Case presentation of 21 yr old male with unresolving

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Case presentation of21 yr old male with
unresolving pharyngeal ulcers

• Hoveda Mufti M.D.
• Memorial Hospital of Rhode Island

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• Chief Complaint sore throat
• History of presenting illness A 21 yr old man of
  Cape Verde origin presented to ED with complaints
  of a sore throat. He was empirically treated with
  Penicillin G IM and Ibuprofen although a rapid
  strep test was negative. Patient was discharged
  from the ED, over the next 14 days his symptoms
  persisted and he developed painful oral and
  scrotal ulcers.
• He returned to the ED and was given Ceftriaxone
  IM and Azithromycin PO and was discharged with a
  follow up appointment with the Infectious
  Disease clinic.

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• Patient did not experience relief of his symptoms
  and developed a painful lower extremity rash
  which he described as red bumps that broke out
  all over his lower legs.
• The oral ulcers persisted, he experienced new
  onset odynophagia, his rash progressed and his
  overall condition deteriorated. He returned to
  the ED 19 days after his first presentation and
  he was admitted for further workup.

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• Review of Systems
• Negative for
• changes in vision, headaches, sinus pain,
  shortness of breath, cough, hemoptysis, chest
  pain, nausea, vomiting, diarrhea, abdominal pain,
  melena, bleeding per rectum, fever, dysuria,
  urethral discharge, hematuria, joint pains, calf
  pain, sick contacts, recent travel.
• Positive for
• Wt loss of 5 lbs over one month, some fatigue in
  addition to the above complaints.

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• Past medical history
• Non significant.
• No prior hospitalizations.
• frequent sore throat unable to elaborate
  further. Not followed by a primary care
  physician.
• Surgical none
• Allergies NKDA, no food allergies.
• Medications Tylenol prn, Advil prn, ASA prn.

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• Family history no family members with similar
  complaints.
• Social
• Fork lift driver. Came to the US in 1992.
• Drinks 6 pack beer daily, cocaine (snorts) and
  marijuana use several times a week.
• Denies IV drug use.
• 2-3 female sexual partners currently. Denies
  homosexual encounters. No barrier protection
  used.

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• Physical Examination
• BP 132/80 P 70/min R 16/min T 97.9 S 98
  RA
• General No apparent distress. Alert and oriented
  to place, person and time.
• Head and neck
• Facial acne, shallow ulcers 0.5-1 cm with minimal
  white exudate around both tonsillar pillars, and
  2x2 cm ulcer partially covered by yellow exudate
  on left posterior pharyngeal wall. No visible
  bleeding. Enlarged, firm lymph node approximately
  8mm in right anterior cervical chain.
• Anicteric sclera, pupils equal and reactive to
  light, no nasal mucosa ulcerations.

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• Lungs clear to auscultation bilaterally.
• CVS Regular rate and rhythm of s1 and s2.
• ABD soft, non-tender and non-distended. Bowel
  sounds audible. Liver palpable 1 cm below costal
  margin. No other masses appreciated.
• GU 0.5 cm shallow painful ulcers on scrotum.
  Regular margins, clean based. No urethral
  discharge, no inguinal lymphadenopathy or
  scarring.

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• Extremities
• multiple 0.5-1 cm erythematous, minimally raised,
  purple lesions on lower extremities bilaterally
  and circumferentially below knees, tender to
  palpation.
• 2-4 5 mm pustules w/ yellow discharge on left
  lower leg.
• Left ankle minimally swollen and tender to
  palpation at tibiotalar joint.
• Rectal OB negative. No perianal lesions.

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• Laboratory data
• Rapid strep test and culture negative.
• Throat culture for gonorrhea negative
• Urine/serum tests for Gonorrhea/chlamydia
  negative
• STS negative.
• Upper respiratory culture/Viral throat culture
  negative .
• HIV negative
• Herpes DFA negative
• PPD negative (lt 4mm)
• Hb 11.5 Hct 34.2
• WBC 7.9 N 67 E 0.7
• Platelets 495
• Na 139 K 3.3 CL 100 HCO3 23 BUN 10
• Creatinine 0.7

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• AST, ALT, amylase, lipase normal
• UA nitrite/ LE negative, WBC/RBC negative,
  Protein 1.
• ESR 109
• Chest PA and Lateral normal with no evidence of
  hilar adenopathy.

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• Differential diagnosis of oral ulcers
• Herpes Simplex
• Benign apthous ulcers
• Inflammatory bowel disease
• SLE
• Drugs such as methotrexate
• Pemphigus vulgaris
• Hematological disorders such as lymphoma

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Differential diagnosis of multi-system diseases
with such a presentation

• SLE
• Familial mediterranean fever
• Sarcoidosis
• Reactive arthritis
• Multiple sclerosis
• Behcets disease
• Malignancies
• Henoch Schonlein purpura

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• Course in hospital
• Pt was seen by Infectious disease and
  Rheumatology and Behcets disease was considered
  the most likely diagnosis. Dermatology obtained
  biopsies of lower extremities which showed
  pathology suggestive of Behcets disease.
• This is considered a chronic inflammatory
  vascular disease of unknown etiology.

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Pathology of Behcets disease

• Panniculitis with perivascular infiltrates of
  lymphocytes and neutrophils
• Leukocytoclastic vasculitis
• Fibrin thrombi
• Intraepidermal or subepidermal vesicles and
  ulcerations
• Suppurative folliculitis

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• Patient was started on oral Colchicine,
  Indomethacin and triamcinolone ointment for oral
  ulcers, which significantly improved his pain.
  Ophthalmology consulted to rule out complications
  of this disease, patients ocular exam was normal.
• He was discharged the next day with instructions
  to follow up with rheumatology in one week.

