Rosen & Barkin's 5-Minute Emergency Medicine Consult (694 page)

• Sexually transmitted disease
  
• 12 million new cases diagnosed annually worldwide
  
• Acquired via mucous membranes/disrupted skin
  
• Divided into 3 stages:
  
  • Primary syphilis:
    
    • Painless chancre or ulcer
      
  • Secondary syphilis:
    
    • Replication and hematogenous spread
      
    • Begins 3–6 wk after primary lesion
      
    • Late latent secondary phase
      
  • Tertiary or late syphilis:
    
    • Very uncommon
      
    • Cardiovascular and neurologic symptoms
      

Treponema pallidum
:

• Spirochete bacteria
  

DIAGNOSIS

• 21-day incubation period
  
• No constitutional symptoms
  
• Chancre:
  
  • Painless papule at site of inoculation
    
  • Clean-based, circular, sharply defined borders:
    
    • Solitary lesions
      
    • Commonly on penis, vulva, and rectum
      
    • Bilateral regional lymphadenopathy
      
  • Heals spontaneously in 3–6 wk
    
• Rectal chancre:
  
  • Painful or painless
    
  • Rectal irritation/discharge
    
  • Painless enlargement of lymph nodes
    

• Occurs 3–6 wk after primary lesion
  
• Disseminated stage
  
• Rash (most common):
  
  • Symmetric, diffuse, polymorphous, papular, or maculopapular rash
    
  • Rash may be diverse and not fit a pattern
    
  • Starts on trunk and flexor extremities
    
  • Spreads to involve palms and soles:
    
    • Discrete, red/reddish-brown
      
    • 0.5–2 cm in diameter
      
• Condyloma lata:
  
  • Large raised gray/white lesions, painless, moist
    
  • Mucous membranes:
    
    • Oral cavity and perineum
      
    • Very contagious
      
    • Intertriginous areas
      
    • Flat rectal warts
      
• Systemic symptoms:
  
  • Fever, headache, malaise, anorexia, sore throat, myalgias, and weight loss
    
• Diffuse lymphadenopathy:
  
  • Palpable nodes at inguinal, axillary, posterior cervical, femoral, and/or epitrochlear regions
    
  • Painless, firm, and rubbery
    
• Less common:
  
  • “Moth-eaten” alopecia
    
  • Syphilitic meningitis
    
  • Scleritis
    
• Loss of lateral 3rd of eyebrows
  
• Painless mucosal lesions (mucous patches)
  
• Secondary stage resolves spontaneously in 1–2 mo
  

• Begins after primary and secondary symptoms resolve.
  
• Period of no symptoms but positive serology:
  
  • CSF normal
    
• Late latent stage not infectious except for fetal transmission in pregnant women
  
• Persists for lifetime or develops into tertiary syphilis
  

• Occurs in about 15% of patients with untreated latent secondary syphilis
  
• Can appear 10–20 yr after initial infection
  
• Neurologic and cardiovascular involvement:
  
  • Destructive stages of disease
    
• Neurosyphilis (most common):
  
  • Asymptomatic:
    
    • Positive CSF – Venereal Disease Research Laboratories (VDRL)
      
    • CSF pleocytosis (10–100 lymphocytes)
      
    • Elevated CSF protein at 50–100 mg/dL
      
  • Meningitis:
    
    • Aseptic; CSF with positive VDRL, higher protein, and lower glucose (compared with above)
      
    • Cranial nerve palsy, including isolated 8th nerve palsy
      
  • General paresis:
    
    • Loss of cortical function
      
    • Argyll Robertson pupils (small fixed pupils that do not react to strong light, but do react to accommodative convergence)
      
  • Tabes dorsalis (peripheral neuropathy)
    
• Degeneration of posterior columns/posterior or dorsal roots of spinal cord
  
• Dementia
  
• Paresthesias, abnormal gait, and lightning (sudden, severe) pain of extremities/trunk
  
• Progressive loss of reflexes, vibratory/position sensation
  
• Positive Romberg sign
  
• Vision: Optic atrophy
  
• Pupils: Argyll Robertson pupils
  
• Urinary incontinence
  
• Gummas:
  
  • Late benign syphilis of cutaneous skin/viscera:
    
    • Bone, brain, abdominal viscera, etc.
      
