When it comes to condition similar to Behcet disease, you cannot expect a better move making you closer toward the right diagnosis than taking an efficient medic
al history. Despite the fact that technology is playing an important role in empowerment of health practitioners, the medical history is more like the mainstay of
the diagnosis. The effect of behavioral, hereditary, environmental and social
factors on patient well-being and illness must be reflected in the patient’s
history. The best statement describing the importance of the medical history is
“A complete and accurate
history is the foundation for all future patient care”.
Building a clinical
diagnosis of an autoimmune disease require a full case history and clinical
exam (general exam and special exam), A thorough past (medical) history allows
the clinician to define the patient’s problem and, along with the result of
physical examination, assists in formulating an etiologic and/or pathologic
diagnosis in most cases.
Background and history
Mr. S is a 25-year old male. Saudi
and originally from Najran. He
grew up in Al Khobar and presently living
there. Single. Newly graduated from the United States in the specialty of
chemical engineering. Unemployed (resigned his last job as an engineer due to
the deterioration of his health condition). His family consists of 6 members,
parents, sister and two brothers, all are alive and in overall good health.
History and Condition
When Mr. S was
18 years old, one month after graduating from high school he was diagnosed with
Behcet’s syndrome. The initial symptoms that started with Mr. S was successive weakness
in vision (to the point of not being able to see), both
of his eyes are involved, accompanied by pan uveitis along with multiple
follicular lesion in the skin. Back then, based on history
and clinical exam the attending doctor referred the eye problems he had to
Retinal Vasculitis as a conclusive indicator of behcet disease together with
manifestation of the other symptoms mentioned previously. Mr. S was under take
of immunosuppressive course and later on he was prescribed Humira “adalimumab”.
X presented to King Fahad Hospital of the
University (KFHU) for a follow up, shortly after obtaining consent, and
while interviewing him I was going through the medical history, as an answer of
my question about the past history, he points out and noted that since he was
treated with Humira for the last seven years, he was in good compliance until 6
months when he finished the last dose of Humira and was not able to renew, so
he stayed five to six months without treatment. Groin area started to manifest ulcers
progressively, it started around 6 months ago (before stopping Humira) but
increased when stopped, followed by progressive dysarthria, dysphagia and
slurred speech. Also, he developed painful genital ulcers and skin lesions in
the area of the armpit.
back to the clinic after few days in order to meet Mr. S and start to gain the
medication history from him. However, when I arrived at the clinic, the physician
in charge of the case recalled and stated that the patient’s condition has deteriorated
and he have been hospitalized in the Medical ward and start a course of IV steroids.
I have met Mr. S, he said that he was unsteady (lack of balance) in which he
cannot perform the normal daily activities by himself. His condition developed,
there was an absence and he was admitted as case of Neuro behcet syndrome.
on, Mr. S manifest remarkable progress in his condition.
Description, Predisposing Factors and Clinical Presentation
Behçet’s syndrome (BS) is a systemic
vasculitis (inflammation of blood vessels), it can cause skin and mucosal
lesions, oral and genital ulcers, inflammation of the uvea (uveitis) leading to
blurring of vision and visual impairment. Also, it could cause major arterial
and venous vessel disease, GI ulceration and neurological manifestations. These
can present in various combinations and sequences in patients as time passes. (bestpractice.bmj.com, 2016).
Nervous system involvement, known as Neuro-behcet syndrome (NBS).
And is one of the rarest and difficult complications of BS but is still of
paramount importance due to its mortality and morbidity rate. NBS is seen in
about 5-10% of all cases. Besides the mentioned symptoms of BS, NBS is
characterized with an acute or subacute brainstem syndrome or hemiparesis, as
well as with other various neurological manifestations. There are two types of
NBS, acute type and chronic progressive type. In attacks of acute NB, administration
of corticosteroids is usually necessary and effective, although they are
sometimes self-limiting. In addition to that, interferon alpha and infliximab might
also be effective during the attacks in acute NB. On the other hand, one should
keep in mind that corticosteroids, cyclophosphamide, and azathioprine are not
effective at all, as to chronic progressive NB. ??????
The etiology and the pathogenesis of
Behçet disease are not clear but are presumed to be multifactorial, involving
genetic, infectious, and immunological factors. Increasing evidence suggests
that antigens derived from infectious agents (eg, Streptococcus sanguis,
herpes simplex virus, heat shock proteins) are implicated in the pathogenesis
of the disease, and it has also become increasingly apparent that these events,
once triggered, may be influenced by numerous interdependent and independent
genetic regions. (medscape.com, 2016)
Factors that may increase your risk of
Age. Although children and older
adults can develop the condition. Behcet’s syndrome commonly affects
men and women in their 20s and 30s.
Where you live. People
from countries in the Middle East and Far East, including Japan, Iran,
Korea, China and Turkey, are more likely to develop BS.
Sex. The disease is usually
more severe in men. Even though, Behcet’s disease occurs in both men and
Genes. As outlined before, a
higher risk of developing Behcet’s is associated with having certain genes.
“In regard to clinical presentation, oral ulceration, the
hallmark of this disease, is usually the initial clinical symptom and can
precede other manifestations by years. Ulcers are typically painful, appear in
crops, and are nonscarring. As well as Genital ulcers appear in the vulva and
vagina in females and scrotum and penis in men. Ulcers are painful, recurring,
Other clinical manifestation includes:
Ocular manifestations, it may present with uveitis. Skin
manifestations. In small or large joints, arthralgia and arthritis can occur.