(compiled by Gwilym ab Ioan a long term Behçet's
is a multisystem inflammatory disorder characterised by recurrent
oral ulcers, genital ulcers and ocular inflammation, and which
frequently involves the joints, skin, central nervous system (CNS)
and gastrointestinal tract. Classified as a systemic vasculitis, it
can involve both the arteries and veins of almost any organ. The
aetiology of Behçets disease remains unknown, but the most widely
held hypothesis of disease pathogenesis is that a profound
inflammatory response is triggered by an infectious agent in a
genetically susceptible host".
(Sara E. Marshall MB BCh, MRcp
(Ire), MRcPath (UK), PhD Senior Lecturer in Immunology Wright
Fleming Institute, Imperial College School of Medicine, Norfolk
Place, London W2 1PG, UK)
Please click on the "PodCast"
icon on the right to hear a joint talk in the form of a
question and answer session given by Drs. Yusuf Yazici,
MD (hosting) and Hasan Yazici, MD. This talk was given
at the Behcet's Syndrome Centre in New York City - in
November, 2006. You may download & save the file which
is in MP3 format. It can also be played directly on your
PC using any media player software. Playing time is
this video that explains the symptoms, diagnosis & care
required for Behçet's sufferers.
It is based on
real life accounts from people who suffer with Behçet's
Disease. Dr. Yusuf Yazici (one of the leading doctors
involved in the field of Behçet's & who is also featured
in the pod cast above) gives a commentary in this video.
have chronic symptoms others have milder initial
symptoms (like Sanya Richards). In all cases Behçet's
can be a truly serious, painful and dangerous condition
as by it's very nature it can attack virtually any organ
in the body. Whilst fatalities due to the symptoms are
relatively rare, this disease can severely handicap
people who suffer with it.
The disease referred to as Behçets is rare in the UK.
It is sometimes referred to as the "Silk Route" or "Silk Road Disease" (from the Mediterranean basin to Japan),suggesting perhaps that an as-yet-unknown genetically
determinedfactor was spread via the migration of
old nomadic tribes.
(through absolutely no
fault of their own), many in the medical profession in this country are not familiar with it and
consequently have a very basic knowledge of it's manifestations, symptoms and treatment,
due mainly to it's rare occurrence in the UK.
Prevalence is 0.3-6.6 cases per 100,000
population. The prevalence is highest in the Middle East, China and
Most common among patients from
their third decade on. An
age of onset younger than 25 years is associated with a higher
prevalence of eye disease and active clinical disease.
Males are affected more commonly than females.
Behçet's disease is associated with HLA-B51.
Familial cases have been reported, but inheritance is probably not
(Online Mendelian Inheritance in Man) Johns Hopkins University
An Introduction to this Web-page
I have suffered from Behçet's Disease since
circa 1982 - in those days it was generally referred to as Behçet's Syndrome (correctly pronouncedBeh-chets[ info. ]. The incorrect
pronunciation seems to be widespread amongst the medical fraternity
in the UK & other English speaking parts of the World - perhaps being a primary indicator of most
Doctor's general depth of knowledge of this rare & debilitating
The purpose of this web page is
not to highlight general medical ignorance about the condition, or to point to
inadequacies, to the contrary, it is to provide a
good basic knowledge to the reader (both medical professionals and sufferers
alike) about Behçets Disease in the hope that it will aid the professional
in providing quicker diagnosis and treatment with corresponding faster and more efficient
relief from the symptoms for any sufferer.
