Abstract
A 22-year-old male patient with Weber–Christian disease (WCD) presenting with periorbital swelling is a very rare occurrence. Lobular panniculitis in the periorbital and anterior tibial skin was diagnosed in this patient, based on the histological findings from biopsy specimens. The differential diagnosis was done before administering systemic corticosteroid. A dramatic response was achieved several days later, but relapse with intractable fever occurred 1 month later. The use of other immunosuppressive agents failed, and he died of septic shock 2 months later. The preceding infection may have been the immunopathologic factor in the development of WCD. Other effective treatments for WCD may be described in the future.
References
McNutt NS, Moreno A, Contreras F (1997) Inflammatory disease of the subcutaneous fat. In: Eldev DE, et al (eds) Lever’s Histopathology of the skin, 8th edn. Lippincott–Raven, Philadelphia, 429–455
Requena L, Sanchez Yus E (2001) Panniculitis: Part II. Mostly lobular panniculitis. J Am Acad Dermatol 45:325–361
Yoshida T, Ito H, Matsubara Y, Kitumura Y, Sasaoka A, Chijiwa T, Hashimoto K, Komatsu T, Mokiri T, Itahara T (2003) Weber–Christian disease presenting with ocular manifestations. Clin Rheumatol 22:339–342
Panush PS, Yonker RA, Dlesk A, Longley S, Caldwell JR (1985) Weber–Christian disease. Analysis of 15 cases and review of the literature. Medicine 64:181–191
Iwasaki T, Hamano T, Ogata A, Hashimoto N, Kakishita E (1999) Successful treatment of a patient with febrile, lobular panniculitis (Weber–Christian disease) with oral cyclosporin A: implications for pathogenesis and therapy. Intern Med 38:612–614
Ter Poorten Mc, Thiers Bh (2002) Panniculitis. Dermatol Clin 20:421–433
Martin RJ, Michals EL, Voth MR (1977) Cyclophosphamide-induced remission in Weber–Christian disease: case report. Mil Med 142:158
Enk AH, Knop J (1988) Treatment of relapsing idiopathic nodular panniculitis (Pfeifer–Weber–Christian disease) with mycophenolate mofetil. J Am Acad Dermatol 39:508
Hinata M, Someya T, Yoshizaki H, Seki K, Takeuchi K (2005) Successful treatment of steroid-resistant Weber–Christian disease with biliary ductopenia using cyclosporin A. Rheumatology (Oxford) 44:821–382
Kovacs M, Hafner J, Gabor V, Sipos J (2004) Successful treatment of Weber–Christian panniculitis with cyclosporin-A. Orv Hetil 145:827–831
Ostrov BE, Atherya BH, Eichenfield AH, Goldsmith DP (1996) Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporine A. Semin Arthritis Rheum 25:404–413
Humeniuk JM, Gliptis EG (July 1–5, 2002) Infliximab in the treatment of panniculitis. World Congress of Dermatology
Beutler BA, Milsark IW, Cerami A (1985) Cachectin/tumor necrosis factor: production, distribution, and metabolic fate in vivo. J Immunol 135:3972–3977
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Wang, H.P., Huang, C.C., Chen, C.H. et al. Weber–Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema. Clin Rheumatol 26, 1002–1004 (2007). https://doi.org/10.1007/s10067-006-0235-0
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DOI: https://doi.org/10.1007/s10067-006-0235-0