behet 's disease ( bd ) is a chronic inflammatory disease that is characterized by recurrent oral and genital ulceration and uveitis1 ) , the other clinical manifestations include skin lesions , arthritis , thrombosis , neurologic symptoms and intestinal ulceration .
polychondritis is known as a rare complication of bd and no case of bd combined with polychondritis had been reported on until firestein et al
. reported on five patients with both bd and relapsing polychondritis ( rp ) in 19852 ) .
we recently experienced a case of a 40-year - old male patient with both bd and polychondritis , and we report herein on this unusual case along with a review of the literature .
a 40-year - old man was referred to our hospital for further evaluation of his fever , oral ulcers and skin rashes that had lasted for 10 days .
he had previously suffered from intermittent oral ulceration and uveitis that had been treated with oral prednisolone and intraocular injections of triamcinolone at another hospital 1 year prior to this admission . on the physical examination ,
his blood pressure was 130/80 mmhg , the pulse rate was 80 per minute and the body temperature was 38.4. he had conjunctival injections in both eyes and several aphthous ulcers on the tongue ( figure 1a ) and buccal mucosa .
a small round ulcer with tenderness was found on the penile root ( figure 1b ) .
several round erythematous skin lesions were present on the chest and thigh ( figure 2a ) .
his left knee joint was swollen ( figure 2b ) and tender , and needle aspiration revealed turbid inflammatory synovial fluid .
the laboratory findings were as follows : hemoglobin 14.8 g / dl , white blood cell count 10,400/l , platelet count 291,000/l , erythrocyte sedimentation rate 104 mm / hr and c - reactive protein 205 mg / l ( normal range , 0~3.4 mg / l ) .
the renal and liver function tests were normal and the tests for rheumatoid factor and antinuclear antibody were negative .
the urinalysis was normal and no microorganisms were grown on both the blood and urine cultures .
his chest radiography showed no abnormal findings and mild splenomegaly was observed on abdominal sonography .
skin biopsy was performed and the histologic examination showed perivascular mononuclear cell infiltrations , extravasation of the red blood cells and fibrinoid necrosis of the blood vessel walls ; all of these findings were compatible with cutaneous vasculitis combined with bd ( figure 3 ) . on the 5th day of admission , both ear auricles became swollen , red and tender ( figure 4 ) .
he remembered having a similar auricular inflammation 2 years ago that had improved after taking non - steroidal anti - inflammatory drugs . after he was administered with colchicine 1.2 mg / day and prednisolone 30 mg / day , his clinical symptoms improved and he was discharged on the tenth day of admission .
polychondritis is an inflammation of the various catilagenous structures such as the auricle , nose and trachea , and it has also been known to involve the eye and the joints .
when polychondritis is relapsing , it is called relapsing polychondritis ( rp)8 ) . in rp ,
various cutaneous manifestations are also seen , including palpable purura , erythema nodosum , panniculitis , livedo reticularis and urticaria .
there have been case reports on polychondritis combined with other rheumatologic diseases such as systemic lupus erythematosus ( sle ) , rheumatoid arthritis ( ra ) and systemic vasculitis9 - 11 ) .
however , there have been no reports of polychondritis combined with bd until firestein et al .
first reported on five patients who had the features characteristic of both bd and rp , and those authors termed this as mouth and genital ulcers with inflamed cartilage ( magic ) syndrome2 ) .
polychondritis is still known as a rare complication of bd and only five more cases of bd combined with polychondritis have been reported on since firestein 's report3 - 7 ) . in our patient ,
bd was diagnosed based upon the recurrent oral and genital ulcers , the skin vasculitis , the arthritis and the past history of uveitis .
the chondritis developed at the bilateral auricles , eye and the joints during the acute exacerbation period of bd , and this resolved with the improvement of the symptoms of bd .
this suggests that the development of polychondritis may have been associated with bd in our case . as for treatment for the combined cases of bd and rp , colchicine , steroid and dapsone
skin involvement in bd includes various lesions such as erythema nodosum , pustules , papules , pseudofolliculitis , acneiform folliculits , sweet 's syndrome , or pyoderma gangrenosum12 ) .
the histopathology of the papulopustular lesions usually shows vasculopathy that is characterized by the perivascular infiltration of mononuclear cells , and this infiltrate is mainly composed of lymphocytes .
the erythema nodosum - like lesion is known to be characterized by extravascular neutrophilic infiltration and panniculitis , and this can be followed by lymphocyte infiltration during the evolution of the skin lesions14 ) . in our case , the main histopathylogic finding of the skin lesions was perivascular mononuclear cell infiltration and fibrinoid necrosis of the vessel walls , which are the characteristic findings of vasculopathy . although cases of bd combined with polychondritis have rarely been reported , bd and polychondritis do not seem to be separate entities .
compared the frequency of the various clinical features in bd and rp ( table 1 ) , and they showed that some of the manifestations that are considered unique for one disease could be found in the other disease2 ) .
these similarities between the clinical manifestations of bd and rp imply that there seems to be something in common for the pathogenesis of bd and polychondritis .
the pathogenesis of both bd and rp are associated with autoimmunity . in patients with rp ,
the antibodies to collagen type ii , ix and xi are elevated in the sera15 , 16 ) and the frequency of human leukocyte antigen ( hla)-dr4 is increased17 ) .
additionally , rp has been associated with other autoimmune diseases such as ra and sle9 , 10 ) . in patients with bd ,
autoantibodies such as antiendothelial cell antibody and antibody to -tropomyosin have been found18 - 20 ) and it has been postulated that cross - reactivity and molecular mimicry between the peptides from the streptococcal proteins or the viral heat shock proteins ( hsps ) , which are homologues of human hsps , and the mucosal antigens result in the selection of autoreactive t cells21 ) .
have proposed that autoimmunity to components of the cartilage other than type ii collagen , such as proteoglycans or elastic tissue , could be the common mechanism of bd and rp2 ) .
in particular , elastic tissue is ubiquitous in the human mucosa that is mainly involved in bd , and in the cartilage too , which is the major target of rp .
however , because of the rarity of the combined cases , there have not yet been any investigations about the common pathogenesis of the two diseases , and so additional research is clearly needed .
although there have been only a few reports about polychondritis combined with bd , we think this combination of diseases might have been overlooked so far and closer scrutiny would reveal more cases .
 polychondritis is an inflammatory disorder that affects various catilagenous structures , and the clinical features include auricular , nasal and respiratory tract chondritis . 
 it also involves the eyes , audiovestibular apparatus , joints and vascular structures . 
 polychondritis can be associated with several rheumatologic diseases such as systemic lupus erythematosus , rheumatoid arthritis and systemic vasculitis . 
 however , polychondritis is a rare complication of behet 's disease ( bd ) and only ten cases with combined bd and polychondritis have been reported on around the world . in this report 
 , we describe a 40-year - old korean man with bd who suffered from polychondritis that manifested as bilateral auricular chondritis , conjunctivitis and arthritis . 
INTRODUCTION
CASE REPORT
DISCUSSION