Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency: an update.

@article{Blanco2016NeutrophilicPA,
  title={Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency: an update.},
  author={Ignacio Blanco and Dan Lipsker and Beatriz Lara and Sabina M. Janciauskiene},
  journal={The British journal of dermatology},
  year={2016},
  volume={174 4},
  pages={753-62}
}
Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency (AATD) is a very rare disease. Its estimated prevalence is 1 in 1000 subjects with severe AATD (usually white individuals with a Pi*ZZ genotype). It is manifested clinically by painful recurrent ulcerating subcutaneous nodules, and characterized histologically by dense infiltrates of neutrophils in the deep dermis and connective-tissue septae, with secondary lobular panniculitis. It may be the only clinical manifestation… CONTINUE READING
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