Skip to main content
SpringerLink
Log in
Book cover

Behçet’s Disease pp 129–136Cite as

Dermatologic Manifestation of Behçet’s Disease

  • Chapter

  • 182 Accesses

Abstract

Dermatologic lesions in Behçet’s disease are regarded as important manifestation for diagnosis. Various kinds of cutaneous lesions appear in patients with Behçet’s disease. They present as erythema nodosum-like lesion, papulopustular eruption, erythema multiforme-like lesion, thrombophlebitis, skin ulcer, Sweet’s syndrome-like lesion, bullous necrotizing vasculitis, and pyoderma gangrenosum. The hyperreactivity of the skin to any intracutaneous injection or needle prick, which is known as pathergy, is one clinically-unique feature of the disease.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Altac M, Tüzün Y, Yurdakul S, Binyildiz P, Yazici H: The validity of the pathergy test (non-specific skin hyperreactivity) in Behcet’s disease: a double-blind study by independent observers. Acta Derm Venereol (Stockh) 62: 158–159,1982

    CAS  Google Scholar 

  • Anonymous: Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet 335: 1078–1080,1990

    Google Scholar 

  • Aral O, Dilsen N, Konice M: Positive skin pathergy reactivity as a genetic marker of Behcet’s disease. Paper presented at The Fourth International Conference on Behcet’s Disease. London, Royal Society of Medicine, 1985,173–175

    Google Scholar 

  • Arbesfeld SJ, Kurban AK: Behcet’s disease. New perspectives on an enigmatic Syndrome. J Am Acad Dermatol 19: 767–779,1988

    Article  PubMed  CAS  Google Scholar 

  • Bang D, Honma T, Saito T, Nakagawa S, Ueki H, Lee S: The pathogenesis of vascular changes in erythema nodosumlike lesions of Behçet’s Syndrome: an electron microscopic study. Hum Pathol 18: 1172–1179,1987

    Article  PubMed  CAS  Google Scholar 

  • Bang D, Honma T, Saito T, Nakagawa S, Ueki H, Lee S: Ultrastructure of vascular changes in cutaneous manifestations of Behcet’s disease. Acta Derm Venereol (Stockh) 68: 33–40,1988

    CAS  Google Scholar 

  • Bang D, Sohn S, Lee S: The possible role of fat cells in the pathogenesis of erythema nodosum-like lesions of Behcet’s disease: an immunoelectron microscopic study. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV 1993,371–374

    Google Scholar 

  • Behcet’s Disease Research Committee of Japan: Behcet’s disease: guide to diagnosis of Behcet’s disease. Jpn J Ophthalmol 18:291–229,1974

    Google Scholar 

  • Benamour S, Tak-Tak MT, Rafik M, Amraoui A: Juvenile Behcet’s disease in Morocco. Rev Rhum Engl Ed 63: 536, 1996

    Google Scholar 

  • Blobner F: Zur rezidivierenden hypopyon-iritis. Zeitschrift Augenheik 91: 129–139,1937

    Google Scholar 

  • Budak-Alpdogan T, Alpdogan, Demiray Z, Ergun T, Direskeneh H, Öztürk A, Bayik M, Akoglu T: Positive skin pathergy reaction in patients with chronic myelogenous leukemia. Rev Rhum Engl Ed 63: 553,1997

    Google Scholar 

  • Chajek T, Fainaru M: Behcet’s disease. Report of 41 cases and a review of the literature. Medicine (Baltimore) 54: 179–196,1975

    Article  CAS  Google Scholar 

  • Chams C, Davatchi F, Sharam F, Akbarian M, Gharibdoost F, Nadji A: Longitudinal study of the pathergy phenomenon in Behcet’s disease. Rev Rhum Engl Ed 63: 554,1996

    Google Scholar 

  • Chams C, Mansoori P, Shahram F, Akbarian M, Gharibdoost F, Davatchi F: Iconography of mueocutaneous lesions of Behçet’s disease. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993,359–362

    Google Scholar 

  • Chen K-R, Kawahara Y, Miyakawa S, Nishikawa T: Cutaneous vasculitis in Behcet’s disease: A clinical and histopathologic study of 20 patients. J Am Acad Dermatol 36: 689–696,1997

    Article  PubMed  CAS  Google Scholar 

  • Cho KH, Shin KS, Sohn SJ, Choi SJ, Lee YS: Behcet’s disease with Sweet’s syndrome-like presentation-a report of six cases. Clin Exp Dermatol 14: 20–24,1989

