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Alpha-1-antitrypsin deficiency-associated panniculitis: a case report.

Abstract
Panniculitis is a recognized, but rare complication of α1-antitrypsin (A1AT) deficiency. Less than 60 cases have been reported, mostly in the homozygous PiZZ variant. We report the case of a 55-year old woman with A1AT panniculitis associated with the heterozygous phenotype PiMS and discrete reduction of A1AT serum levels. In addition, the pathophysiology of the disease, clinical and histopathological features, and current treatment possibilities are briefly reviewed.
AuthorsAndré Laureano, Rodrigo Carvalho, Alexandra Chaveiro, Jorge Cardoso
JournalDermatology online journal (Dermatol Online J) Vol. 20 Issue 1 Pg. 21245 (Jan 15 2014) ISSN: 1087-2108 [Electronic] United States
PMID24456948 (Publication Type: Case Reports, Journal Article, Review)
Chemical References
  • Dapsone
  • Colchicine
Topics
  • Biopsy
  • Colchicine (therapeutic use)
  • Contraindications
  • Dapsone
  • Erythema (etiology)
  • Female
  • Glucosephosphate Dehydrogenase Deficiency (complications)
  • Heterozygote
  • Humans
  • Middle Aged
  • Panniculitis (etiology, pathology)
  • alpha 1-Antitrypsin Deficiency (complications, diagnosis, genetics, physiopathology)

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