On numerous occasions
during my medical treatments (for various ailments over the years but mostly
associated with Behçets) I have been habitually confronted with Doctors & other
medical staff who are hampered by their inadequate basic knowledge of this
disease. Consequently a lot of their time, my time and the important time needed
for quick diagnosis and correct treatment (because the vascular involvement of BD can be significant and
life-threatening, diagnosing and treating vascular involvement early is
vital) .has been inefficiently used because -
quite rightly - Doctors cannot be solely guided by the information
given to them verbally by a patient who is a medical lay-person . Due to this, tests are
often carried out unnecessarily and often periodically repeated by others, because Doctors do not
have the confidence to eliminate certain likelihoods from their diagnosis
because of a degree of ignorance about the manifestations of this disease. Armed
with a little more detailed knowledge, many of the processes of elimination in
the diagnostic procedure stages could be done away with. It is my belief that many
other sufferers of this disease also experience the same problem. I have a
perennial debate with doctors that often insist that what they are witnessing is
an infection rather than inflammation! Invariably treatment starts with
antibiotics. When a course of antibiotics fails to resolve the problem (often
with the reverse effect of aggravating the inflammation) the patient is finally
administered an anti-inflammatory preparation! Of course not all the problems
that I, or other sufferers have, originate with Behçet's Disease - I have my fair
need of antibiotics like all others seem to have in the western hemisphere, however it
is important for the medical practitioner who treats me (or others like me) to
understand the mechanisms of this disease, and be able to attribute certain
symptoms to it when treating a patient who has the disease.
I have decided to compile this
web-site and publish
it on the Internet in order for any medical people who want more detailed in
information to be able to access it. Following a constructive discussion with my
GP I have agreed to the suggestion that this web site
URL address be included in my medical file so that it is easily accessible to
anyone who may treat me in the future. This page may also be accessed by fellow
sufferers, who may find it helpful to pass the URL on to their own GPs, hospital
medical staff or any other medical practitioner who may benefit from the
contents. Please feel free to use the information at will and to circulate
references to it as you see fit.
The remainder of this web-site is made up of
information that I have researched over the years. Where appropriate, references
have been made to source material and there is a table of useful links to other
websites that contain similar information that you may wish to research.
browsing the remainder of this web-page it is recommended that the reader
downloads and views a PDF document containing an excellent paper authored by Sara E. Marshall MB BCh, MRcp (Ire),
MRcPath (UK), PhD Senior Lecturer in Immunology, Wright Fleming Institute,
Imperial College School of Medicine, Norfolk
Place, London W2 1PG, UK - a small excerpt from that document has been reproduced
as an introduction at the
top of this page.
The download document
requires your computer to have a PDF Reader programme installed on
it. If you have this software the
document will open automatically.
If you do not currently have this type of software installed you can
download & install it now for free by clicking on the icon
For the remainder of
this page I have reproduced an article by
Marjan Yousefi, MD,
(Department of Dermatology, Geisinger Medical Centre)
This article has been chosen as it
contains quite an extensive study of the disease with detailed
information that covers the disease in some depth. Slight
adaptations have been made to reflect figures that correspond to UK
statistics for the disease as opposed to the American statistics
used by Marjan Yousefi.
the drug category tables have been removed and included in a separate page dedicated solely to that
subject. A link to that page can be found at the top of this page
under the tab "drugs".
Bookmark navigation and an altered document format has been
introduced to aid the ease of reading and to help in locating
specific headings & sub headings. The content material has not been
changed or altered in any way from the author's original input.
disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet,
who first described the triple-symptom complex of recurrent oral aphthous
ulcers, genital ulcers, and uveitis.
complex, multisystemic disease includes involvement of the mucocutaneous,
ocular, cardiovascular, renal, gastrointestinal, pulmonary, urologic, and
central nervous systems and the joints, blood vessels, and lungs.
characterized by oral aphthae and by at least 2 of the following: (1) genital
aphthae, (2) synovitis, (3) posterior uveitis, (4) cutaneous pustular vasculitis,
(5) meningoencephalitis, (6) recurrent genital ulcers, and (7) uveitis in the
absence of inflammatory bowel disease or collagen vascular disease.
morbidity is typical; the leading cause is ophthalmic involvement, which can
result in blindness. The effects of the disease may be cumulative,
especially with neurologic, vascular, and ocular involvement.
mortality rate is low, but death can occur from neurologic involvement,
vascular disease, bowel perforation, cardiopulmonary disease, or as a
complication of immunosuppressive therapy.
affected more often, and with more severe disease, than women in some
Mediterranean areas. In Iran, for example, the male-to-female ratio was 24:1
among 1712 patients. In Turkey, the ratio was 16:1 among 427 patients.
symptoms, which may be recurrent, may precede the onset of the mucosal membrane
ulcerations by 6 months to 5 years.
the onset of BD, patients may experience a variety of symptoms.