    Article  PubMed  CAS  Google Scholar 

  • Chun SI, Su WPD, Lee S, Rogers RS III: Erythema nodosumlike lesions in Behcet’s Syndrome: a histopathologic study of 30 cases. J Cutan Pathol 16: 259–265,1989

    Article  PubMed  CAS  Google Scholar 

  • da Cunha RM, Horta AB, Castro AS: [Behcet’s disease. Experience of an internal medicine Service from 1982 to 1991]. Acta Med Port 5: 571–574,1992

    PubMed  Google Scholar 

  • Davatchi F, Shahram F, Akbarian M, Gharibdoost F, Chams C, Chams H, Mansoori P, Nadji A: Classification tree for the diagnosis of Behçet’s disease. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993,245–248

    Google Scholar 

  • Davies PG, Fordham JN, Kirwan JR, Barnes CG, Dinning WJ: The pathergy test and Behcet’s Syndrome in Britain. Ann Rheum Dis 43: 70–73,1984

    Article  PubMed  CAS  Google Scholar 

  • Dilsen N, Konice M, Aral O: Our diagnostic criteria of Behcet’s disease-an overview. Paper presented at The Fourth International Conference on Behcet’s Disease. London, Royal Society of Medicine, 1985a, 177–180

    Google Scholar 

  • Dilsen N, Konice M, Aral O, Aykut S: Standardization and evaluation of the skin pathergy test in Behcet’s disease and controls. Paper presented at The Fourth International Conference on Behçet’s Disease. London, Royal Society of Medicine, 1985b, 169–172

    Google Scholar 

  • Dilsen N, Konice M, Aral O, Inanc M, Gul A, Ocal L: Important imphcations of skin pathergy test in Behcet’s disease. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993,229–233

    Google Scholar 

  • Djawari D, Hornstein OP, Scholz J: Enhancement of granulocyte Chemotaxis in Behçet’s disease. Arch Dermatol Res 270:81–88,1981

    Article  PubMed  CAS  Google Scholar 

  • Endoh M, Yokozeki H, Maruyama R, Matsunaga T, Katarama I, Nishioka K: Incontinentia pigmenti and Behcet’s disease: A case of impaired neutrophil Chemotaxis. Dermatology 192: 285–287,1996

    Article  PubMed  CAS  Google Scholar 

  • Fellner MJ, Kantor I: Behcet’s Syndrome: Skin puncture test as guide in therapy. N Y State J Med 64: 1760–1761,1964

    PubMed  CAS  Google Scholar 

  • Fresko I, Yazici H, Bayramicli M, Yurdakul S, Mat C: Effect of surgical cleaning of the skin on the pathergy phenomenon in Behcet’s Syndrome. Ann Rheum Dis 52: 619–620, 1993

    Article  PubMed  CAS  Google Scholar 

  • Friedman-Birnbaum R, Bergman R, Alzen E: Sensitivity and specificity of pathergy test resuhs in Israeli patients with Behcet’s disease. Cutis 45: 261–264,1990

    PubMed  CAS  Google Scholar 

  • Fujikawa S, Suemitsu T: Bebtet disease in children: a nationwide retrospective survey in Japan. Acta Paediatr Jpn 39: 285–289,1997

    Article  PubMed  CAS  Google Scholar 

  • Gamble CN, Wiesner KB, Shapiro RF, Boyer WJ: The immune complex pathogenesis of glomerulonephritis and pulmonary vascuhtis in Behcet’s disease. Am J Med 66: 1031–1039,1979

    Article  PubMed  CAS  Google Scholar 

  • Gharibdoost F, Davatchi F, Shahram F, Akbarian M, Chams C, Chams H, Mansoori P, Nadji A: Clinical manifestations of Behçet’s disease in Iran analysis of 2176 cases. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993, 153–158

    Google Scholar 

  • Ghayad E, Tohme A: Bebtet’s disease in Lebanon: report of 100 cases. J Med Liban 43: 2–7,1995

    PubMed  CAS  Google Scholar 

  • Gül A, Esin S, Dilsen N, Konie M, Wigzell H, Biberfeld P: Immunohistology of skin pathergy reaction in Behcet’s disease. Paper presented at the Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV 1993, 343–347

    Google Scholar 

  • Haim S, Sobel JD, Friedman-Birnbaum R, Lichtig C: Histological and direct immunofluorescence study of cutaneous hyperreactivity in Behcet’s disease. Br J Dermatol 95: 631–636,1976

    Article  PubMed  CAS  Google Scholar 

  • Hamza M, Cheour I, Sammari H: The skin pathergy test in Behçet’s disease, connectivite and spondyloarthropathy. Rev Rhum Engl Ed 63: 553,1997

