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• W3007668858 abstract "Abstract Background Hypergammaglobulinemic purpura of Waldenström (HGPW), a rare cutaneous eruption characterized by the triad of recurrent episodes of lower extremity petechiae, symptoms of stinging and burning, and lower extremity edema, is poorly described in children. Some children have been reported to follow a benign course, while others are eventually diagnosed with fulminant rheumatologic disease. Objectives To determine the distinguishing features of HGPW including the spectrum of disease manifestations and clinical outcomes. Methods This is a multicenter, retrospective case series of six children with HGPW combined with a literature review of 45 previously published pediatric cases. Results Most children were eventually diagnosed with systemic disease (63%) or developed autoantibody accumulation suggestive of evolving disease (71%). The most common diagnoses were Sjogren's syndrome and systemic lupus erythematosus. The mean duration between onset of cutaneous eruption and diagnosis of systemic disease was 5.6 years, underscoring that HPGW patients often present with a rash that precedes the development of systemic symptoms. Conclusions Diagnosis of HGPW should prompt initial screening for rheumatologic disease with long‐term rheumatology follow‐up, as the majority of patients present with evolving manifestations of systemic disease." @default.
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• W3007668858 date "2020-02-27" @default.
• W3007668858 modified "2023-10-16" @default.
• W3007668858 title "Hypergammaglobulinemic purpura of Waldenström in children" @default.
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• W3007668858 doi "https://doi.org/10.1111/pde.14120" @default.
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