Weber-Christian disease: a case report

Česká verzia

Authors: Kristína Brázdilová ¹; Daniel Čierny ¹; Katarína Hrubišková ¹; Lukáš Plank ²; Zdenko Killinger ¹; Juraj Payer ¹
Authors place of work: 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 the journal: Vnitř Lék 2018; 64(10): 961-965
Category: Kazuistika

Summary

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

Zdroje

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>.