    Google Scholar 

  • Honma T, Bang D, Saito T, Nakagawa S, Ueki H, Lee S: Ultrastructure of lymphocyte-mediated fat-cell lysis in erythema nodosum-like lesions of Behcet’s Syndrome. Arch Dermatol 123:1650–1654,1987

    Article  PubMed  CAS  Google Scholar 

  • Hunt SJ, Santa-Cruz DJ: Neutrophilic dermatoses. Semin Dermatol 8: 266–275,1989

    PubMed  CAS  Google Scholar 

  • Inoue C, Itoh R, Kawa Y, Mizoguchi M: Pathogenesis of mueocutaneous lesions in Behcet’s disease. J Dermatol 21: 474–480,1994

    PubMed  CAS  Google Scholar 

  • Jadassohn W, Franceschetti A, Hunziker N: Zur „Behcet in reaction”. Hautarzt 12: 64–65,1961

    PubMed  CAS  Google Scholar 

  • Jensen T: Sur les ulcerations aphteuses de la muqueues de la bouche et de la peau genitale combines avec les symptomes oculaires (Syndrome Behcet. Acta Dermatol Venereol 22: 64–79,1941

    Google Scholar 

  • Jorizzo JL: Behcet’s disease. An update based on the 1985 International Conference in London. Arch Dermatol 122: 556–558,1986

    Article  PubMed  CAS  Google Scholar 

  • Jorizzo JL, Hudson RD, Schmalstieg FC, Daniels JC, Apisarnthanarax P, Henry JC, Gonzalez EB, Ichikawa Y, Cavallo T: Behcet’s Syndrome: immune regulation, circulating immune complexes, neutrophil migration, and colchicine therapy. J Am Acad Dermatol 10: 205–214,1984

    Article  PubMed  CAS  Google Scholar 

  • Jorizzo JL, Schmalstieg FC, Solomon ARJ, Cavallo T, Taylor RS III, Rudioff HB, Schmalstieg EJ, Daniels JC: Thahdomide effects in Behçet’s Syndrome and pustular vasculitis. Arch Intern Med 146: 878–881,1986

    Article  PubMed  CAS  Google Scholar 

  • Jorizzo JL, Solomon AR, Cavallo T: Behcet’s Syndrome. Immunopathologic and histopathologic assessment of pathergy lesions is useful in diagnosis and follow-up. Arch Pathol Lab Med 109: 747–751,1985

    PubMed  CAS  Google Scholar 

  • Jorizzo JL, Solomon AR, Zanolli MD, Leshin B: Neutrophihc vascular reactions. J Am Acad Dermatol 19: 983–1005,1988

    Article  PubMed  CAS  Google Scholar 

  • Katzenehenbogen I: Survey of 22 cases of Behçet’s Disease: the significance of specific skin hyperreactivity. Paper presented at The 13th Congress of International Dermatology. Munich, Springer-Verlag, 1967, 321–324

    Google Scholar 

  • Kienbaum S, Zoubouhs CC, Waibel M, Orfanos CE: Chemotactic neutrophilic vasculitis: a new histopathological pattern of vasculitis found in mueocutaneous lesions of patients with Adamantiades-Behcet’s disease. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993,337–341

    Google Scholar 

  • Kim D-K, Chang SN, Bang D, Lee E-S, Lee S: Clinical analysis of 40 cases of childhood-onset Behcet’s disease. Pediatr Dermatol 11:95–101,1994

    Article  PubMed  CAS  Google Scholar 

  • Kim HJ, Bang D, Lee SH, Yang DS, Kim DH, Lee KH, Lee S, Kim HB, Hong WP: Behcet’s Syndrome in Korea: a look at the clinical picture. Yon Sci Med J 29: 72–78,1988

    CAS  Google Scholar 

  • Kone-Paut I, Bernard JL: [Behcet disease in children in France]. Arch Fr Pediatr 50: 561–565,1993

    PubMed  CAS  Google Scholar 

  • Lakhanpal S, O’Duffy JD, Lie JT: Pathology. In Plotkin GR, Calabro JJ, O’Duffy JD, eds. Behcet’s disease: a contemporary Synopsis. Mount Kisco, New York, Futura Pub. Co Inc, 1988, 101–142

    Google Scholar 

  • Lakhanpal S, Tani K, Lie JT, Katoh K, Ishigatsubo Y, Ohokubo T: Pathologic features of Behcet’s Syndrome: a review of Japanese autopsy registry data. Hum Pathol 16: 790–795,1985

