soc Med. 1985;78( (suppl 11) ):37-39. 16. Geltner D. Therapeutic approaches in mixed cryoglobulinemia . Springer Semin Immunopathol . 1988;10:103-113.Crossref 17. Ralston SH, Boyle IT. Essential mixed cryoglobulinaemia presenting with chronic perianal skin ulceration . Scot Med J. 1988;33:311-312. 18. Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis J Am Acad Dermatol. 1985;13:193-200.Crossref 19. Petersen J. Essential cryoglobulinaemia . Scand J Haematol. 1985;35:501-504.Crossref 20. Narita H, Ogata K, Kikuchi I, Inoue S. A case of cryoglobulinemic gangrene in myeloma with fatal outcome despite successful skin grafting . Dermatologica. 1980;160:125-130.Crossref 21. Berkman EM, Orlin JB. Use of plasmapheresis and partial plasma exchange in the management of patients with cryoglobulinemia . Transfusion. 1980;20:171-178.Crossref 22. Council on Scientific Affairs, American Medical Association. Current status of therapeutic plasmapheresis and related techniques . JAMA. 1985;253:819-825.Crossref 23. Karmochkine M, Bussel A, Leon A, Jarrousse B, Baudelot J, Guillevin L. Longterm plasma exchange: analysis of indications, outcome and side effects . Ann Med Interne (Paris) . 1994;145:373-375. 24. Leitman SF, Ciavarella D, McLeod B, et al. Guidelines for Therapeutic Hemapheresis . Bethesda, Md. American Association of Blood Banks; 1994. 25. Pusey CD. Plasma exchange in immunological disease . Prog Clin Biol Res. 1990;337:419-424. 26. Valbonesi M, Montani F, Mosconi L, Florio G, Vecchi C. Plasmapheresis and cytotoxic drugs for mixed cryoglobulinemia . Haematologia. 1984;17:341-351. 27. Pineda AA. Applications of therapeutic apheresis . Mayo Clin Proc. 1994;69: 893-894.Crossref 28. Nusbacher J. Therapeutic hemapheresis . Clin Lab Med. 1982:2:87-106. 29. McLeod BC, Sassetti RJ. Plasmapheresis with return of cryoglobulin-depleted autologous plasma (cryoglobulinpheresis) in cryoglobulinemia . Blood. 1980; 55:866-870. 30. Evans TW, Nicholls AJ, Shortland JR, Ward AM, Brown CB. Acute renal failure in essential mixed cryoglobulinemia: precipitation and reversal by plasma exchange . Clin Nephrol. 1984;21:287-293. 31. Jones JV, Clough JD, Klinenberg JR, Davis P. The role of therapeutic plasmapheresis in the rheumatic diseases . J Lab Clin Med. 1981;97:589-598.

Journal

Archives of DermatologyAmerican Medical Association

Published: May 1, 1996

References