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Henoch‐Schönlein Purpura in a Child with Hyperimmunoglobulinemia D and Periodic Fever Syndrome
Author(s) -
Wickiser Jonathan E.,
Saulsbury Frank T.
Publication year - 2005
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2005.22210.x
Subject(s) - immunoglobulin d , medicine , purpura (gastropod) , henoch schonlein purpura , immunology , dermatology , antibody , vasculitis , biology , disease , b cell , ecology
This report describes a 3‐year‐old girl with a long history of periodic fever who presented with Henoch‐Schönlein purpura. She was diagnosed with hyperimmunoglobulinemia D and periodic fever syndrome by means of mutation analysis of the mevalonate kinase gene. The serum IgA concentration was markedly elevated, but the serum IgD concentration was normal. This report emphasizes that Henoch‐Schönlein purpura may be an important clinical feature of hyperimmunoglobulinemia D and periodic fever syndrome. In addition, this syndrome should be considered in patients with Henoch‐Schönlein purpura in whom there is a history of recurrent fevers, even when the serum IgD concentration is normal.