Hypergammaglobulinaemic Purpura Associated with IgG Subclass Imbalance and Recurrent Infection

BackgroundHypergammaglobulinemic 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.ObjectivesTo determine the distinguishing features of HGPW including the spectrum of disease manifestations and clinical outcomes.MethodsThis is a multicenter, retrospective case series of six children with HGPW combined with a literature review of 45 previously published pediatric cases.ResultsMost 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.ConclusionsDiagnosis 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.

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Hypergammaglobulinaemic Purpura Associated with IgG Subclass Imbalance and Recurrent Infection

Abstract: We describe a child who initially presented with recurrent infections. He subsequently developed hypergammaglobulinemic purpura but responded well to corticosteroids and hydroxychloroquine.

Cited by 3 publications

References 5 publications

“Benign” hypergammaglobulinemic purpura is not benign in pregnancy

“Benign” hypergammaglobulinemic purpura is not benign in pregnancy

Cutaneous Vascular Diseases

Hypergammaglobulinemic purpura of Waldenström in children

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