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Hypergammaglobulinemic purpura of Waldenstrom - Skin
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Hypergammaglobulinemic purpura of Waldenstrom - Skin

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Contributors: Vimal Prajapati MD, Ben Barankin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Hypergammaglobulinemic purpura, also referred to as hyperglobulinemic purpura of Waldenstrom, is a rare chronic disorder characterized clinically by recurrent crops of non-thrombocytopenic petechiae and purpura on the dependent areas of the body and serologically by polyclonal hypergammaglobulinemia, positive IgG or IgA rheumatoid factor (RF) titers, and elevated erythrocyte sedimentation rate (ESR); many patients also have antibodies to Ro/SS-A and La/SS-B. This condition most commonly affects young to middle-aged females and is frequently exacerbated by prolonged periods of standing. The eruption is usually asymptomatic yet some patients may report burning, stinging, or pruritus. The exact etiology and pathogenesis remains unknown, although an immune-complex mediated mechanism has been postulated.

Hypergammaglobulinemic purpura can be primary (idiopathic) or secondary to an underlying disease. Associated diseases include autoimmune connective tissue disorders, most frequently Sjögren's syndrome and occasionally systemic lupus erythematosus or rheumatoid arthritis, 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. 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.

Codes

ICD10CM:
D89.0 – Polyclonal hypergammaglobulinemia

SNOMEDCT:
190809001 – Waldenstrom hypergammaglobulinemic purpura

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Differential Diagnosis & Pitfalls

  • Immunoglobulin A vasculitis (formerly Henoch-Schönlein purpura) – most commonly affects children with a 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, systemic symptoms (especially gastrointestinal, renal, respiratory or joint) and laboratory abnormalities relating to underlying causes such as autoimmune disorders, drugs, infection, or malignancy, and typical histopathologic findings of leukocytoclastic vasculitis.
  • Pigmented purpuric dermatoses – purpura more copper-brown in color ("cayenne pepper" appearance), males more frequently affected, and 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 with typical histopathologic findings including epidermotropism of atypical lymphocytes, Pautrier's microabscesses, and presence of clonal T-cell receptor gene rearrangement.
  • Idiopathic thrombocytopenic purpura (ITP) – more petechiae and purpura tend to be smaller in size with characteristic history of bleeding and low platelet count in setting of normal bone marrow and absence of disease or medication associated with thrombocytopenia.
  • Thrombotic thrombocytopenic purpura (TTP) – more 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 – characterized by dietary history of inadequate vitamin C intake, perifollicular purpura, corkscrew hairs, spongy bleeding gums, specific radiologic features relating to vitamin C deficiency, and low fasting ascorbic acid levels; more frequently seen in underdeveloped countries.

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Last Updated: 01/16/2019
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Hypergammaglobulinemic purpura of Waldenstrom - Skin
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Hypergammaglobulinemic purpura of Waldenstrom : Hypergammaglobulinemia, ESR elevated, La positive, Ro positive, Lower legs
Clinical image of Hypergammaglobulinemic purpura of Waldenstrom
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