Weber-Christian disease: a case report
Authors:
Kristína Brázdilová 1; Daniel Čierny 1; Katarína Hrubišková 1; Lukáš Plank 2; Zdenko Killinger 1; Juraj Payer 1
Authors‘ workplace:
V. interná klinika LF UK a UNB, Nemocnica Ružinov, Bratislava, Slovenská republika
1; Ústav patologickej anatómie JLF UK a UNM, Martin, Slovenská republika
2
Published in:
Vnitř Lék 2018; 64(10): 961-965
Category:
Case Report
Overview
Weber-Christian disease is a rare disease from the group of chronic fibrosing conditions characterized by inflammation of the adipose tissue – panniculitis and fibrosing with frequent systemic manifestations. Etiopathogenesis of the disease is not fully known, participation of autoimmune mechanisms is anticipated. Here, we report a case of a patient with this rare disease, diagnosed after a long and demanding diagnostic process, including repeated laparotomies. However, after immunosuppressive therapy, clinical and laboratory symptomatology improved rapidly as well as the patient’s quality of life.
Key words:
panniculitis – sclerosing mesenteritis – Weber-Christian disease
Sources
- Requena L, Sanchez YE. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 2001; 45(3): 325–361; quiz 362–364.
- Christian HA. Relapsing febrile nodular nonsuppurative panniculitis. Arch Internal Med 1928; 42: 338–351.
- Milner RDG, Mitchinson H. Systemic Weber-Christian disease. J Clin Path 1965; 18: 150–156.
- George V, Tammisetti VS, Surabhi VR et al. Chronic fibrosing conditions in abdominal imaging. Radiographics 2013; 33(4): 1053–1080. Dostupné z DOI: <http://dx.doi.org/10.1148/rg.334125081>.
- Miranda-Bautista J, Fernández-Simón A, Pérez-Sánchez I et al. Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. World J Gastroenterol 2015; 21(17): 5417–5420. Dostupné z DOI: <http://dx.doi.org/10.3748/wjg.v21.i17.5417>.
- Horton KM, Lawler LP, Fishman EK. CT findings in sclerosing mesenteritis (panniculitis): spectrum of disease. Radiographics 2003; 23(6): 1561–1567. Dostupné z DOI: <http://dx.doi.org/10.1148/rg.1103035010>.
- Neild GH, Rodriquez-Justo M, Wall C et al. Hyper-IgG4 disease: report and a characterisation of a new disease. BMC Med 2006; 4: 23. Dostupné z DOI: <http://dx.doi.org/10.1186/1741–7015–4-23>.
- Conway SP, Smithells RW, Peters WM. Weber-Christian panniculitis. Ann Rheum Dis 1987; 46(4): 339–341.
- Irami AF, Cesar de CG, de Oliveira SA et al. Sclerosing mesenteritis – update on diagnostic and therapeutic approach. Transl Biomed 2016; 7: 1. Dostupné z DOI: <http://dx.doi.org/10.21767/2172–0479.100048>.
- Gu GL, Wang SL, Wei XM et al. Sclerosing mesenteritis as a rare cause of abdominal pain and intraabdominal mass: a cases report. Cases Journal 2008; 1(1): 242. Dostupné z DOI: <http://dx.doi.org/10.1186/1757–1626–1-242>.
- Negalur VG, Negalur BV. Weber Christian Disease. J Assoc Physicians India 2003; 51: 724–725.
- Hamrick-Turner J, Chiechi M, Abbitt P et al. Neoplastic and inflammatory processes of the peritoneum, omentum and mesentery: diagnosis with CT. Radiographics 1992; 12(6): 1051–1068. Dostupné z DOI: <http://dx.doi.org/10.1148/radiographics.12.6.1439011>.
- Agostinis P, Scott CA. Weber-Christian disease: ultrasound can see it. Ital J Med 2014; 8(1): 69–75. Dostupné z DOI: <https://doi.org/10.4081/itjm.2014.170>.
Labels
Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
2018 Issue 10
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