Behcet's syndrome

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In rheumatology, Behcet's Syndrome, or Behçet's Disease, is a rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology, although it may be an autoimmune disease and have genetic components.

The syndrome is characterized by mucocutaneous ulceration in the mouth and genital region, and uveitis with hypopyon. Recurrent oral ulcers are a key marker.[1] Other symptoms include painful, stiff and swollen joints [2]. The neuro-ocular form may cause blindness and death. Synovitis; thrombophlebitis; gastrointestinal ulcerations; retinal vasculitis;optic atrophy and meningitis may occur as well."[3]

A variation may be Hughes-Stovin Syndrome which is pulmonary artery aneurysms associated with systemic thrombosis.[4][5]

Epidemiology

Apropos of the latter, it was first described in Turkey and Greece, then a cluster of cases was found in Japan, and further studies found an increased concentration along the traditional Silk Road.

Diagnosis

Treatment

Topical steroids or sucralfate can help oral or skin lesions.[6] Colchicine has also been used to prevent mucocutaneous relapse,[7] and also for joint disease.

Inhibitors of tumor necrosis factor-alpha have been of benefit, including etanercept, infliximab, and, with due regard for risk, thalidomide for severe lesions. [8]

Notable cases

Hughes-Stovin Syndrome was diagnosed in the "The Down Low" in season 6, episode 10 of the television series House.

References

  1. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. May 5 1990;335(8697):1078-80.
  2. http://www.nlm.nih.gov/medlineplus/behcetssyndrome.html
  3. Anonymous (2015), Behcet's syndrome (English). Medical Subject Headings. U.S. National Library of Medicine.
  4. Emad Y, Ragab Y, Shawki Ael-H, Gheita T, El-Marakbi A, Salama MH (2007). "Hughes-Stovin syndrome: is it incomplete Behçet's? Report of two cases and review of the literature.". Clin Rheumatol 26 (11): 1993-6. DOI:10.1007/s10067-007-0609-y. PMID 17457658. Research Blogging.
  5. Erkan D, Yazici Y, Sanders A, Trost D, Yazici H (2004 Jul-Aug). "Is Hughes-Stovin syndrome Behçet's disease?". Clin Exp Rheumatol 22 (4 Suppl 34): S64-8. PMID 15515789.
  6. Augusto C Posadas & Jeffrey R Lisse (22 June 2009), "Behcet Disease: Treatment & Medication", eMedicine
  7. Aktulga E, Altac M, Muftüoglu A, et al. A double blind study of colchicine in Behçet's disease. Haematologica. Jun 1980;65(3):399-402
  8. Vedat Hamuryudan, et al. (15 March 1998), "[http://www.annals.org/content/128/6/443.full Thalidomide in the Treatment of the Mucocutaneous Lesions of the Behcet Syndrome A Randomized, Double-Blind, Placebo-Controlled Trial]", Ann Intern Med 128 (6): 443-450
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