Adamantiades-Behet syndrome

Adamantiades Behcet's syndrome: Description, Causes and Risk FactorsCommonly known as Behcet's disease.Only in Greek this disease is referred as Adamantiades Behçet's or even Adamantiades' disease.Alternative Names: Behcet's Disease; Franceschetti-Valerio syndrome; Halushi-Behcet's Syndrome; Mucocutaneous ocular syndrome; oculobuccogenital syndrome; Touraine's Aphthosis; triple symptom complex; recurrent hypopyon; iridocyclitis septica; cutaneomucouveal syndrome; uveoencephalitic syndrome.Abbreviation: BD.BD is most prevalent in the Mediterranean region, Middle East, and Far East, with an estimated prevalence of 1 case per 10,000 persons. Both genetic and environmental factors contribute to the development of the disease. Adult males are most commonly affected. The mean age at onset ranges from the mid 20s to the 50s. Adamantiades-Behcet's syndrome is relatively rare in children and the elderly.The exact cause of Behcet's is unknown, but it may be an autoimmune disorder, which means the body's immune system mistakenly attacks some of its own healthy cells. Both genetic and environmental factors may be responsible for Behcet's disease. Behcet's disease is not contagious and does not spread from one person to another.Risk Factors Include:HLA-B51 allele.  
  • Herpes simplex virus.
  • Hepatitis C virus.
  • Parvovirus B19.
  • Streptococcus sanguis.
  • The ubiquitous antigens, including heat shock protein of microorganisms.Adamantiades Behcet's syndrome
Symptoms:Common symptoms include:
  • Headache.
  • Fever.
  • Diarrhea.
  • Weakness.
Other symptoms Include:Mouth sores and canker sores.
  • Acne likes sores on the body especially on the lower legs.
  • Sores in the genitals.
  • Uveitis.
  • Vasculitis.
  • Joint swelling and pain.
  • Abdominal pain.
Diagnosis:Differential diagnosis of Adamantiades Behcet's syndrome include chronic oral aphthosis, Herpes simplex virus infection, Sweet's syndrome,HLA-B27-related syndromes such as ankylosing spondylitis, Crohn's disease and ulcerative colitis, ?Multiple sclerosis.Diagnosing Behcet's disease is difficult because no specific test confirms it.There is no definitive test for the disease, and a diagnosis is based on key symptoms of the disease, which can take several months or even years to appear. Symptoms that are key to a diagnosis include:Mouth sores at least three times in 12 months.
  • Any two of the following symptoms: recurring genital sores, eye inflammation with loss of vision, characteristic skin lesions, or positive pathergy test.
Treatment:Although there is no cure for Adamantiades Behcet's syndrome, people can usually control their symptoms with proper medication, rest, and exercise. Treatment goals are to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on the person's symptoms and their severity.The choice of the treatment depends on the patient's clinical manifestations. Significant ocular, neurologic, gastrointestinal, vascular, or other serious end organ manifestations typically require treatment with steroids and other immunosuppressive agents. Ulcers that are refractory to these treatments require systemic steroids, immunosuppressive agents.Other activities include:Rest.
  • Exercise.
  • Healthy lifestyle.
Disclaimer: The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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