Hyperimmunoglobulin E syndrome with juvenile dermatomyositis and calcinosis

Juvenile dermatomyositis (JDM) is a rare childhood disease with autoimmune association. Environmental factors are known to trigger JDM in genetically susceptible individuals (Schmieder et al., Dermatol Online 6:3, 2009 ). Calcinosis is a well-established complication of JDM. Prevalence is higher in children (30–70%; Özkaya et al., Erciyes Med J 30(1):40–43, 2008 ). Hyperimmunoglobulin E syndrome is a primary immunodeficiency syndrome with multiple recurrent abscess formation and raised serum immunoglobulin E levels. We report a case of JDM with calcinosis cutis universalis with hyperimmunoglobulin E syndrome. With a previous similar case report (Min et al., Korean J Intern Med 14:95–98, 1999 ), this could well be a new sequence syndrome where abscesses are the trigger for the onset of JDM.