Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Dysproteinemic Purpura of the Hypergammaglobulinemic Type: Clinical Features and Differential Diagnosis

Dysproteinemic Purpura of the Hypergammaglobulinemic Type: Clinical Features and Differential... Abstract Dysproteinemic purpura is a more or less chronic condition characterized by purpura and by a serum protein pattern which is abnormal qualitatively, quantitatively, or both. Three types of this condition have been recognized. In 1943 "purpura hyperglobulinemica" was described by Waldenström1; in his patients purpura of the legs of a chronic, recurrent type was accompanied by an increase in serum γ-globulin and elevated sedimentation rate without definite, recognizable systemic disease. Subsequently, macroglobulinemia of Waldenström2,3 in 1944 and cryoglobulinemia of Lerner and Watson4 in 1947 were recognized as distinct dysproteinemic syndromes with hyperglobulinemia. Lerner and Watson stated that purpura cryoglobulinemica could be considered a special type of purpura hyperglobulinemica of Waldenström. In this paper "dysproteinemic purpura of the hypergammaglobulinemic type" will be used to designate the original syndrome of "purpura hyperglobulinemica of Waldenström."* Although a peculiar, hyperpigmented purpura of the legs is the References 1. Other synonyms in the literature are "benign hyperglobulinemic purpura,"11,23 "idiopathic benign hyperglobulinemic purpura,"12 and "purpura of idiopathic hyperglobulinemia."7 2. The Sia water test consists of dropping serum into distilled water and observing the degree of cloudiness. If much opaqueness results, the test is positive and indicative of proteins of abnormal quality such as macroglobulins. 3. Normal serum albumin has a molecular weight of 65,000 with a sedimentation constant Sf 4.5 and globulin 150,000-300,000 with constant Sf 7. 4. The lower Tiselius value for albumin and γ-globulin is explained by preceding precipitation. 5. Waldenström, J.: Clinical Methods for Determination of Hyperproteinemia and Their Practical Value for Diagnosis, Contributing to Study of "Essential High Sedimentation Reaction," Nord. med. 20:2288, 1943. 6. Ferriman, D. G., and Anderson, A. B.: Macroglobulinaemia of Waldenström , Brit. M. J. 2:402, 1956.Crossref 7. Voigt, A. E., and Frick, P. G.: Macroglobulinemia of Waldenström: A Review of the Literature and Presentation of a Case , Ann. Int. Med. 44:419, 1956.Crossref 8. Lerner, A. B., and Watson, C. J.: Studies of Cryoglobulins: I. Unusual Purpura Associated with the Presence of a High Concentration of Cryoglobulin (Cold Precipitable Serum Globulin) , Am. J. M. Sc. 214:410, 1947.Crossref 9. Lindeboom, G. A.: Purpura Hyperglobulinemica , Dermatologica 96:337, 1948.Crossref 10. Oberste-Lehn, H.: Ein Fall von Purpura hyperglobulinaemica unter dem Bilde der Schambergschen Erkrankung , Zentralbl. Haut. u. Geschlechtskr. 7:404, 1949-1950. 11. Fleischmajer, R.; Rein, C. R.; Pascher, F., and Sims, C. F.: Purpura of Idiopathic Hyperglobulinemia (Waldenström's Purpura Hyperglobulinemica) , presented before the 77th Annual Meeting of the American Dermatological Association, Belleair, Fla., 1957 . 12. Bender, F., and Gerlach, U.: Zur Kenntnis der Purpura hyperglobulinaemica , Hautarzt 6:456, 1955. 13. Waldenström, J.: Three New Cases of Purpura Hyperglobulinemica: A Study in Long Lasting Benign Increase in Serum Globulin , Acta. med. scandinav. ( (Supp. 266) ) 142:931, 1952.Crossref 14. Waldenström, J.: Purpura hyperglobulinämica und verwandte Zustände , Verhandl. deutsch. Gesellsch. inn. Med. 58:557, 1952. 