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A peculiar rash and red eye

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Q1: What is the most likely diagnosis?

Behçet’s disease. Behçet’s disease is a vascultic disorder characterised by recurrent aphthous mouth ulcers, genital ulceration, ocular lesions, and skin lesions.

Q2: What is the rash around the intravenous cannula site and what is the pathophysiology for this phenomenon?

This peculiar rash is called pathergy. This phenomenon of pathergy is a hallmark of this disorder. A positive pathergy is said to be present if the patient develops 2–4 mm of erythema 24–48 hours after #25 gauge needle prick to a depth of 5 mm.

The possible explanation for this phenomenon is increased superoxide production by neutrophils, increased chemotaxis, and excessive production of lysozomal enzymes.

Discussion

Behçet’s disease was first described by a Turkish dermatologist, Hulusi Behçet (1889–1948), in 1937. The disorder is characterised by a triad of aphthous stomatitis, genital ulcers, and uveitis. The disease characteristically takes a chronic course of remissions and relapses with a male preponderance. Behçet’s disease is associated with HLA-B51 and is mainly observed in countries around the Mediterranean and in the Far East.

Two sets of criteria are used for the diagnosis of Behçet’s disease: international criteria, derived in 1990, and the O’Duffy criteria.

The international criteria include recurrent oral ulceration, plus two of the following:

  • Recurrent genital ulcerations.

  • Eye lesions (anterior uveitis or posterior uveitis).

  • Cells in the vitreous.

  • Retinal vasculitis.

  • Skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, acneiform nodules in a post-adolescent patient not taking corticosteroids).

  • Positive pathergy test.

The O’Duffy criteria require that, in addition to recurrent aphthous ulceration, any two of the following must be present:

  • Genital ulcers.

  • Uveitis.

  • Cutaneous pustular vasculitis.

  • Synovitis.

  • Meningoencephalitis.

  • Exclusion of inflammatory bowel disease, systemic lupus erythematosus, Reiter’s syndrome, and herpetic infections.

Box 1: Differential diagnosis of oral aphthous ulcers

  • Idiopathic.

  • B12/folate/iron deficiency.

  • Gluten sensitive enteropathy.

  • Inflammatory bowel disease.

  • Behçet’s disease.

  • Stevens-Johnson syndrome.

  • Menstrually related.

Treatment

Studies for the treatment of Behçet’s disease are small and only a few randomised controlled trials have been performed. Assessment of the efficacy of the treatment is limited by the natural waxing and waning of the disease, the need to tailor therapy to the clinical manifestations that are present, and variable response rates to the treatment used.

Non-steroidal anti-inflammatory drugs are most commonly given for the treatment of arthritis but are not always useful. Steroids, sulphasalazine, methotrexate, cyclosporin, and other immunosuppressive agents may be used for more severe arthritis, although there are no large trials available with these agents.

Mild oral and genital ulcers can be treated with local corticosteroids. Thalidomide has also been used successfully in treating mucocutaneous lesions. Colchicine, which has been traditionally used for every aspect of Behçet’s syndrome, seems to be effective mainly for the treatment of the mucocutaneous lesions in female patients.

Significant ocular, neurological, gastrointestinal, vascular, or other serious end organ manifestations require treatment with steroids and other immunosuppressive agents.

Interferon-alfa has been beneficial in ocular, neurological, mucocutaneous, and articular manifestations in a number of case reports and case series.4 Anecdotal evidence of the beneficial effects of antitumour necrosis factor therapy has been reported.5

Final diagnosis

Behçet’s disease.

Box 2: Conditions to be considered with acute iritis

  • Sarcoidosis.

  • Ankylosing spondylitis.

  • Juvenile rheumatoid arthritis.

  • Inflammatory bowel disease.

  • Psoriasis.

  • Reiter’s syndrome

  • Behçet’s disease.

  • Herpes infections.

  • Lyme disease.

  • Tuberculosis, leprosy, onchocerciasis.

Table 1

 Clinical manifestations

References

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