Decreased or elevated temperature
of the substernal and temporal regions
history of repeated sore throats, tonsillitis, myalgias, and migratory
erythralgias without overt arthritis is common.
diagnosis of BD is based on clinical criteria because of the absence of a
pathognomonic laboratory test. The period between the appearance of an
initial symptom and a major or minor secondary manifestation can be up to a
decade in many cases.
number of different criteria or classification systems that have been
introduced over the past 25 years reflects the failure of any single one to
meet clinical demands. The revised 1987 criteria of the Japanese group (Mizushima)
have been widely applied.
recently, the diagnostic criteria of the International Study Group for
Behçet Disease have been applied to establish a firmer diagnosis.
major limitation of these criteria is the fact that recurrent oral
ulceration is the characteristic symptom for the diagnosis of BD. For
example, patients with uveitis and genital ulcers, without oral aphthosis,
would not be considered to have BD, although this is, in fact, a
far-advanced form of the disease.
Therefore, the authors recommend that both sets of criteria be applied
concurrently until a more exact system is devised.
Diagnostic criteria from the Behçet syndrome research committee of Japan
(1987 revision) are as follows:
Recurrent aphthous ulceration of the oral mucous membrane
Eye lesions - Iridocyclitis, chorioretinitis, retinouveitis,
definite history of chorioretinitis or retinouveitis
Arthritis without deformity and ankylosis
Gastrointestinal lesions characterized by ileocecal ulcers
Central nervous system symptoms
Complete - Four major features
Incomplete - (1) 3 major features, (2) 2 major and 2 minor features,
or (3) typical ocular symptom and 1 major or 2 minor features
Possible - (1) 2 major features or (2) 1 major and 2 minor features
International criteria for the classification of BD (1990) are as follows:
Recurrent oral ulceration - Minor aphthous or major aphthous or
herpetiform ulceration observed by a physician or reported reliably by a
patient that recurs at least 3 times in one 12-month period plus 2 of
Recurrent genital ulceration - Recurrent genital aphthous ulceration
or scarring, especially males, observed by a physician or reliably
reported by a patient
Eye lesions - (1) Anterior uveitis, posterior uveitis, and cells in
vitreous upon slit-lamp examination or (2) retinal vasculitis
observed by physician (ophthalmologist)
Skin lesions - (1) Erythema nodosumlike lesions observed by
physician or reliably reported by a patient, pseudofolliculitis, and
papulopustular lesions or (2) acneiform nodules consistent with BD,
observed by a physician, and in postadolescent patients not
Positive pathergy test - An erythematous papule larger than 2 mm at
the prick site 48 hours after the application of a 20- to 22-gauge
sterile needle, which obliquely penetrated avascular skin to a depth
of 5 mm as read by a physician at 48 hours
are applicable if no other clinical explanation is present.
Genital ulcers resemble their oral counterparts but may cause greater
scarring. They have been found in 56.7-97% of cases, but their
appearance is mostly a secondary symptom that accompanies oral ulcers.
males, the ulcers usually occur on the scrotum, penis, and groin.
females, they occur on the vulva, vagina, groin, and cervix.
Ulcers have also been found in the urethral orifice and perianal area.
Epididymitis may arise and is a minor diagnostic criterion for the
disease according to the Behçet Disease Research Committee of Japan.
additional genital symptom is orchiepididymitis, observed in 10.8% of
Ocular involvement is the major cause of morbidity and the most dreaded
complication because it occasionally progresses rapidly to blindness. It
is reported in 47-65% of patients with BD. Childhood-onset Behçet
uveitis is more common in males (Tugal, 2003).
most diagnostically relevant lesion is posterior uveitis, ie, retinal
vasculitis. Other lesions include anterior uveitis, iridocyclitis,
chorioretinitis, scleritis, keratitis, vitreous hemorrhage, optic
neuritis, conjunctivitis, retinal vein occlusion, and retinal
neovascularization. Hypopyon, which was considered the hallmark of BD,
is now uncommon.
disease is usually present from the outset but also may develop within
the first few years. Decreased visual acuity is a result of secondary
glaucoma, cataracts, or vitreous hemorrhage. Blindness has been reported
to occur within 4-5 years from the onset of ocular symptoms. Retinal
vein thrombosis leading to sudden blindness is not rare.
