Case Reports
doi: 10.7326/0003-4819-86-6-742.
Alpha1-antitrypsin deficiency with severe panniculitis. Report of two cases
- PMID: 301370
- DOI: 10.7326/0003-4819-86-6-742
Item in Clipboard
Case Reports
Alpha1-antitrypsin deficiency with severe panniculitis. Report of two cases
Ann Intern Med.
1977 Jun.
Abstract
Two patients with profound decrease of alpha1-antitrypsin (PiZZ) presented with severe pannicultis (Weber-Christian disease); one had systemic panniculitis including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and alpha1-antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease.
Similar articles
-
Genetic study of a family with two members with Weber Christian disease (panniculitis) and alpha 1 antitrypsin deficiency.Am J Med Genet. 1982 Sep;13(1):57-62. doi: 10.1002/ajmg.1320130110. Am J Med Genet. 1982. PMID: 6982619
-
Familial occurrence of alpha 1-antitrypsin deficiency and Weber-Christian disease.Arch Dermatol. 1983 Mar;119(3):198-202. Arch Dermatol. 1983. PMID: 6600598
-
Cyclophosphamide therapy for Weber-Christian disease associated with alpha 1-antitrypsin deficiency.South Med J. 1986 Nov;79(11):1425-7. doi: 10.1097/00007611-198611000-00024. South Med J. 1986. PMID: 3490700
-
Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature.Arch Dermatol. 1987 Dec;123(12):1655-61. Arch Dermatol. 1987. PMID: 3318708 Review.
-
Alpha-1-antitrypsin deficiency panniculitis.Dermatol Clin. 2008 Oct;26(4):447-51, vi. doi: 10.1016/j.det.2008.05.001. Dermatol Clin. 2008. PMID: 18793976 Review.
Cited by
-
alpha1-Antitrypsin deficiency . 6: new and emerging treatments for alpha1-antitrypsin deficiency.Thorax. 2004 Oct;59(10):904-9. doi: 10.1136/thx.2003.006551. Thorax. 2004. PMID: 15454659 Free PMC article. Review.
-
Weber-Christian panniculitis with systemic cytophagic histiocytosis.Klin Wochenschr. 1988 Apr 15;66(8):365-72. doi: 10.1007/BF01735796. Klin Wochenschr. 1988. PMID: 3392893
-
Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review.Int J Surg Case Rep. 2016;28:223-226. doi: 10.1016/j.ijscr.2016.09.019. Epub 2016 Sep 28. Int J Surg Case Rep. 2016. PMID: 27736709 Free PMC article.
-
Weber-Christian panniculitis and chronic active hepatitis.Eur J Pediatr. 1986 Aug;145(3):227-9. doi: 10.1007/BF00446074. Eur J Pediatr. 1986. PMID: 3769982
-
Relationship between Weber-Christian panniculitis and the ZZ phenotype of alpha1-antitrypsin.Arch Dermatol Res. 1985;277(4):328-9. doi: 10.1007/BF00509091. Arch Dermatol Res. 1985. PMID: 3873915 No abstract available.
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources
Research Materials
Miscellaneous
