Pic & Case

good ppl....
Wilson's Disease
Answer: e
• First described by Westphal, not Wilson, in 1883
• An autosomal recessive defect in biliary excretion of copper
results in deposition in the liver, brain, and cornea, among
other tissues
• Kayser-Fleischer rings are more frequent in patients with
neurologic complications
• Neurologic findings include the following:
Personality changes
Rigidity
Tremor
Spasticity
Dysarthria
Dysphagia
• Liver manifestations include the following:
Cirrhosis
Cholestasis
Hepatitis
• Arthropathy occurs in 50% of patients
• Wilson’s disease was universally fatal until 1951, when it
was discovered that British antilewisite, a chelator used for
arsenic poisoning, increased urinary copper excretion and
resulted in reversal of the tremor and rigidity of Wilson’s
disease
• Wilson’s disease is now treated with penicillamine (increases
urinary excretion of copper) with or without zinc (inhibits
copper absorption in the gastrointestinal tract).

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Case 2:

A 16-year-old high school boy has a sore throat and the rash
shown here, which started on the trunk and spread to the arms
and legs but not the palms and soles. The appearance of his
tongue is also shown. What is the most likely diagnosis?
a. Measles
b. Kawasaki disease
c. Toxic shock syndrome
d. Scarlet fever
e. Ehrlichiosis

right... dr ayyad
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Answer: d
Scarlet Fever
• The causative agent is group A streptococcus (Streptococcus
pyogenes)
• Strawberry tongue is caused by enlarged papillae
• The characteristic “sandpaper” rash spreads from trunk to
extremities, beginning 1 to 2 days after initial fever, sore
throat, and vomiting. Circumoral pallor also is thought to
be characteristic
• After about a week, the rash desquamates, as do the palms
and soles
• Hyperpigmentation in the bend of the elbow (Pastia’s sign)
is thought by some to be pathognomonic of scarlet fever
• Differential diagnosis includes measles, toxic shock syndrome,
staphylococcal scalded skin syndrome, erythema infectiosum
(fifth disease), Kawasaki disease, and drug reactions
• Before the advent of antibiotics and antipyretics, severe cases
resulted in extremely high fevers (107°-108°F), painful
lymphadenopathy, delerium, convulsions, and death
• Scarlet fever is much less common now than in the past. It
is estimated that less than 5% of streptococcal infections in
the United States present as scarlet fever
• Scarlet fever predisposes to rheumatic fever, the incidence
of which also has declined with the advent of effective
treatment
• Penicillin remains the treatment of choice

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Case 3:

A 34-year-old male smoker reports having rest pain and lower
extremity digital ulcers for 2 months. He also reports a 1-year
history of claudication. Which one of the following is the best
means of preventing amputation?

a. Smoking cessation
b. Calcium channel blockers
c. Peripheral artery bypass
d. Pentoxifylline
e. Warfarin anticoagulation

you are right......
Answer: a
Buerger’s Disease (Thromboangiitis Obliterans)
• The cause of Buerger’s disease is unknown, but it is closely
linked to smoking
• Few, if any, cases occur in the absence of tobacco use
• The disease affects the small and medium-sized arteries and
veins of the extremities
• Affected vessels are thrombosed and infiltrated with inflammatory
cells, resulting in ischemia
• Intraluminal thrombus contains microabscesses
• Markers that are common in other vasculitides are usually
negative or normal in Buerger’s disease, including sedimentation
rate, C-reactive protein, complement, cryoglobulins,
antinuclear antibodies, and rheumatoid factor
• In the Mayo Clinic experience, the incidence of the disease
declined from 104 per 100,000 in 1947 to 12.6 per 100,000 in
1986
• Most patients (75%) are men
• 40% of persistent smokers with Buerger’s disease face
amputation
• The disease arrests with smoking cessation, and patients
who quit smoking usually avoid amputation

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Case 4:

A 78-year-old male smoker presents with profound weight loss
and a chronic cough. During World War II he was employed at
the local navy shipyard. Which one of the following is the most
common pulmonary finding in this disorder?
a. Pulmonary embolism
b. Pleural effusion
c. Pleural plaques
d. Pulmonary fibrosis
e. Malignant mesothelioma

great dr.ayyad ....
Answer: c

Asbestos Exposure

• Exposure to asbestos fibers has several clinical associations,
including pleural effusions, pleural plaques (most common),
and malignant mesothelioma. Lower lobe abnormalities
predominate in asbestos exposure
• Cumulative dose and time since first exposure are key
determinants to the toxicity of asbestos illnesses. Clinical
manifestations may occur 15 to 40 years after initial exposure
• Two classes of asbestos fibers:
Serpentine–curly. Chrysotile is a subtype and accounts
for 95% of asbestos used worldwide
Amphibole–straight and rodlike, greater persistence in
lung tissue, and generally considered more pathogenic
than serpentine asbestos fibers, which more readily
dissolve in vivo
• Asbestosis refers to diffuse interstitial lung disease
(pulmonary fibrosis), which in severe cases may lead to
cor pulmonale
• Evidence suggests that release of free radicals has a pivotal
role in inducing lung injury after asbestos exposure
• Smoking does not increase the death rate in patients with
malignant mesothelioma. It does, however, greatly increase
the death rate in patients with asbestosis (up to 3 times)
• Smoking in combination with asbestos exposure substantially
increases the risk for development of bronchogenic carcinoma
(shown in this case)