Histologic findings include media thickening, elastic fiber splitting,
and perivascular round cell infiltration.
4 types of vascular lesions recognized in persons with BD are arterial
occlusions, venous occlusions, aneurysms, and varices.
Venous involvement is usually limited to occlusion, with the varices
Affected sites of the venous system are the superior vena cava, inferior
vena cava, deep femoral vein, and subclavian vein.
Arterial complications account for 7% of cases. Aneurysm and occlusion
are most common.
subclavian artery and pulmonary artery are the most common arteries
occluded. Depending on the site, arterial occlusions can have different
clinical presentations. Pulseless disease is due to subclavian artery
Hypertension can originate from renal artery stenosis.
Femoral artery stenosis and intermittent claudication cause avascular
necrosis of the femoral head.
Pulmonary vasculitis can produce dyspnea, chest pain, cough, or
Aneurysm formation accounts for most vascular deaths. Common sites of
aneurysms are the abdominal aorta, femoral artery, and thoracic artery.
Because the vascular involvement of BD can be significant and
life-threatening, diagnosing and treating vascular involvement early is
Pregnant women with BD may experience more severe symptoms during the
course of the pregnancy, especially in the first trimester. Overall,
pregnancy does not seem to markedly affect the course of BD (Uzun,
Close follow-up is necessary to monitor the health of the mother and
presence of pathergy strongly suggests the diagnosis of BD.
Following a needle prick or intradermal injection with saline or dilute
histamine, the puncture site becomes inflamed and develops a small
sterile pustule from hyperactivity of the skin to any intracutaneous
pustular reaction of the skin is thought to denote increased neutrophil
Higher positivity (84-98%) is found in Mediterranean areas and the
Middle East than in the Far East (40-70%), with Western countries having
significantly lower positivity than the other regions.
HLA-B51 or its B101 allele is significantly associated with BD
in Japan, Korea, Turkey, and France and with ocular manifestations in
Britain. Although HLA-B51 transgenic mice do not develop any
manifestations of BD, their neutrophils show excessive function.
MICA allele is a polymorphic MHC class Irelated A gene (MICA)
family. The MICA6 allele has recently been shown to be
significantly associated with BD (74%), compared with controls (45.6%)
in Japan. The relationship between MICA6 and BD was confirmed
in France. The MICA6 allele is thought to be in linkage
disequilibrium with HLA-B51; consequently, the search for genes related
to BD continues. A recent study of 23 Japanese patients showed that the
MICA6 allele had no significant association with BD, but it
showed a strong association with HLA-B51; therefore, the association
between MICA6and BD may be a secondary phenomenon related to
HLA-B51 (Nishiyama, 2006).
MEFV gene mutations, seen in persons with Mediterranean fever, are
increased in persons with BD. This mutation has been associated with
vascular BD (Atagunduz, 2003).
Levels of tumor necrosis factor-alpha (TNF-alpha) have been reported to
be significantly elevated in BD patients; thus, reports of TNF-alpha
blockers having therapeutic benefits have been reported. Park et al
analyzed TNF-alpha haplotypes in the promoter response element that
affect the binding affinity of certain transcription factors. Their
study showed that TNF-alpha -1031*C, -863*A, -857*C, and -308*G alleles
were significantly associated with BD. TNF-alpha haplotypes in the
promoter response elements may be useful in identifying those more
susceptible to BD (Park, 2006).