    Article  PubMed  CAS  Google Scholar 

  • Lee E-S, Lee SH, Bang D, Lee S: Sweet’s syndrome-like skin lesions in Behcet’s Syndrome: an additional cutaneous manifestation. Paper presented at the The Fifth International Conference on Behçet’s Disease. Minnesota, Rochester, Marcel Dekker, Inc, 1989a, 223–227

    Google Scholar 

  • Lee S: Chair’s summary; Behçet’s disease. Paper presented at the The 18th World Congress of Dermatology. New York, Parthenon Publishing Company 1992,1073–1076

    Google Scholar 

  • Lee S, Lee E-S, Bang D, Lee SH, Hong C-S: Behçet’s Syndrome and systemic lupus erythematosus: a case analysis. Paper presented at The Fifth International Conference on Behcet’s Disease. Minnesota, Rochester, Marcel Dekker, Inc, 1989b, 243–245

    Google Scholar 

  • Lee SH, Chung KY, Lee WS, Lee S: Behcet’s Syndrome associated with bullous necrotizing vascuhtis. J Am Acad Dermatol 21:327–330,1989c

    Article  CAS  Google Scholar 

  • Lee WS, Kim SJ, Ahn SK: Behcet’s disease as a part of the Symptom complex of SLE? J Dermatol 23: 196–199,1996

    PubMed  CAS  Google Scholar 

  • Mangelsdorf HC, White WL, Jorizzo JL: Behcet’s disease. J Am Acad Dermatol 34: 745–750,1996

    Article  PubMed  CAS  Google Scholar 

  • Mansoori P, Chams C, Davatchi F, Shahram F, Akbarian M, Gharibdoost F, Nadji A, Chams H: Relationship of pathergy phenomenon and Behçet’s disease (BD) manifestations. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993,367–369

    Google Scholar 

  • Matsumura N, Matsumura Y, Mizushima Y: Studies on cutaneous hyperreactivity and leukocyte Chemotaxis in Behcet’s disease. Paper presented at The Second International Symposium on Behçet’s Disease. Istanbul, Excerpta Medica, Turkey. 1977,219–223

    Google Scholar 

  • Menni S, Piccinno R, Bioichini A, Delle-Piane RM, Bardare M: Incontinentia pigmenti and Behcet’s Syndrome: An unusual combination. Acta Derm Venereol (Stockh) 66: 351–354, 1986

    CAS  Google Scholar 

  • Mizoguchi M, Chikakane K, Goh K, Asahina Y, Masuda K: Acute febrile neutrophihc dermatosis (Sweet’s Syndrome) in Behcet’s disease. Br J Dermatol 116: 727–734,1987

    Article  PubMed  CAS  Google Scholar 

  • Mizoguchi M, Matsuki K, Mochizuki M, Watanabe R, Ogawa K, Harada S, Hino H, Amagai M, Juji T: Human leukocyte antigen in Sweet’s Syndrome and its relationship to Behcet’s disease. Arch Dermatol 124:1069–1073,1988

    Article  PubMed  CAS  Google Scholar 

  • Mizushima Y, Inaba G, Mimura Y, Ono S: [Diagnostic criteria for Behcet’s disease in 1987, and guidehne for treating Behcet’s disease]. Saishin Igaku 43: 391–393,1988

    Google Scholar 

  • Mochizuki M, Morita E, Yamamoto S, Yamana S: Characteristics of T ceü hnes established from skin lesions of Behcet’s disease. J Dermatol Sci 15: 9–13,1997

    Article  PubMed  CAS  Google Scholar 

  • Muller W, Lehner T: Quantitative electron microscopy microscopical analysis of leukocyte infihration in oral ulcers of Behcet’s Syndrome. Br J Dermatol 106: 535–544,1982

    Article  PubMed  CAS  Google Scholar 

  • Nazzaro P: Cutaneous manifestations of Behcet’s disease. Paper presented at the International Symposium on Behcet’s Disease. Rome, Karger, 1964,15–41

    Google Scholar 

  • O’Duffy JD: Summary of International Symposium on Behcet’s Disease. Istanbul, September 29–30, 1977. J Rheumatol 5: 229–233,1978

    PubMed  Google Scholar 

  • O’Duffy JD, Carney JA, Deodhar S: Behcet’s disease. Report of 10 cases, 3 with new manifestations. Ann Intern Med 75: 561–570,1971

    PubMed  Google Scholar 

  • Oguz O, Serdaroglu S, Tuzun Y, Erdogan N, Yazici H, Savaskan H: Acute febrile neutrophihc dermatosis (Sweet’s Syndrome) associated with Behcet’s disease. Int J Dermatol 31: 645–646,1992

    Article  PubMed  CAS  Google Scholar 

  • Poweh FC, Schroeter AL, Su WPD, Perry HO: Pyoderma gangrenosum: A review of 86 patients. Q J Med 217: 173–186, 1985