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Case 5:

A 50-year-old woman presents with the lesions shown here,
which are yellow on cut section. All of the following are true
about this condition except:
a. These lesions develop in 10% of patients with primary
biliary cirrhosis
b. These lesions consistently correlate with increased
cholesterol levels
c. These lesions are benign
d. Incidence increases with age
e. Women are more likely to have this condition than men

good qusei....
Answer: b

Xanthelasma (Xanthoma Palpebrarum)

• Xanthelasma is the most common cutaneous xanthoma
• Soft, semisolid, or calcific yellow plaques on eyelids or inner
canthi are characteristic
• Diagnosis is purely clinical
• Microscopic examination reveals lipid-filled foamy histiocytes
• There is no consistent association with hyperlipidemia
• Serum lipid values are normal in many series (25%-70%;
average, 50%)
• Xanthelasma is more common in women than in men
• Incidence increases with age
• Lesions tend to be symmetric, permanent, and progressive
• Xanthelasma may be associated with increased β-lipoprotein
values in young adults
• Xanthelasma is often noted in descriptions of primary
biliary cirrhosis, but it is a late manifestation and occurs
in only 10% of patients with this type of cirrhosis
• The lesions alone are benign
• Unless the lesions obstruct vision, treatment is for cosmetic
reasons only
• If pursued, treatment may involve excision, laser ablation,
and topical trichloroacetic acid
• Recurrence after all forms of treatment is common

Case 6:

A 13-year-old Sephardic Jewish boy had recurrent fever,
abdominal pain, arthritis, pleuritic chest pain, and the skin
lesions shown here. What is the treatment of choice?
a. Nafcillin
b. Cephalexin
c. Colchicine
d. Prednisone
e. Acetaminophen

colchicine for life

Answer: c

Familial Mediterranean Fever (FMF, or Familial
Paroxysmal Polyserositis)

• FMF is an autosomal recessive disease that affects mostly
patients of non-Ashkenazi (e.g., Sephardic) Jewish, Armenian,
Turkish, or Arabic descent
• FMF is marked by recurrent paroxysmal episodes lasting 12
to 72 hours involving inflammation of serosal tissues (e.g.,
pleura, peritoneum, and synovium). The commonly reported
symptoms include fever (96%-100%), abdominal pain (89%-
96%), chest pain (33%-57%), arthritis or arthralgias (21%-76%),
erysipelas-like rash (12%-41%), and amyloidosis (2%)
• Laboratory abnormalities during attacks often include
increased sedimentation rate, increased leukocyte count
(neutrophilic predominance), increased fibrinogen and
other acute-phase reactants, and microscopic hematuria
and proteinuria. These abnormalities usually resolve after
the attack
• Onset usually is before age 20 years, but late occurrences
have been reported
• The predominant gene mutation is thought to have originated
from a single common ancestor who lived about 2,500 years
ago, before the Babylonian captivity of the ancient Jews
• Colchicine prophylaxis (1-2 mg per day) greatly reduces the
number of attacks and may eliminate them altogether. This
therapy has been instrumental in decreasing the frequency
of amyloidosis in FMF

i have no idea....

Case 7:

A 42-year-old woman presents with pink-red macules of the
face, palms, and soles. She has had multiple sexual partners.
After she is treated with an antibiotic, fever, hypotension, and
worsening of the rash develop. Which one of the following
occurred?
a. Drug allergy
b. Sepsis syndrome
c. Jarisch-Herxheimer reaction
d. Angioedema
e. Stevens-Johnson reaction

Answer: c

Jarisch-Herxheimer Reaction

• The Jarisch-Herxheimer reaction occurs within 1 to 2 hours
of treatment of syphilis with antibiotics, especially penicillin
• The reaction is caused by release of pyrogen from the spirochetes
• It is most common during the treatment of secondary syphilis
(70%-90% of cases)
• The reaction is characterized by the following:
Fevers
Chills
Sweats
Headache
Hypotension
Worsening of the skin lesions
• The reaction usually resolves within 24 hours of treatment
• Treatment of the reaction is supportive only
• Similar reactions have been reported with treatment of the
following:
Lyme disease
Borreliosis
Brucellosis
Typhoid fever
Trichinellosis

Case 8:

A 54-year-old woman with diabetes arrives in the emergency
department complaining of light-headedness and confusion.
Her electrocardiogram is shown here. What do you conclude?
a. Acute pericarditis
b. Chronic pulmonary hypertension
c. Acute pulmonary embolism
d. Acute inferolateral myocardial infarction
e. Acute posterolateral myocardial infarction