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Discussion diagnosis

• Behcets disease is a clinical diagnosis.
• International criteria published in 1990 require
  the presence of recurrent oral ulcers 3 times in
  one year plus any two of the following, after
  exclusion of other systemic diseases
• Recurrent genital ulcers
• eye lesions such as uveitis, retinal vasculitis,
  or cells in vitreous on slit lamp examination.
• Skin lesions erythema nodosum,
  pseudo-folliculitis papulopustular lesions or
  acneiform pustules
• Positive pathergy test 2mm plus papule
  developing over 24-48 hrs after oblique insertion
  of a 20 gauge needle into skin.

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Pathophysiology

• Etiology is unknown.
• Considered a multi-systemic vasculitis.
• Other theories propose an autoimmune response to
  an antigen such as infectious agent or
  environmental agents like heavy metals, in those
  with genetic predisposition.
• Acute phase reactants (ESR, CRP) maybe be
  elevated. Behcets disease is a difficult
  diagnosis to make oral ulcers and other lesions
  must be documented by a physician and symptoms
  may not occur at once and may be separated by a
  period of several years.

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• Prevalence
• More common in Middle Eastern and Far Eastern
  populations (Japan, Korea, China) but is seen
  worldwide.
• Turkey 80-370/100,000
• China 13-20/100,000
• It also varies between men and women indifferent
  ethnicities for e.g. its more common in men in
  the Middle East, and in women in the Far East
  (Korea, Japan).

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Clinical presentation of Behcets

• A common clinical feature in patients is painful,
  recurrent muco-cutaneous ulcers.
• Oral ulcers
• Size ranges from a few mm to 2 cm.
• Grossly and histologically similar to common oral
  ulcers. Multiple.
• Often heal spontaneously in 1-3 weeks.

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Genital ulcers

• Occur in 75 of patients.
• Appear similar to oral ulcers.
• Most frequently scrotal in men, vulvular in
  women.
• Epididymitis, salpingitis, urethritis may occur.
• Cutaneous lesions
• Acneiform lesions, nodules, erythema nodosum,
  superficial thrombophlebitis, palpable purpura,
  pyoderma-gangrenosum.
• Pathergy test
• Less common in North American/European patients.
• Dermatographism response to light scratching of
  skin present in some patients.

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Ocular disease

• Less common in North American patients.
• Uveitis is bilateral, episodic sometimes with no
  resolution between episodes.
• Hypopyon purulent material present in anterior
  chamber indicating severe anterior uveitis.
• Posterior uveitis, retinal vasculitis, vascular
  occlusion, optic neuritis may progress to
  blindness if untreated. May require
  immunosuppressive treatment.
• Glaucoma, cataracts can occur.

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• Neurological disease
• More common in males
• Occurs in less than 20 of patients
• Commonest abnormalities are vascular thrombosis
  and focal parenchymal lesions.
• Manifestations include progressive personality
  change, psychiatric disorders, dementia, aseptic
  meningitis, encephalitis. Peripheral neuropathy
  is not common.
• Arteritis can lead to dissection, aneurysmal
  dilatation and subarachnoid hemorrhage.

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Vascular disease

• Venous involvement occurs more commonly than
  arterial.
• Superficial and deep venous thrombosis is common.
• Many patients have small vessel vasculitis.
• 30 have large vessel involvement, including
  stenosis and aneurysms.
• Pulmonary arterial involvement is characteristic.
• Hemoptysis made indicate arterio-bronchial
  fistula.
• However coronary vascular disease and myocardial
  infarction are uncommon.

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Renal disease

• Usually mild when present - glomerulonephritis.
• Arthritis
• is nonerosive, inflammatory oligoarthritis
• Occurs in 50 of patients
• Asymmetric and usually does not result in
  deformities.
• Commonly affects medium/large joints.
• Occurs particularly during exacerbations, lasts
  1-3 wks.

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GI disease

• Symptoms include nausea, diarrhea, abdominal
  pain.
• Oral ulcers in Behcets are indistinguishable
  from those found in IBD.
• When gastrointestinal ulcerations occur they are
  found in terminal ileum, cecum, ascending colon.

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Prognosis and clinical course

• Variable with exacerbations and remissions.
  Typical presentation is initially with
  mucocutaneous lesions and subsequently ocular and
  neurological disease occurring years after
  diagnosis.
• Mortality is low and is usually related to
  pulmonary or CNS hemorrhage or bowel perforation.
  
• Most common cause of morbidity is ocular disease
  which can result in blindness if untreated.

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Treatment

• Is based on degree of involvement and individual
  presentation.
• Studies of treatment protocols are small.
• Agents used- mucocutaneous disease
• Topical steroids
• Colchicine
• Dapsone

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• Severe mucocutaneous disease
• Methotrexate
• Prednisone
• Interferon Alpha
• Thalidomide
• Systemic disease
• Prednisone
• Azathrioprine
• Cyclophosphamide
• Anti-TNF drugs

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Concluding points

• The diagnosis is difficult particularly in
  patients with partial symptomatology and also
  because it relies on clinical criteria. Therefore
  a high level of suspicion is warranted on part of
  physicians who may be the first to encounter
  diseases initial manifestation. In this patient
  the series of symptoms fortunately presented in a
  rapid sequence prompting further investigation.
• Behcets disease was probably first described by
  Hippocrates in the 5th century and introduced to
  the modern world by Turkish dermatologist Hulusi
  Behcet in 1937.
• The etiology remains ill-defined and the field is
  open for development of newer and better
  treatments.