15. Taylor, F. E., and Battle, J. D.: Benign Hyperglobulinemic Purpura: Case Report , Ann. Int. Med. 40:350, 1954.Crossref 16. Seiden, G. E., and Wurzel, H. A.: Idiopathic Benign Hyperglobulinemic Purpura , New England J. Med. 255:170, 1956.Crossref 17. Linke, A.: Primäre und sekundäre Dysproteinämie mit Purpura und Raynaud-Syndrom , Arch. Dermat. u. Syph. 191:123, 1950. 18. Dörken, H.: Primäre und sekundäre Purpura hyperglobulinaemica , Acta haemat. 10:340, 1953.Crossref 19. Dörken, H.: Die Purpura hyperglobulinaemica (Waldenström) und ihre Zuordnung , Verhandl. deutsch. Gesellsch. inn. Med. 60:761, 1954. 20. Waldenström, J.: Zwei interessante Syndrome mit Hyperglobulinämie (Purpura hyperglobulinaemica und Macroglobulinämie) , Schweiz. med. Wchnschr. 78:927, 1948. 21. Curtz, F. R.: Purpura hyperglobulinaemica , Ugesk. laeger 108:340, 1946. 22. Humerfelt, S.: Purpura hyperglobulinaemia , Nord. med. 34:1101, 1947. 23. Mielke, H. G.: Purpura hyperglobulinaemica (Waldenström) , Ärztl. Wchnschr. 8:241, 1953. 24. Fallet, G. H., and Haenni, A.: A propos d'un nouveau cas de purpura hyperglobulinémique de Waldenstroem , Praxis 43:71, 1954. 25. Vannuchi, V., and Doni, A.: Su di alcune manifestazioni microangiopatiche in protidoplatmotie: I. A proposito di un caso di porpora iperglobulinemica , Riv. crit. clin. med. 54:349, 1954. 26. Martin, E.: Démonstrations cliniques , Helvet. med. acta 22:288, 1955. 27. Kay, H. E., and Robertson, K. M.: Benign Hyperglobulinemic Purpura , J. Path. & Bact. 70: 543, 1955. 28. Fabiani, F.; Lucentini, L., and Marinoni, G. F.: Considerazioni su alcuni nuovi casi di porpora iperglobulinemica , Policlinico (sez. prat.) 62:1257, 1955. 29. Schmengler, F. E., and Esser, H.: Zur Pathogenese der Purpura hyperglobulinaemica , Klin. Wchnschr. 30:30, 1952. 30. Latvalahti, J., and Halonen, P. I.: Purpura hyperglobulinaemica , Ann. med. int. Fenniae , 41: 91, 1952. 31. Worth, G., and Schmidt, H.: Kasuistischer Beitrag zum Syndrom der "Purpura hyperglobulinaemica," München. med. Wchnschr. 94:2370, 1952. 32. Gautier, A., and Maurice, P. A.: Purpura hyperglobulinémique de Waldenstroem et maladie de Besnier-Boeck-Schaumann , Schweiz. med. Wchnschr. 83:1110, 1953. 33. Weinreich, J.: Die diagnostische und Abgrenzung von Makroglobulinämie (Waldenström) and Purpura hyperglobulinaemica (Waldenström) , München. med. Wchnschr. 97:1488, 1955. 34. Weise, H. J.: Sekundäre Purpura hyperglobulinaemica Waldenström mit akuter Polyarthritis als Grundkrankheit , Acta haemat. 15:23, 1956. 35. Berthoud, E.; Fallet, G., and Scheidegger, J. J.: Le purpura hyperglobulinémique de Waldenström , Semaine hôp. Paris 32:2135, 1956. 36. Bruns, G.: Morphologische Milzbefunde bei der Purpura hyperglobulinaemica (Waldenström) , Klin. Wchnschr. 31:169, 1953. 37. Horster, J. A.: Purpura hyperglobulinaemica , Acta Hemat. 4:201, 1950. 38. Mackay, I. R.; Eriksen, N.; Motulsky, A. G., and Volwiler, W.: Cryo- and Macroglobulinemia Electrophoretic, Ultracentrifugal and Clinical Studies , Am. J. Med. 20:564, 1956. 39. Volpé, R.; Bruce-Robertson, A.; Fletcher, A. A., and Charles, W. B.: Essential Cryoglobulinaemia: Review of the Literature and Report of a Case Treated with ACTH and Cortisone , Am. J. Med. 20:533, 1956. 40. Steinhardt, M. J., and Fisher, G. S.: Cold Urticaria and Purpura as Allergic Aspects of Cryoglobulinemia , J. Allergy 24:335, 1953. 41. Jadassohn, W.; Paillard, R., and others: Purpura de Schönlein-Henoch et purpura hyperglobulinémique , Dermatologica , 110, 355, 1955. 42. Feinstein, A. R., and Petersdorf, R. G.: The Clinical Significance of Hyperglobulinemia: I. Diagnostic Implications , Ann. Int. Med. 44:899, 1956. 43. Kierland, R. R.: Pigmented Purpuric Diseases of the Lower Extremities , M. Clin. North America 35:457, 1951. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Dermatology American Medical Association

