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Behçet's disease (Pediatrics - PEDS)

Low urgency
-Moderately severe

Behcet's disease or Behcet's syndrome is a chronic rheumatic disease that causes inflammation of the blood vessels (vasculitis) of unknown cause.

The most frequent symptoms are: aphthous ulcers in the mouth, genital ulcers, skin ulcers, inflammation of some parts of the eye and/or inflammation of the joints. Less common symptoms are: thrombosis, encephalitis, inflammation of the digestive system (colitis, gastritis) and blindness.

Diagnosis is clinical, there is no specific test to diagnose the disease.

Treatment is according to the individual symptoms and the time of onset. Medications are aimed at reducing inflammation or trying to regulate the immune system (corticosteroids, non-steroidal anti-inflammatory drugs, immunosuppressants, etc.).

Bibliographic references
  1. Yazici H, Fresko I, Yurdakul S. Behçet's syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 2007; 3:148.
  2. Calamia KT, Wilson FC, Icen M, et al. Epidemiology and clinical characteristics of Behçet's disease in the US: a population-based study. Arthritis Rheum 2009; 61:600.
  3. Mok CC, Cheung TC, Ho CT, et al. Behçet's disease in southern Chinese patients. J Rheumatol 2002; 29:1689.
  4. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med 1999; 341:1284.
  5. Haralampos M. Moutsopoulos. Síndrome de Behçet. Harrison. Principios de Medicina Interna. Volumen 2. 19ª Edición. 2194.
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Symptoms

    Genital ulcer

    Blisters and/or pustules on the skin

    Mouth ulcer or sore

    Swollen, hot, red joint

    Blurry vision

Symptoms to watch out for

Fever (temperature higher than 100.4 ºF)
Blurry vision
Signs of dehydration: more tiredness than usual, dizziness, dry mouth and tongue.

Self-care

Relative rest, rest until symptoms subside.
Maintain a fluid intake of 6 - 8 cups per day.
Take nonsteroidal anti-inflammatory drugs.
Apply a cream or ointment that contains a corticosteroid.
Consult with your general practitioner for the indication of corticosteroids, immune system inhibitors and/or biological drugs.
Consult with your primary care physician regarding the prescription of glucocorticoid eye drops.
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