Investigations of the etiology of BD have focused predominantly on
herpes simplex virus infection, streptococcal infection, and
autoimmunity or cross-reactivity between microbial and oral mucosal
Behçet suggested the herpes simplex virus as a causative agent in his
first report. Polymerase chain reaction studies have remarkably improved
the diagnostic significance of viral infections, especially herpes
simplex virus. Herpes simplex virus DNA has been detected in saliva,
genital ulcers, and intestinal ulcers of patients with BD. BD-like
symptoms have been induced in an Institute for Cancer Research mouse
after inoculation of herpes simplex virus into its earlobe.
Acquired hypersensitivity to streptococcal antigens plays an important
role in the etiopathology of BD.
multiplicity of etiologic factors may have a common denominator in the
65-kd microbial heat shock protein (HSP), which shows significant
homology with the human 60-kd mitochondrial HSP. Indeed, the uncommon
serotypes of Streptococcus sanguis found in BD cross-react with
the 65-kd HSP, which also shares antigenicity with an oral mucosal
T-cell epitope mapping has identified 4 peptides derived from the
sequence of the 65-kd HSP that specifically stimulates T-cell receptor (TCR+)
lymphocytes from patients with BD.
These peptides (111-125, 154-172, 219-233, and 311-325) show
significant homology with the corresponding peptides (136-150,
179-197, 244-258, 336-351) derived from the human 60-kd HSP.
B-cell epitopes within mycobacterial HSP65 or human HSP60 overlap
with the T-cell epitopes, and both immunoglobulin G and
immunoglobulin A antibodies have been identified.
Among the 4 T- and B-cell epitopes, peptide 336-351 of the 60-kd HSP
is significantly associated with BD in Britain, Japan, and Turkey.
HSP60/65 was also found to be significantly increased in the
epidermal cells of BD skin lesions, and antibody levels to HSP65
were significantly elevated in the cerebrospinal fluid from patients
with neurological manifestations of BD.
An experimental model of BD uveitis was established in rats, in
which subcutaneous immunization with peptide 336-351 and adjuvants
elicited uveitis in approximately 80% of Lewis rats. Furthermore, a
mucosal model of induction of uveitis was developed in rats by oral
or nasal administration of peptide 336-351 without an adjuvant, and
this is consistent with the oral onset of ulceration in more than
90% of patients with BD.
persons with BD, the Th1 cytokine interferon (IFN) level is elevated in
serum, in skin T cells, and cerebrospinal fluid, and interleukin (IL)12
is generated by the stimulation of CD4+ T cells with the HSP
peptide 336-351, although IL-12 can also be secreted by neutrophils in
persons with BD. However, the concentration of the Th2 cytokine IL-6 is
also increased in the serum of patients with BD, especially in the
active stage, as was also found with IL-10 upon stimulation of the
peripheral blood mononuclear cell.
Stimulation with S sanguis (KTH-1) of T-cell lines
generated from patients with BD suggests that Th1-type mRNA is induced
(IL-2 and IFN).
Investigations of intracellular IFN and IL-4 suggest that polarization
toward the Th1 type of cells occurs in patients with active BD because
of a significant increase in the intracellular IFN that was not observed
with IL-4. However, the converse was found in another investigation by
stimulating peripheral blood mononuclear cells, with increased Th2
cytokines (IL-10 and decreased IL-2 or IFN) in active BD.
intracellular adhesion molecule 1 was enhanced in human dermal
microvascular endothelial cells after treatment with serum from patients
with BD, and this may have induced increased adhesion of T cells to the
Levels of the proinflammatory cytokines tumor necrosis factor (TNF)alpha,
TNF receptor 1, IL-1, and IL-8 are elevated in the serum of persons with
BD and remain unregulated in peripheral blood mononuclear cells and
neutrophils of patients with BD (Duzgun, 2005). Levels of IL-10 and
IL-13 may also be elevated (Aridogan, 2003).
Plasma levels of vascular endothelial growth factor, a proinflammatory
cytokine, is significantly higher in persons with active BD (Cekmen,
Neopterin is produced by human monocytes and macrophages in response to
interferon-gamma (IFN-gamma) released from activated T cells; thus, it
serves as a marker for cellular immune activation. Kose et al showed
that serum levels of neopterin were significantly higher in active and
inactive BD patients than in controls. Those with active disease had
higher levels than those with inactive disease (Kose, 2006).