Dysproteinemic Purpura of the Hypergammaglobulinemic Type: Clinical Features and Differential Diagnosis

A.M.A. Archives of Dermatology , Volume 77 (1) – Jan 1, 1958

Loading next page...
 
/lp/american-medical-association/dysproteinemic-purpura-of-the-hypergammaglobulinemic-type-clinical-hdEEHrsogK
Publisher
American Medical Association
Copyright
Copyright © 1958 American Medical Association. All Rights Reserved.
ISSN
0096-5359
DOI
10.1001/archderm.1958.01560010025005
Publisher site
See Article on Publisher Site

Abstract

Abstract Dysproteinemic purpura is a more or less chronic condition characterized by purpura and by a serum protein pattern which is abnormal qualitatively, quantitatively, or both. Three types of this condition have been recognized. In 1943 "purpura hyperglobulinemica" was described by Waldenström1; in his patients purpura of the legs of a chronic, recurrent type was accompanied by an increase in serum γ-globulin and elevated sedimentation rate without definite, recognizable systemic disease. Subsequently, macroglobulinemia of Waldenström2,3 in 1944 and cryoglobulinemia of Lerner and Watson4 in 1947 were recognized as distinct dysproteinemic syndromes with hyperglobulinemia. Lerner and Watson stated that purpura cryoglobulinemica could be considered a special type of purpura hyperglobulinemica of Waldenström. In this paper "dysproteinemic purpura of the hypergammaglobulinemic type" will be used to designate the original syndrome of "purpura hyperglobulinemica of Waldenström."* Although a peculiar, hyperpigmented purpura of the legs is the References 1. Other synonyms in the literature are "benign hyperglobulinemic purpura,"11,23 "idiopathic benign hyperglobulinemic purpura,"12 and "purpura of idiopathic hyperglobulinemia."7 2. The Sia water test consists of dropping serum into distilled water and observing the degree of cloudiness. If much opaqueness results, the test is positive and indicative of proteins of abnormal quality such as macroglobulins. 3. Normal serum albumin has a molecular weight of 65,000 with a sedimentation constant Sf 4.5 and globulin 150,000-300,000 with constant Sf 7. 4. The lower Tiselius value for albumin and γ-globulin is explained by preceding precipitation. 5. Waldenström, J.: Clinical Methods for Determination of Hyperproteinemia and Their Practical Value for Diagnosis, Contributing to Study of "Essential High Sedimentation Reaction," Nord. med. 20:2288, 1943. 6. Ferriman, D. G., and Anderson, A. B.: Macroglobulinaemia of Waldenström , Brit. M. J. 2:402, 1956.Crossref 7. Voigt, A. E., and Frick, P. G.: Macroglobulinemia of Waldenström: A Review of the Literature and Presentation of a Case , Ann. Int. Med. 44:419, 1956.Crossref 8. Lerner, A. B., and Watson, C. J.: Studies of Cryoglobulins: I. Unusual Purpura Associated with the Presence of a High Concentration of Cryoglobulin (Cold Precipitable Serum Globulin) , Am. J. M. Sc. 214:410, 1947.Crossref 9. Lindeboom, G. A.: Purpura Hyperglobulinemica , Dermatologica 96:337, 1948.Crossref 10. Oberste-Lehn, H.: Ein Fall von Purpura hyperglobulinaemica unter dem Bilde der Schambergschen Erkrankung , Zentralbl. Haut. u. Geschlechtskr. 7:404, 1949-1950. 11. Fleischmajer, R.; Rein, C. R.; Pascher, F., and Sims, C. F.: Purpura of Idiopathic Hyperglobulinemia (Waldenström's Purpura Hyperglobulinemica) , presented before the 77th Annual Meeting of the American Dermatological Association, Belleair, Fla., 1957 . 12. Bender, F., and Gerlach, U.: Zur Kenntnis der Purpura hyperglobulinaemica , Hautarzt 6:456, 1955. 13. Waldenström, J.: Three New Cases of Purpura Hyperglobulinemica: A Study in Long Lasting Benign Increase in Serum Globulin , Acta. med. scandinav. ( (Supp. 266) ) 142:931, 1952.Crossref 14. Waldenström, J.: Purpura hyperglobulinämica und verwandte Zustände , Verhandl. deutsch. Gesellsch. inn. Med. 58:557, 1952. 