title: Hyperimmunoglobulin E syndrome with juvenile dermatomyositis and calcinosis.
abstract: 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.
authors.affiliation: Department of Pediatrics, St Stephens Hospital, Tis Hazari, New Delhi, Delhi 110054, India. bedangshu@gmail.com
authors.forename: Bedangshu
authors.initials: B
authors.lastname: Saikia
authors.forename: Himanshu
authors.initials: H
authors.lastname: Aneja
authors.forename: Jyoti
authors.initials: J
authors.lastname: Jain
authors.forename: Jacob M
authors.initials: JM
authors.lastname: Puliyel
mesh_terms: D000038:Abscess / Q000097:blood* / Q000150:complications / Q000473:pathology; D002114:Calcinosis / Q000097:blood* / Q000209:etiology / Q000473:pathology; D002648:Child; D003882:Dermatomyositis / Q000097:blood* / Q000150:complications / Q000473:pathology; D006801:Humans; D016867:Immunocompromised Host; D007073:Immunoglobulin E / Q000097:blood*; D007589:Job Syndrome / Q000097:blood* / Q000473:pathology; D008297:Male; D018908:Muscle Weakness
chemical_list: D007073:Immunoglobulin E