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Hyperglobulinemic purpura of Waldenström
Other Resources UpToDate PubMed

Hyperglobulinemic purpura of Waldenström

Contributors: Erin X. Wei MD, Vimal Prajapati MD, Ben Barankin MD, Lowell A. Goldsmith MD, MPH, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Hyperglobulinemic purpura of Waldenström is a rare chronic disorder characterized by recurrent crops of nonthrombocytopenic petechiae and purpura on the dependent areas of the body, polyclonal hypergammaglobulinemia, and elevated ESR. This condition most commonly affects young to middle-aged females (late teens to 40s) but can be seen in children.

Hyperglobulinemic purpura of Waldenström is classified as primary (idiopathic) or secondary to an underlying disease. Associated diseases include autoimmune connective tissue disorders, most commonly Sjögren syndrome; occasionally systemic lupus erythematosus, rheumatoid arthritis, autoimmune hepatitis, hepatitis C, or cystic fibrosis; and rarely hematologic malignancies, such as monoclonal gammopathy, lymphoma, or multiple myeloma. The onset of hypergammaglobulinemic purpura often precedes the underlying disease by many years, with some associated diseases developing 10 or more years later. Autoantibodies that are commonly positive include Ro/SS-A, La/SS-B, antinuclear antibodies (ANA), and rheumatoid factor (RF). The presence of anti-Ro/SS-A and anti-La/SS-B antibodies may predict a higher likelihood of developing an associated autoimmune connective tissue disorder. Mild anemia or lymphopenia can also be found.

The petechiae and purpura are frequently exacerbated by prolonged periods of standing, wearing tight clothing, alcohol intake, and exercise. The eruption is usually asymptomatic, yet some patients may report burning, stinging, pruritus, or lower extremity swelling. The pathogenesis of purpura is thought to be due to immune complex deposition in the blood vessels of the lower extremities. Truncal involvement can also be seen.

When this condition is seen in pregnancy, it is not considered benign; all reported cases are associated with anti-SS-A antibodies, with potential increased risk of poor maternal / fetal outcome including intrauterine fetal growth restriction, congenital heart block, and fetal demise. More than half of children, as reported in the literature, will go on to develop an autoimmune condition.

Codes

ICD10CM:
D89.0 – Polyclonal hypergammaglobulinemia

SNOMEDCT:
190809001 – Waldenstrom hypergammaglobulinemic purpura

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Differential Diagnosis & Pitfalls

  • Immunoglobulin A (IgA) vasculitis (formerly Henoch-Schönlein purpura) – Characteristic clinical tetrad of palpable purpura, abdominal pain, arthritis, and hematuria and typical histopathologic findings of leukocytoclastic vasculitis with IgA deposition in blood vessel walls.
  • Cutaneous small vessel vasculitis – Classic presentation is palpable purpura; histopathologic findings are typical of leukocytoclastic vasculitis.
  • Pigmented purpuric dermatoses – Purpura is more copper-brown in color ("cayenne pepper" appearance); males are more frequently affected; no associated underlying disease.
  • Purpuric contact dermatitis – Rare disorder resulting from allergy to textile dyes and resins; patch testing will reveal responsible allergens.
  • Mycosis fungoides – Can appear as pigmented purpura-like eruption, differentiated histopathologically.
  • Idiopathic thrombocytopenic purpura (ITP) – Petechiae, purpura, and characteristic history of bleeding and low platelet count in setting of normal bone marrow; absence of disease or medication associated with thrombocytopenia.
  • Thrombotic thrombocytopenic purpura (TTP) – Petechiae and purpura tend to be smaller in size with characteristic clinical pentad of thrombocytopenia, microangiopathic hemolytic anemia (presence of schistocytes on peripheral blood smear), renal failure, neurologic deficits, and fever.
  • Scurvy – Inadequate vitamin C intake, perifollicular purpura, corkscrew hairs, spongy bleeding gums, and low fasting ascorbic acid levels.

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Therapy

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References

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Last Reviewed:12/17/2020
Last Updated:01/18/2021
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Hyperglobulinemic purpura of Waldenström
Hyperglobulinemic purpura of Waldenström : Hypergammaglobulinemia, ESR elevated, La positive, Ro positive, Lower legs
Clinical image of Hyperglobulinemic purpura of Waldenström
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