15. Taylor, F. E., and Battle, J. D.: Benign Hyperglobulinemic Purpura: Case Report , Ann. Int. Med. 40:350, 1954.Crossref 16. Seiden, G. E., and Wurzel, H. A.: Idiopathic Benign Hyperglobulinemic Purpura , New England J. Med. 255:170, 1956.Crossref 17. Linke, A.: Primäre und sekundäre Dysproteinämie mit Purpura und Raynaud-Syndrom , Arch. Dermat. u. Syph. 191:123, 1950. 18. Dörken, H.: Primäre und sekundäre Purpura hyperglobulinaemica , Acta haemat. 10:340, 1953.Crossref 19. Dörken, H.: Die Purpura hyperglobulinaemica (Waldenström) und ihre Zuordnung , Verhandl. deutsch. Gesellsch. inn. Med. 60:761, 1954. 20. Waldenström, J.: Zwei interessante Syndrome mit Hyperglobulinämie (Purpura hyperglobulinaemica und Macroglobulinämie) , Schweiz. med. Wchnschr. 78:927, 1948. 21. Curtz, F. R.: Purpura hyperglobulinaemica , Ugesk. laeger 108:340, 1946. 22. Humerfelt, S.: Purpura hyperglobulinaemia , Nord. med. 34:1101, 1947. 23. Mielke, H. G.: Purpura hyperglobulinaemica (Waldenström) , Ärztl. Wchnschr. 8:241, 1953. 24. Fallet, G. H., and Haenni, A.: A propos d'un nouveau cas de purpura hyperglobulinémique de Waldenstroem , Praxis 43:71, 1954. 25. Vannuchi, V., and Doni, A.: Su di alcune manifestazioni microangiopatiche in protidoplatmotie: I. A proposito di un caso di porpora iperglobulinemica , Riv. crit. clin. med. 54:349, 1954. 26. Martin, E.: Démonstrations cliniques , Helvet. med. acta 22:288, 1955. 27. Kay, H. E., and Robertson, K. M.: Benign Hyperglobulinemic Purpura , J. Path. & Bact. 70: 543, 1955. 28. Fabiani, F.; Lucentini, L., and Marinoni, G. F.: Considerazioni su alcuni nuovi casi di porpora iperglobulinemica , Policlinico (sez. prat.) 62:1257, 1955. 29. Schmengler, F. E., and Esser, H.: Zur Pathogenese der Purpura hyperglobulinaemica , Klin. Wchnschr. 30:30, 1952. 30. Latvalahti, J., and Halonen, P. I.: Purpura hyperglobulinaemica , Ann. med. int. Fenniae , 41: 91, 1952. 31. Worth, G., and Schmidt, H.: Kasuistischer Beitrag zum Syndrom der "Purpura hyperglobulinaemica," München. med. Wchnschr. 94:2370, 1952. 32. Gautier, A., and Maurice, P. A.: Purpura hyperglobulinémique de Waldenstroem et maladie de Besnier-Boeck-Schaumann , Schweiz. med. Wchnschr. 83:1110, 1953. 33. Weinreich, J.: Die diagnostische und Abgrenzung von Makroglobulinämie (Waldenström) and Purpura hyperglobulinaemica (Waldenström) , München. med. Wchnschr. 97:1488, 1955. 34. Weise, H. J.: Sekundäre Purpura hyperglobulinaemica Waldenström mit akuter Polyarthritis als Grundkrankheit , Acta haemat. 15:23, 1956. 35. Berthoud, E.; Fallet, G., and Scheidegger, J. J.: Le purpura hyperglobulinémique de Waldenström , Semaine hôp. Paris 32:2135, 1956. 36. Bruns, G.: Morphologische Milzbefunde bei der Purpura hyperglobulinaemica (Waldenström) , Klin. Wchnschr. 31:169, 1953. 37. Horster, J. A.: Purpura hyperglobulinaemica , Acta Hemat. 4:201, 1950. 38. Mackay, I. R.; Eriksen, N.; Motulsky, A. G., and Volwiler, W.: Cryo- and Macroglobulinemia Electrophoretic, Ultracentrifugal and Clinical Studies , Am. J. Med. 20:564, 1956. 39. Volpé, R.; Bruce-Robertson, A.; Fletcher, A. A., and Charles, W. B.: Essential Cryoglobulinaemia: Review of the Literature and Report of a Case Treated with ACTH and Cortisone , Am. J. Med. 20:533, 1956. 40. Steinhardt, M. J., and Fisher, G. S.: Cold Urticaria and Purpura as Allergic Aspects of Cryoglobulinemia , J. Allergy 24:335, 1953. 41. Jadassohn, W.; Paillard, R., and others: Purpura de Schönlein-Henoch et purpura hyperglobulinémique , Dermatologica , 110, 355, 1955. 42. Feinstein, A. R., and Petersdorf, R. G.: The Clinical Significance of Hyperglobulinemia: I. Diagnostic Implications , Ann. Int. Med. 44:899, 1956. 43. Kierland, R. R.: Pigmented Purpuric Diseases of the Lower Extremities , M. Clin. North America 35:457, 1951.

Journal

A.M.A. Archives of DermatologyAmerican Medical Association

Published: Jan 1, 1958

References