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2005, Number 1

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Dermatología Cosmética, Médica y Quirúrgica 2005; 3 (1)

Behçet´s disease: Report of 14 cases in two hospitals of Brasília, Brazil

Carvalho CIM, Barbosa KL, Veloso D, da Silva SW, Correa LFA

Language: Portugu?s
References: 33
Page: 27-33
PDF size: 222.83 Kb.


ABSTRACT

Background: Behçet’s disease is an episodic disorder of unknown etiology. It presents a chronic course with periods of acute exacerbations. Its diagnosis is made based on clinical criteria in wich the most important are: recurrent oral and genital ulcerations and panuveitis.
Objective: Analysis of the clinical and epidemiological data and treatment of Behçet disease in two hospitals in Brasília-DF, Brazil.
Methods: Retrospective study of fourteen patients with diagnosis of Behçet’s disease who fulfilled the O’Duffy criteria and the International Study Group for Behçet´s disease. The analysis was proceeded on the clinical and epidemiological data, histopathology, treatment, morbidity and mortality among those patients from 1990 to 2000.
Results: There were no predominance of sex and race. From the 14 patients, ten ranged from 20 and 30 years of age. Nine patients had both diagnostic criteria while five only one of them. The clinical manifestations observed were: ocular disease in 10/14 patients, being anterior uveitis the most common form of presentation. Cutaneous lesions in 10/14 patients. Osteoarticular disease in 8/14 patients. Two (2/14) patients presented neurological involvement. The histopathological study of skin lesions specimens showed a neutrofilic vasculitis in the nine patients in which the biopsy was done. Sequelae: Two patients became blind and one patient progressed to dislalia. There were no deaths among the 14 patiens.Treatment was individualized according to the severity of the disease. The best results were observed with thalidomide. Immunesupressive agents and corticosteroids were used in multisystem disease and in severe ocular involvement.
Conclusion: Behçet’s disease is multisystemic disease, presenting with considerable morbidity especially with ocular disease and central nervous system involvement.


REFERENCES

  1. International Study Group for Behçet’s disease. Criteria for diagnosis of Behçet’s disease. Am J Med 1976; 61: 17-18

  2. Ghate JV, Jorizzo JL. Behçet’s disease and complex aphthosis. J AM Acad Dermatol 1999; 40: 1-18

  3. O’Duffy JD, Goldstein NP. Neurologic involvement in seven patients with Behçet’s disease. Am J Med 1976; 61: 170-178

  4. Yazici H, Tuzun Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdogan H et al. Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behcet's syndrome. Ann Rheum Dis 1984; 43: 783-789

  5. O´Duffy JD. Behçet´s disease. Curr Opin Rheumatol 1994, 6: 39-43

  6. Önder M, Gürer MA. The multiple faces of Behcet´s disease and its aetiological factors. JEADV 2001; 15: 126-136

  7. Langford CA. Immunologic Disorders, Vasculitis. J Allergy Clin Immunol 2003, 111: S602-612

  8. Hirohata T, Kuratsune M, Nomura A, Jimi S. Prevalence of Behcet's syndrome in Hawaii with particular reference to the comparison of the Japanese in Hawaii and Japan. Hawaii Med J 1975; 34: 244-248

  9. Scherrer MAR. Doença de Behçet: Revisão baseada na 6a Conferência Internacional – Paris, 1993. An bras Dermatol, 1996; 71: 35-42

  10. Scherrer MAR. Doença de Behçet: um enigma oriental. An bras Dermatol 1994; 69: 323-326

  11. Garrote GFD; Costa MB, Borges PC; Rassi DM. Doença de Behçet. An bras Dermatol, 1995; 70: 325-330

  12. Jorizzo JL. Behçet’s disease. Neurol Clin 1987; 5: 427-440

  13. Mangelsdorf HC, White WL, Jorizzo JL. Behçet’s disease: Report of twenty-five patients from the United States with prominent mucocutaneous involvement. J AM Acad Dermatol 1996; 745-750

