Document Detail

SAPHO osteomyelitis and sarcoid dermatitis in a patient with DiGeorge syndrome.
MedLine Citation:
PMID:  16491384     Owner:  NLM     Status:  MEDLINE    
We report the development and spontaneous resolution of annular erythematous skin lesions consistent with sarcoid dermatitis in a child with DiGeorge syndrome (DGS) carrying the 22q11.2 microdeletion. The skin lesion developed after she was treated with isoniazid (INH) following exposure to active tuberculosis (TB). After resolution of the skin lesions, this child developed sterile hyperplastic osteomyelitis consistent with SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) osteomyelitis in her right mandible triggered by an odontogenic infection. This child had congenital heart disease, dysmorphic facies, recurrent sinopulmonary infection, gastroesophageal reflux disease, scoliosis, reactive periostitis, and developmental delay. She had a low CD4 and CD8 T cell count with a normal 4/8 ratio, but normal cell proliferation and T cell cytokine production in response to mitogens. When she was presented with sterile osteomyelitis of right mandible, she revealed polyclonal hypergammaglobulinemia with elevated erythrocyte sedimentation rate (ESR)/angiotensin converting enzyme (ACE) levels, but negative CRP. Autoimmune and sarcoidosis workup was negative. Inflammatory parameters gradually normalized following resolution of odontogenic infection and with the use of non-steroidal anti-inflammatory drugs (NSAIDs). The broad clinical spectrum of DGS is further expanded with the development of autoimmune and inflammatory complications later in life. This case suggests that patients with the DGS can present with unusual sterile inflammatory lesions triggered by environmental factors, further broadening the clinical spectrum of this syndrome.
Harumi Jyonouchi; Kenneth W Lien; Helen Aguila; Gaetano G Spinnato; Sanjeev Sabharwal; Beth A Pletcher
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2006-02-21
Journal Detail:
Title:  European journal of pediatrics     Volume:  165     ISSN:  0340-6199     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2006 Jun 
Date Detail:
Created Date:  2006-05-01     Completed Date:  2007-06-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  370-3     Citation Subset:  IM    
Division of Pulmonary, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, University of Medicine and Dentistry of New Jersey (UMDNJ)-New Jersey Medical School, Newark, NJ 07101-1709, USA.
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MeSH Terms
Acquired Hyperostosis Syndrome / complications*,  immunology
Dermatitis / complications*,  immunology
DiGeorge Syndrome / complications*,  immunology
Osteomyelitis / complications*

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