• Granulomatous, cellular hypersensitivity reaction:
  
  • Round, irregular, or serpiginous shape
    
  • “Great pox”
    
• Cardiovascular:
  
  • Thoracic aortic aneurysm (ascending most common):
    
    • Dilated aorta and aortic valve regurgitation
      
    • Aortic valve insufficiency
      
    • Coronary thrombosis
      
    • Destructive lesions of skeletal structures or skin
      
• HIV-infected:
  
  • Strong association with syphilis
    
• Increased incidence of neurosyphilis
  

• In utero infection:
  
  • Age <2 yr:
    
    • Hepatosplenomegaly, rash, condyloma lata, rhinitis (snuffles), jaundice (nonviral hepatitis), osteochondritis
      
• Older children (syphilis stigmata):
  
  • Interstitial keratitis, nerve deafness, anterior bowing of shins, frontal bossing, mulberry molars, Hutchinson teeth, saddle nose, etc.
    

Rapid plasma reagin (RPR)

• Serology:
  
  • Nontreponemal test:
    
    • RPR
      
    • VDRL
      
    • Positive 14 days after chancre appears
      
    • Early false negatives, especially ≤7 days after primary chancre
      
    • Repeat negative test in 2 wk and correlate with disease activity
      
    • False positives in 1–2% of general population
      
    • 4-fold change in titer clinically significant
      
    • 100% sensitivity in secondary syphilis
      
    • Nonreactive after successful treatment
      
  • Treponemal antibody test:
    
    • Fluorescent treponemal antibody absorption (FTA–ABS)
      
    • Hemagglutination assay for antibody to
      T. pallidum
      (MHA–TP)
      
    • More sensitive and specific
      
    • 1% false-positive rate
      
    • Confirmatory test
      
    • Reactive for patient’s lifetime
      
    • More costly and harder to perform
      
  • Dark-field microscopy:
    
    • Identifies treponemes from primary and secondary lesions
      
    • Suspicious early lesions with negative serology (early primary syphilis)
      
    • False negatives with ointments, creams
      
    • Oral specimen unsuitable
      
  • CSF analysis for tertiary neurosyphilis:
    
    • Tertiary syphilis
      
    • Positive VDRL/RPR
      
    • Lymphocytes >5/mL
      
    • Protein >45 mg/dL
      
    • Decreased glucose
      

• Genital ulcer:
  
  • Chancroid (painful)
    
  • Genital herpes:
    
    • Vesicular, multiple lesions
      
  • Lymphogranuloma venereum
    
  • Granuloma inguinale
    
  • Superficial fungal infection
    
  • Carcinoma
    
• Secondary and tertiary syphilis:
  
  • Pityriasis rosea
    
  • Drug-induced rash
    
  • Acute febrile exanthems
    
  • Psoriasis
    
  • Lichen planus
    
  • Scabies
    
  • Infectious mononucleosis
    
  • Viral illness
    
  • Bacteremia
    
  • Tertiary syphilis:
    
  • Psychosis
    
  • Dementia
    
  • Multiple sclerosis
    
  • Meningitis
    
  • Encephalitis
    
  • Delirium
    
  • Unknown overdose
    

TREATMENT

Lower BP and establish IV access for aortic dissection.

• Treatment other than penicillin with increased relapse rate:
  
  • Desensitize those allergic to penicillin.
    
• Pregnancy:
  
  • Treat with penicillin even in latent syphilis.
    
  • If patient allergic to penicillin, admit for desensitization.
    
• Jarisch–Herxheimer reaction:
  
  • Transient febrile reaction to therapy
    
  • May be owing to antigen liberation from spirochetes or activation of complement cascade
    
  • Peaks at 8 hr, resolves in 24 hr
    
  • Symptoms:
    
    • Fever, headache, malaise, worsening rash
      
  • Treat with antipyretics
    
  • No serious sequelae
    
• Recommended testing:
  
  • Sexual partners
    
  • Concomitant sexually transmitted diseases including HIV
    
  • Repeat serology test in 6 and 12 mo.
    

• Early primary, secondary, early latent (<1 yr):
  
  • Benzathine penicillin G: 2.4 million U IM
    
  • Doxycycline: 100 mg PO BID for 14 days
    
  • Tetracycline: 500 mg PO QID for 14 days
    
• Late latent (>1 yr) except neurosyphilis:
  
  • Benzathine penicillin G: 2.4 million U IM 3 times over 2 wk on days 0, 7, and 14
    
  • Doxycycline: 100 mg PO BID for 4 wk
    
  • Tetracycline: 500 mg PO QID for 4 wk
    
• Neurosyphilis:
  
  • Penicillin G: 3–4 million U IV q4h for 10–14 days
    
  • Procaine penicillin: 2.4 million U IM daily +
    
  • Probenecid: 500 mg PO QID for 10–14 days
    
• Congenital syphilis:
  
  • Penicillin G: 50,000 U/kg IM q8–12h for 10–14 days; or
    
  • Procaine penicillin: 50,000 U/kg IM daily for 10–14 days
    

FOLLOW-UP