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Hyperglobulinemic purpura

MedGen UID:
19585
•Concept ID:
C0034151
•
Disease or Syndrome
Synonyms: Benign Hyperglobulinemic Purpura of Waldenström; Hyperglobulinemic Purpura; Hyperglobulinemic Purpura of Waldenström; Hyperglobulinemic Purpura, Waldenstrom; Hyperglobulinemic Purpuras; Purpura, Hyperglobulinemic; Purpura, Waldenstrom Hyperglobulinemic; Purpuras, Hyperglobulinemic; Waldenstrom Hyperglobulinemic Purpura; Waldenström Hyperglobulinemic Purpura
SNOMED CT: Hyperglobulinemic purpura (402852007)
 
Monarch Initiative: MONDO:0006792

Definition

Purplish or brownish red discoloration of the skin associated with increase in circulating polyclonal globulins, usually gamma-globulins. This syndrome often occurs on the legs of women aged 20 to 40 years. [from MONDO]

Professional guidelines

PubMed

Hyperglobulinemic purpura; report of 14 cases fulfilling Waldenstrom's criteria.
WILSON SJ, BOLINGER RE
J Kans Med Soc 1957 Mar;58(3):166-9. PMID: 13406348

Recent clinical studies

Etiology

Hypergammaglobulinemic purpura of Waldenström in children.
Theisen E, Lee DE, Pei S, Schauder DM, Chiu YE, Brandling-Bennett H, Curran ML, Klein-Gitelman M, Co DO, Arkin LM
Pediatr Dermatol 2020 May;37(3):467-475. Epub 2020 Feb 27 doi: 10.1111/pde.14120. PMID: 32104943
Clinical and biological differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren's syndrome: results of a large multicentre study.
Quartuccio L, Isola M, Baldini C, Priori R, Bartoloni E, Carubbi F, Gregoraci G, Gandolfo S, Salvin S, Luciano N, Minniti A, Alunno A, Giacomelli R, Gerli R, Valesini G, Bombardieri S, De Vita S
Scand J Rheumatol 2015;44(1):36-41. Epub 2014 Sep 30 doi: 10.3109/03009742.2014.923931. PMID: 25268749
Hypergammaglobulinemic purpura of Waldenström.
Frankel A, Ingraffea A, Massé M, Zhou H
Cutis 2010 Jul;86(1):23-4. PMID: 21049762
Hypergammaglobulinemic purpura of Waldenström. (A report of serologic and immunogenetic studies and long-term follow-up in 18 patients).
Invernizzi F, Cattaneo R, Fenini MG, Carella G, Granatieri C, Savi M, Zanussi C
Ric Clin Lab 1988 Jan-Mar;18(1):23-36. doi: 10.1007/BF02918816. PMID: 3259714
Benign hyperglobulinemic purpura of Waldenström: a report of seven patients and long-term follow-up.
Lozada JA, Maldonado N, Sánchez J, Fernández C, Cortés M
Bol Asoc Med P R 1988 Dec;80(12):455-9. PMID: 3233123

Diagnosis

Hypergammaglobulinemic purpura of Waldenström in children.
Theisen E, Lee DE, Pei S, Schauder DM, Chiu YE, Brandling-Bennett H, Curran ML, Klein-Gitelman M, Co DO, Arkin LM
Pediatr Dermatol 2020 May;37(3):467-475. Epub 2020 Feb 27 doi: 10.1111/pde.14120. PMID: 32104943
Hypergammaglobulinemic purpura of Waldenström.
Lewin JM, Hunt R, Fischer M, Kamino H, Franks AG Jr
Dermatol Online J 2012 Dec 15;18(12):2. PMID: 23286792
Hypergammaglobulinemic purpura in systemic autoimmune rheumatic diseases: predictive value of anti-Ro(SSA) and anti-La(SSB) antibodies and treatment with indomethacin and hydroxychloroquine.
Senécal JL, Chartier S, Rothfield N
J Rheumatol 1995 May;22(5):868-75. PMID: 8587074
Hypergammaglobulinemic purpura of Waldenström.
Finder KA, McCollough ML, Dixon SL, Majka AJ, Jaremko W
J Am Acad Dermatol 1990 Oct;23(4 Pt 1):669-76. doi: 10.1016/0190-9622(90)70271-i. PMID: 2229494
Hypergammaglobulinemic purpura in cystic fibrosis.
Nielsen HE, Lundh S, Jacobsen SV, Høiby N
Acta Paediatr Scand 1978 Jul;67(4):443-7. doi: 10.1111/j.1651-2227.1978.tb16351.x. PMID: 307897