Vascular changes leading to vasculitis and thrombosis are important
pathological features of BD.
recent study proposes that immunoglobulin to carboxy-terminal subunit of
Sip1 (Sip1 C-ter) may be a useful novel autoantigen in BD.
Interestingly, this autoantigen level was not only elevated in 41% of BD
patients, but also in 45% of those patients with primary vasculitis,
thus this marker may also point at endothelial dysfunction in vasculitis
Antiendothelial cell antibodies are detected in diseases with
immune-mediated vascular damage and show significantly increased
prevalence in BD.
cells (mostly CD4 cells), B cells, and neutrophils are infiltrated
Esmat et al showed much higher serum lipoprotein(a) levels in patients
with vascular complications and lower levels of serum nitrites during
disease activity (Esmat, 2006).
Another study demonstrated an association of factor V Leiden and G20210A
prothrombin mutation with thrombosis in BD patients (Ricart, 2006).
Prostanoid synthesis in endothelial cells or vessel walls is impaired,
whereas von Willebrand factor, thromboxane, and thrombomodulin are
level of endothelin 1 and 2 is increased in patients with BD vascular
Endothelial celldependent vasodilator function is significantly
impaired in patients with BD and is demonstrated by high-resolution
and pathogenesis of BD remain obscure, although many reviews describe a
is thought to affect vessels of all sizes; the various skin lesions are thought
to be secondary to small vessel vasculitis.
histopathology is variable, dependent upon the type of lesion. Pathergic lesions
are characterized by a heavy neutrophilic infiltrate without fibrin within the
vessel walls. Folliculitis, acneiform lesions, and dermal abscesses have been
described in BD.
nodosumlike lesions show a perivascular lymphocytic infiltrate of lymphocytes
in the deep dermis and septa with a lymphocytic vasculitis but lack the
histiocytic granulomas of typical erythema nodosum.
ulcers have a nonspecific pathology with a variable infiltrate of lymphocytes,
macrophages, and neutrophils at the base of the ulcer.
subsets with a preponderance of helper-inducer cells over T suppressor-cytotoxic
cells have been observed in lesions.
of erythema nodosumlike lesions shows microvascular changes and
lymphocyte-mediated fat cell lysis. Additionally, small dermal blood vessels
embolized by thrombi are observed at the sites of the needle prick reaction (pathergy)
and at the erythema nodosumlike lesions.
changes in fat cells may be caused by vascular changes brought about by the
specific degeneration of endothelial cells and vascular stenosis associated with
the delayed-type hypersensitivity reaction.
Although multiple therapeutic modalities have been
used, treatment is far from satisfactory. Treatment of BD seems to be
symptomatic and empiric. Therapeutic efficacy has been difficult to evaluate
because of the variable course of the disease and the limited number of cases
for clinical investigation.
Tetracycline remains the drug of choice for aphthous stomatitis and oral
ulcers of BD.
patient dissolves the contents of a 250-mg tetracycline capsule in 5 mL
of water or flavored syrup and holds the solution in the mouth for
approximately 2 minutes before swallowing. This is repeated 4 times
Topical corticosteroids are effective for oral or genital ulcerations if
they are applied during the prodromal stage of ulceration.
Other useful drugs include lidocaine gel (2%), sucralfate suspension,
and 5% amlexanox.
Corticosteroids are the mainstay of treatment for all the various
clinical manifestations. Although they have a beneficial effect on acute
manifestations, no definite evidence indicates they are effective for
adverse effects of long-term steroid therapy must be considered.
Mucocutaneous lesions and arthritis have been treated with nonsteroidal
anti-inflammatory drugs, zinc sulfate, levamisole, colchicine, dapsone,
or sulfapyridine and thalidomide (use is strictly limited because of its
teratogenicity). Immunosuppressive therapy with azathioprine,
chlorambucil, or cyclophosphamide has been used.
