Literature DB >> 15987301

Alpha1-antitrypsin deficiency-associated panniculitis: case report and review of treatment options.

P G Ortiz1, B G Skov, E Benfeldt.   

Abstract

Alpha(1)-antitrypsin deficiency, a relatively frequent mutation in the population, is associated with the development of panlobular emphysema and liver cirrhosis. The deficiency is in rare cases associated with the development of panniculitis, and very differentiated clinical courses have been reported in the literature. We report a case of panniculitis in a patient with alpha(1)-antitrypsin deficiency and describe briefly the pathophysiology of the disease and current treatment possibilities.

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Year:  2005        PMID: 15987301     DOI: 10.1111/j.1468-3083.2005.01194.x

Source DB:  PubMed          Journal:  J Eur Acad Dermatol Venereol        ISSN: 0926-9959            Impact factor:   6.166


  5 in total

1.  Severe postoperative wound healing disturbance in a patient with alpha-1-antitrypsin deficiency: the impact of augmentation therapy.

Authors:  Marionna Cathomas; Alexandra Schüller; Daniel Candinas; Roman Inglin
Journal:  Int Wound J       Date:  2015-03-26       Impact factor: 3.315

2.  [Panniculitis of the thigh and lung emphysema in a 54-year-old patient].

Authors:  M Eckhard; K P Kröll; T Karrasch; F Roller; U Feek; U Lange; M Noethe; W Seeger; S Ghofrani; A Schäffler
Journal:  Internist (Berl)       Date:  2015-06       Impact factor: 0.743

3.  New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy.

Authors:  B Gross; M Grebe; M Wencker; J K Stoller; L M Bjursten; S Janciauskiene
Journal:  Dermatology       Date:  2009-02-16       Impact factor: 5.366

Review 4.  ELANE mutations in cyclic and severe congenital neutropenia: genetics and pathophysiology.

Authors:  Marshall S Horwitz; Seth J Corey; H Leighton Grimes; Timothy Tidwell
Journal:  Hematol Oncol Clin North Am       Date:  2012-11-07       Impact factor: 3.722

5.  Cutaneous Manifestation of Alpha-1 Antitrypsin Deficiency: A Case of Panniculitis.

Authors:  Sofia Lopes; Carla Damas; Filomena Azevedo; Alberto Mota
Journal:  Indian J Dermatol       Date:  2018 Jul-Aug       Impact factor: 1.494

  5 in total

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