Therapy

Combination therapy of prednisolone and mizoribine improves cryoglobulinemic vasculitis with purpura and skin ulcers.
Hamaguchi M, Kawahito Y, Tsubouchi Y, Kohno M, Yamamoto A, Ishino H, Wada M, Yoshikawa T
Clin Rheumatol 2007 Jul;26(7):1170-2. Epub 2006 Apr 14 doi: 10.1007/s10067-006-0295-1. PMID: 16614792
Hypergammaglobulinemic purpura in systemic autoimmune rheumatic diseases: predictive value of anti-Ro(SSA) and anti-La(SSB) antibodies and treatment with indomethacin and hydroxychloroquine.
Senécal JL, Chartier S, Rothfield N
J Rheumatol 1995 May;22(5):868-75. PMID: 8587074
Cutaneous leukocytoclastic vasculitis with hyperglobulinemia and splenomegaly. A variant of hyperglobulinemic purpura of Waldenström.
Hudson CP, Callen JP
Arch Dermatol 1984 Sep;120(9):1224-6. PMID: 6206803
Immune complex vasculitis, polymyositis, and hyperglobulinemic purpura.
Ringel SP, Thorne EG, Phanuphak P, Lava NS, Kohler PS
Neurology 1979 May;29(5):682-9. doi: 10.1212/wnl.29.5.682. PMID: 155783
Benign hypergammaglobulinemic purpura of Waldenström.
Kyle RA, Gleich GJ, Bayrid ED, Vaughan JH
Medicine (Baltimore) 1971 Mar;50(2):113-23. doi: 10.1097/00005792-197103000-00003. PMID: 4944119

Prognosis

Hypergammaglobulinemic purpura of Waldenström in children.
Theisen E, Lee DE, Pei S, Schauder DM, Chiu YE, Brandling-Bennett H, Curran ML, Klein-Gitelman M, Co DO, Arkin LM
Pediatr Dermatol 2020 May;37(3):467-475. Epub 2020 Feb 27 doi: 10.1111/pde.14120. PMID: 32104943
Refractory primary Sjögren syndrome successfully treated with bortezomib.
Jakez-Ocampo J, Atisha-Fregoso Y, Llorente L
J Clin Rheumatol 2015 Jan;21(1):31-2. doi: 10.1097/RHU.0000000000000210. PMID: 25539431
Clinical and biological differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren's syndrome: results of a large multicentre study.
Quartuccio L, Isola M, Baldini C, Priori R, Bartoloni E, Carubbi F, Gregoraci G, Gandolfo S, Salvin S, Luciano N, Minniti A, Alunno A, Giacomelli R, Gerli R, Valesini G, Bombardieri S, De Vita S
Scand J Rheumatol 2015;44(1):36-41. Epub 2014 Sep 30 doi: 10.3109/03009742.2014.923931. PMID: 25268749
Hypergammaglobulinemic purpura of Waldenström.
Frankel A, Ingraffea A, Massé M, Zhou H
Cutis 2010 Jul;86(1):23-4. PMID: 21049762
Hypergammaglobulinemic purpura in systemic autoimmune rheumatic diseases: predictive value of anti-Ro(SSA) and anti-La(SSB) antibodies and treatment with indomethacin and hydroxychloroquine.
Senécal JL, Chartier S, Rothfield N
J Rheumatol 1995 May;22(5):868-75. PMID: 8587074

Clinical prediction guides

Refractory primary Sjögren syndrome successfully treated with bortezomib.
Jakez-Ocampo J, Atisha-Fregoso Y, Llorente L
J Clin Rheumatol 2015 Jan;21(1):31-2. doi: 10.1097/RHU.0000000000000210. PMID: 25539431
Hypergammaglobulinemic purpura in systemic autoimmune rheumatic diseases: predictive value of anti-Ro(SSA) and anti-La(SSB) antibodies and treatment with indomethacin and hydroxychloroquine.
Senécal JL, Chartier S, Rothfield N
J Rheumatol 1995 May;22(5):868-75. PMID: 8587074
IgG2 deficiency in primary Sjögren's syndrome and hypergammaglobulinemic purpura.
Eriksson P, Almroth G, Denneberg T, Lindström FD
Clin Immunol Immunopathol 1994 Jan;70(1):60-5. doi: 10.1006/clin.1994.1011. PMID: 8261670
Hypergammaglobulinemic purpura of Waldenström. (A report of serologic and immunogenetic studies and long-term follow-up in 18 patients).
Invernizzi F, Cattaneo R, Fenini MG, Carella G, Granatieri C, Savi M, Zanussi C
Ric Clin Lab 1988 Jan-Mar;18(1):23-36. doi: 10.1007/BF02918816. PMID: 3259714
Hypergammaglobulinemic purpura and Sjögren's syndrome in a child.
Celada A, Beck D, Chavaz P, Kapanci Y, Cruchaud A
Helv Paediatr Acta 1980 Dec;35(6):569-76. PMID: 7204101

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