  14. Mamo JG, Azzam SA. The rate of visual loss in Behcet’s. Arch Ophthalmol 1970; 84: 451-452

  15. Wolf SM, Schotland DL, Phillips LL. Involvement of nervous system in Behcet's syndrome. Arch Neurol 1965; 12: 315-325

  16. Jorizzo JL, Abernethy JL, White WL, Mangelsdorf HC, Zouboulis CC, Sarica R et al. Mucocutaneous criteria for the diagnosis of Behcet's disease: an analysis of clinicopathologic data from multiple international centers. J Am Acad Dermatol 1995; 32: 968-976

  17. Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S et al. Thalidomide in the treatment of the mucocutaneous lesions of Behçet's syndrome: a randomized double-blinded, placebo controlled trial. Ann Intern Med 1998; 128: 443-450

  18. Benezra D, Cohen E. Treatment and visual prognosis in Behçet's disease. Br J Ophthalmol 1986; 70: 589-592

  19. Kidd D. Neurological complications of Behçet´s syndrome. ACNR 2003; 3: 8-11

  20. Jorizzo JL, Hudson RD, Schmalstieg FC, Daniels JC, Apisarnthanarax P, Henry JC. Behcet's syndrome: immune regulation, circulating immune complexes, neutrophil migration, and colchicine therapy. J Am Acad Dermatol 1984; 10: 205-214

  21. O'Duffy JD. “Behçet's disease”. In: Kelley WN, Harris ED, Ruddy ES, Sledge CB, (eds). Textbook of rheumatology. 2nd ed. Philadelphia: WB Saunders; 1985; 1174-1178

  22. Chajet T, Fainaru M. Behçet’s disease: report of 41 cases and a review of literature. Medicine 1975; 54: 179-196

  23. Chamberlain MA. Behçet’s syndrome in 32 pacients in Yokshire. Ann Rheum Dis 1977; 36: 491-499

  24. Mason MR, Barnes CG. Behçet’s syndrome with arthritis. Ann Rheum Dis 1969; 28: 95-103

  25. Balabanova M, Calamia KT, Perniciaro C, O’Duffy JD. A study of the cutaneous manifestations of Behçet’s disease in patients from the United States. J AM Acad Dermatol 1999; 41: 540-545

  26. Sampaio SAP, Rivitti EA. “Dermatologia”. In: Hecht Milton (ed). Inflamações não-infecciosas. 2a ed. São Paulo: Editora Artes Médicas; 2000, 647-648

  27. Jorizzo JL, Soloman AR, Cavallo T. Behcet's syndrome: immunopathological and histopathological assessment of pathergy lesions is useful in diagnosis and follow-up. Arch Pathol Lab Med 1985; 109: 747-751

  28. Miyachi Y, Tangiguchi S, Ozaki M, Horio T. Colchicine in the treatment of cutaneous manifestations of Behçet's disease. Br J Dermatol 1981; 104: 67-69

  29. Mizushima Y, Matsumura W, Mori M. Chemotaxis of leukocytes and colchicine treatment in Behçet's disease. Br J Rheumatol 1979; 6: 108-109

  30. Goldsmith SM, Jorizzo JL. “Behçet’s disease”. In: Lichtenstein LM, Fauci AS (eds). Current therapy in allergy, immunology, and rheumatology. St Louis: Mosby-Year Book, 1922: 222-230

  31. Jorizzo JL, Schmalstieg FC, Solomon AR, Carvalho T, Taylor RS, Rudloff HB et al. Thalidomide effects in Behçet’s syndrome and pustular vascullitis. Arch Intern Med 1986; 146: 878-881

  32. Gardner-Medwin JM, Smith NJ, Powell RJ. Clinical experience with thalidomide in the management of severe oral and genital ulceration in conditions such as Behçet's disease: use of neurophysiologic studies to detect thalidomide neuropathy. Ann Rheum Dis 1994; 53: 828-832

  33. Saenz A, Ausejo M, Shea B, Wells G, Welch V, Tugwell P. Pharmacotherapy for Behcet´s syndrome (Cochrane Review). In: The Cochrane Library, 2003, issue 2. Oxford: Update Software




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