Uveitis and central nervous system involvement is treated with systemic
corticosteroids, azathioprine, or cyclosporine.
Anticoagulants are given to patients with thromboses.
Recent therapeutic approaches have included cyclosporine, IFN alfa, IFN
gamma, acyclovir, high-dose corticosteroids or cyclophosphamide pulse
therapy, and FK506 (tacrolimus, an immunosuppressive agent similar to
FK506 (tacrolimus) has been particularly noteworthy. The Japanese FK506
study group reported that FK506 was effective in treating refractory
uveitis in a dosage-dependent manner. Adverse effects were renal
impairment (28.3%), neurologic symptoms (20.8%), gastrointestinal
symptoms (18.9%), and hyperglycemia (13.2%). The study group also noted
the need for further clinical investigations on FK506 before more
Subcutaneous IFN alfa-2a therapy has resulted in reduced ulcers and eye
disease. Flulike symptoms were the most common adverse effect.
Leukopenia, hair loss, and development of antinuclear and antithyroid
antibodies were reported less commonly (Alpsoy, 2003; Kotter, 2003).
the possible role of TNF-alpha in the pathogenesis of BD, infliximab, a
chimeric monoclonal immunoglobulin G antibody that inhibits TNF-alpha,
and etanercept, a TNF receptor blocker, have steroid-sparing effects and
have decreased the frequency of attacks in patients with BD (Sfikakis,
2002). Case reports describe treatment of patients with recalcitrant
disease or those in whom conventional immunosuppressive agents have
failed (Katsiari, 2003; Ohno, 2004; Tugal-Tutkun, 2005. Etanercept has
been used at 25 mg twice a week (Melikoglu, 2005). Infliximab has
resulted in responses after etanercept failed (Estrach, 2002).
Infliximab infusions of 5-10 mg/kg have been used with variable dosing
schedules. Tuberculosis was a reported adverse effect of infliximab
infusion in one BD patient (Ohno, 2004).
Dermatologist - For evaluation of mucocutaneous lesions (ie, oral ulcer,
genital ulcer, skin lesions)
Ophthalmologist - For evaluation of eye involvement
Rheumatologist or orthopedic surgeon - For evaluation of joint involvement
Neurologist or psychiatrist - For evaluation of CNS involvement
medicine specialist - For evaluation of gastrointestinal, pulmonary, renal,
or endocrine involvement
surgeon - For evaluation of gastrointestinal involvement
surgeon or cardiologist - For evaluation of cardiovascular involvement
nose, and throat specialist or dentist - For evaluation of oral cavity
The goals of
pharmacotherapy are to reduce morbidity and to prevent complications.
Corticosteroids have been used for all of the various clinical manifestations of
BD. Anti-inflammatory and immunosuppressive agents are used to treat
mucocutaneous lesions and arthritis associated with this disease. Anticoagulants
are administered to patients with thromboses.
clinical course of BD is variable, even in the early stages, making
determinations of the long-term prognosis difficult.
appear to have a poorer prognosis.
disease usually runs a protracted course, with attacks generally lasting for
several weeks and recurring more frequently early in the disease.
Mucocutaneous and arthritic involvement usually occur early.
morbidity is usual; the leading cause is ophthalmic involvement, which can
result in blindness. The effects of the disease may be cumulative,
especially for neurologic, vascular, and ocular involvement.
Mortality is low, but patients may die from neurologic involvement, vascular
disease, bowel perforation, cardiopulmonary disease, or as a complication of
9: Typical positive pathergy reaction
at injection site.
10: Ocular involvement showing
Additional Photos & Note by web-page author:
Below is this web-site author's previous leg inflammation.
Taken 16th June 2008 at Bronglais General Hospital, Aberystwyth, Wales.
The condition was treated as Cellulitis - due to similarities in the
outward appearance of the condition, (which was in fact a Erythema
nodosum-like flare up of BD). The treatment insisted on was an IV
infusion of antibiotics despite the author's suggestion to the doctors
that it was not an infection but inflammation due to BD! Hence one of
the reasons for this web-page.