Behçet disease is a chronic relapsing systemic disease with possible life-threatening presentations. Management of this disease can be challenging and reports of the off-label use of anti-TNFα agents for the treatment of severe manifestations are increasing, with good results. The authors report a case of Behçet disease with a sudden and severe multi-systemic onset successfully treated with infliximab

Alves, R.

Amorim, I.

Pinto-Almeida, T.

Selores, M.

Pinto-Almeida, Teresa

Amorim, Isabel

Alves, Rosário

Selores, Manuela

eScholarship - University of California

A dramatic case of Behçet disease successfully treated with infliximab

Repositório Científico da Unidade Local de Saúde de Santo António

2/9/2014 A dramatic case of Behçet disease successfully treated with infliximab [eScholarship]http://escholarship.org/uc/item/0d19629q 1/4Dermatology Online JournalUC DavisFigure 5A dramatic case of Behçet disease successfully treated with infliximabTeresa Pinto-Almeida, Isabel Amorim, Rosário Alves, Manuela SeloresDermatology Online Journal 19 (4): 18 Department of Dermatology, Centro Hospitalar do Porto – Hospital de Santo António, Porto, PortugalAbstractBehçet disease is a chronic relapsing systemic disease with possible life-threatening presentations. Management of this disease canbe challenging and reports of the off-label use of anti-TNFα agents for the treatment of severe manifestations are increasing, with goodresults. The authors report a case of Behçet disease with a sudden and severe multi-systemic onset successfully treated withinfliximab.Case reportA 34-year-old woman presented in our emergency department with a 1-week history of asymptomatic cutaneous lesions and one oralaphthous ulcer, together with fever and myalgia. She was otherwise healthy and was on no regular medication. There was no history ofinfections or drugs before the development of the cutaneous lesions and she denied similar previous episodes.Figure 1 Figure 2Figure 1. Clinical features of the sparse cutaneous lesions on the thorax andabdomenFigure 2. An erythematous pustule on the right upper backFigure 3 Figure 4Figure 3. Erythematous macules and papules on the lower backFigure 4. Cutaneous lesions on the lower limbsPhysical examination revealed erythematous papules and pustules. 2-8 mm, localized on thetrunk and limbs and one aphthous ulcer on the upper lip, 6 mm in diameter. She was febrile (38°C)and there were no other findings on physical examination. Giving the systemic presentation, shewas hospitalized for investigation.2/9/2014 A dramatic case of Behçet disease successfully treated with infliximab [eScholarship]http://escholarship.org/uc/item/0d19629q 2/4Figure 5. Clinical features of theaphthous ulcer on the upper lipFigure 6 Figure 7Figure 6. Genital ulcer developing 2 days after the patient was hospitalizedFigure 7. Two aphthous ulcers on the tongue, developing duringhospitalizationFigure 8 Figure 9Figure 8. Erythema nodosum-like lesions on the lower limbsFigure 9. Highlight of a cutaneous lesion on the thighTwo days later she developed a painful genital ulcer and 2 new aphthous ulcers on the tongue. The cutaneous lesions on the lowerlimbs evolved to erythema nodosum-like nodules. She then developed intense headaches and impaired vision followed by paraparesis,left facial palsy, and loss of urinary sphincter control. At this point, she recalled having recurrent oral aphthous ulcers for the last 2years, but had never considered them to be important. Laboratory workup disclosed leukocytosis (17.32 x 10³/µL, normal 4.00-11.00)with neutrophilia (11.74 x 10³/µL, normal 2.00-7.50) and elevated C reactive protein (144.02 mg/L, normal 0.0-5.0). Serologic andimmunologic studies, blood, and urine cultures were negative.Figure 10 Figure 11Figure 10. Histopathologic examination of a skin biopsy from an erythema-nodosum-like nodules shows a normal epidermis and lymphocyticvasculitis in the dermis (H&E x4)Figure 11. Histopathologic features show the coexistence of lymphocyticvasculitis and mixed panniculitis (H&E x4)Figure 12 Figure 13Figure 12. Higher amplification of the same cutaneous lesions (H&E x20)Figure 13. Inflammatory infiltrate extends to the adnexal structures (H&Ex20)Histopathologic examination of a skin biopsy from an erythema nodosum-like nodule showed a normal epidermis, lymphocyticvasculitis in the dermis, and a mixed panniculitis with an inflammatory infiltrate extending to the adnexal structures at the dermo-2/9/2014 A dramatic case of Behçet disease successfully treated with infliximab [eScholarship]http://escholarship.org/uc/item/0d19629q 3/4hypodermal junction. Pathergy test was negative. Ophthalmologic examination detected papilledema. Cerebral MRI and lumbarpuncture revealed features of rhombencephalitis. HLA-B51 was positive. Altogether, these findings allowed the diagnosis of Behçetdisease and the patient started a 3-day course of pulse iv methylprednisolone 1g qd, followed by oral prednisone, 1 mg/kg/day,without any improvement.Figure 14 Figure 15Figure 14. Complete resolution of the aphthous ulcer on the upper lip after asingle infusion of infliximabFigure 15. Clearance of the cutaneous lesions in the thorax and abdomenwith treatmentFigure 16 Figure 17Figure 16. Resolution of the cutaneous lesions on the backFigure 17. Complete clearing of the erythema nodosum-like nodules on thelower limbs after infliximab therapyFor this reason, she started IV infliximab 5 mg/kg every 6 weeks with complete resolution of clinical disease after the first infusion andwithout sequelae. The patient remains free of the disease after one year of follow-up, still under infliximab therapy.DiscussionBehçet disease is a chronic systemic inflammatory disorder of unknown etiology. The exuberant and sudden onset of the multi-systemic involvement in our patient is uncommon and usually related with a poor prognosis [1, 2]. Along with this severe presentation,the presence of HLA-B51 and the lack of response to high-dose corticosteroids were also predictive of a worse outcome [1, 2, 3].Such a dramatic clinical picture requires aggressive immunosuppressive therapy in order to achieve rapid control of the disease andavoid irreversible damage to vital organs [1, 2, 3]. Anti-TNFα agents have been used in Behçet disease because of a role of TNFαmediated interactions in its pathogenesis [1, 4]. Among these agents, there is greater information on infliximab; 83 percent of Behçetdisease patients on anti-TNFα agents are receiving infliximab [1-3, 5, 6]. This fact, along with the documented high efficacy rates(>85%) and the rapid effect after a single infusion make it a good candidate for severe presentations resistant to standard therapies orfor those intolerant to those treatments [1-6]. For these reasons, we started infliximab in our patient and obtained a rapid andcomplete response. Since relapses are not uncommon after cessation of the anti-TNFα treatment [1, 2, 3], the patient has been kepton the same therapeutic regimen, so far without complications after 1 year of follow-up. In summary, there is sufficient scientificevidence to support the use of infliximab in severe, refractory cases of Behçet disease. At the same time we must keep in mind thatthis entity has a chronic course, with successive remissions and relapses, highlighting the importance of a long-term effective andsafe therapy. Because there are no data regarding the efficacy and safety of infliximab in Behcet disease over the long term, futurestudies are needed in order to optimize treatment protocols and clarify the role of anti-TNFα agents.References1. Aikaterini A, Fragiadaki K, Giavri E, Sfikakis PP. Anti-TNF agents for Behçet’s disease: analysis of published data on 369 patients.Semin Arthritis Rheum. 2011 Aug;41(1):61-70. [PubMed]2. Zaidan M, Lidove O, Sacré K, Klein I, Papo T. “Fulminant” Behçet disease. Presse Med. 2011 Nov;40(11):1087-9. [PubMed]3. Lestre S, Trindade F, Martins A, Baptista J, Feio AB, Lopes MJ. Behçet’s disease - Infliximab in the treatment of mucocutaneousmanifestations. Acta Reumatol Port. 2009 Oct-Dec;34(4):633-40. [PubMed]4. Sugita S, Yamada Y, Kaneko S, Horie S, Mochizuki M. Induction of regulatory T cells by infliximab in Behcet’s disease. InvestOphthalmol Vis Sci. 2011 Jan 21;52(1):476-84. [PubMed]5. Borhani Haghighi A, Safari A, Nazarinia MA, Habibagahi Z, Shenavandeh S. Infliximab for patients with neuro-Behcet’s disease:2/9/2014 A dramatic case of Behçet disease successfully treated with infliximab [eScholarship]http://escholarship.org/uc/item/0d19629q 4/4case series and literature review. Clin Rheumatol. 2011 Jul;30(7):1007-12. [PubMed]6. Abalos-Medina GM, Sánchez-Cano D, Ruiz-Villaverde G, Ruiz-Villaverde R, Quirosa Flores S, Raya Alvarez E. Successful use ofinfliximab in a patient with neuro-Behçet's disease. Int J Rheum Dis. 2009 Sep;12(3):264-6. [PubMed]© 2013 Dermatology Online Journal

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A dramatic case of Behçet disease successfully treated with infliximab

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