    Google Scholar 

  • Price CA: Behcet’s syndrome-a distressing, intriguing malady. J R Coll Gen Pract 18: 38–45,1969

    PubMed  CAS  Google Scholar 

  • Reimer G, Luckner L, Hornstein OP: Direct immunofluorescence in recurrent aphthous ulcers and Behcet’s disease. Dermatologica 167: 293–298,1983

    Article  PubMed  CAS  Google Scholar 

  • Scherrer MA, Vitrai N, Bambirra E, Orefice F: Histologic and clinical study of pathergy in Behcet’s disease in Brazil. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993,363–366

    Google Scholar 

  • Shafaie N, Shahram F, Nadji A, Davatchi F, Akbarian M, Gharibdoost F, Jamshidi A: Iran’s aspects of Behcet’s disease in children, the 1996 survey. Rev Rhum Engl Ed 63: 535,1996

    Google Scholar 

  • Shi H-L, Huang Z-J: Study on cutaneous lesions in Behcet’s disease and meanings of relative laboratory parameters. Paper presented at The Sixth International Conference on Behcet’s Disease. Paris, Elsevier Science Publishers BV, 1993,325–330

    Google Scholar 

  • Shimizu T: Clinicopathological studies on Behcet’s disease. Paper presented at the The Second International Symposium on Behcet’s Disease. Istanbul, Excerpta Medica, 1977,9–43

    Google Scholar 

  • Shimizu T, Ehrlich GE, Inaba G, Hayashi K: Bebtet disease (Bebtet Syndrome). Semin Arthritis Rheum 8: 223–260, 1979

    Article  PubMed  CAS  Google Scholar 

  • Suzuki K, Mizuno N: Intracutaneous test with physiological sahne in Behcet’s disease. Paper presented at The Third International Conference on Behcet’s Disease. Tokyo, University of Tokyo Press, 1981,333–342

    Google Scholar 

  • Tüzün Y, Akac M, Yazici H, Basoz A, Yurdakul S, Pazarh H, Yalcin B, Muftuoglu A: Nonspecific skin hyperreactivity in Behcet’s disease. Haematologica 65: 395–398,1980

    PubMed  Google Scholar 

  • Tüzün Y, Yazici H, Pazarli H, Yalcin B, Yurdakul S, Muftuoglu A: The usefulness of the nonspecific skin hyperreactivity (the pathergy test) in Behcet’s disease in Turkey. Acta Derm Venereol (Stockh) 59: 77–79,1979

    Google Scholar 

  • Uziel Y, Padeh S, Brik R, Barash J, Mukamel M, Harel L, Press J, Tauber Z, Rakover Y, Wolach B: Juvenile Behcet’s in Israel. Rev Rhum Engl Ed 63: 535,1996

    Google Scholar 

  • Wechsler J, Wechsler B, Revuz J, Godeau P: Skin hyperreactivity response (pathergy) in Behcet’s disease: usefulness of direct immunofluorescence [letter; comment]. J Am Acad Dermatol 23: 329–330,1990

    Article  PubMed  CAS  Google Scholar 

  • Wong RC, Elhs CN, Diaz LA: Behcet’s disease. Int J Dermatol 23:25–32,1984

    Article  PubMed  CAS  Google Scholar 

  • Yurdakul S, Mat C, Tüzün Y, Tüzüner N, Uygun N, Hekim N, Küsem V, Tüzün B, Hamuryudan V, Özyazgan Y, Yazici H: Histochemical examination of papulopustular manifestations of Behcet’s disease. Rev Rhum Engl Ed 63: 553,1996

    Google Scholar 

  • Yücel A, Özbek S, Boga H, Acar MA, Aksungur VL, Memisoglu HR: Pernio-like skin lesions in a patient with Behcet’s disease. Rev Rhum Engl Ed 63: 554,1996

    Google Scholar 

  • Zgradic I: [Behcet’s disease-clinical picture and treatment]. Med Pregl 49: 233–236,1996

    PubMed  CAS  Google Scholar 

  • Zouboulis CC: [Adamantiades-Behçet’s disease in Germany: Historical review and current knowledge]. H+G 71: 491–501,1996

    Google Scholar 

Download references

Authors
  1. Eun-So Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Dongsik Bang
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sungnack Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 2001 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Lee, ES., Bang, D., Lee, S. (2001). Dermatologic Manifestation of Behçet’s Disease. In: Behçet’s Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-56455-0_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-56455-0_19

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-63094-1

  • Online ISBN: 978-3-642-56455-0

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation