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• A 66-year-old man presented to the clinic
with a 4-week history of painful,
edematous, and scaly ears, a condition
that was later accompanied by itchy,
hyperkeratotic plaques on the palms and
soles with surrounding erythema (Panels
A, B, and C). The skin lesions were noted
to be symmetric. Previous treatment with a
0.05% formulation of clobetasol cream
• What is the most likely diagnosis
associated with symptoms of pruritic skin
changes seen in these images?
• Inflammatory bowel disease
• Acute hepatitis C infection
• Cutaneous sarcoidosis
• Acrokeratosis paraneoplastica presents
with skin changes in the hands, feet, nose,
and ears. Periungal tissues may also be
involved. It is associated with malignancies
in the upper respiratory or gastrointestinal
tracts. Cutaneous changes typically involve
psoriasiform dermatitis with surrounding
• Acrokeratosis paraneoplastica of Bazex (also
known as "Acrokeratosis neoplastica," and "Bazex
syndrome") is a cutaneous condition characterized by
psoriasiform changes of hands, feet, ears, and nose,
with involvement of the nails and periungual tissues.
• The condition is associated with carcinomas of the
upper aerodigestive tract
• The pathophysiology of acrokeratosis paraneoplastica
is not understood. Proposed mechanisms include
cross-reactivity between skin and tumor antigens, the
action of tumor-produced growth factors, and even
• Approximately 140 cases of acrokeratosis
paraneoplastica have been reported in the literature.
• Morbidity and mortality in acrokeratosis
paraneoplastica are related directly to the underlying
neoplasm. The skin lesions of acrokeratosis
paraneoplastica rarely improve without successful
treatment of the underlying malignancy.
• Most acrokeratosis paraneoplastica cases are
associated with squamous cell carcinoma (SCC) of the
upper one third of the respiratory or GI tracts (ie,
oropharynx/larynx, lungs, esophagus)
• Treatment of acrokeratosis paraneoplastica depends
on the type and stage of the underlying neoplasm.
Successful treatment of the underlying malignancy
typically improves the skin lesions.
• Improvement has been reported with the
use of systemic retinoids, topical and oral
corticosteroids, salicylic acid, topical
vitamin D analogues, and psoralen plus
• One report suggests a role for zinc
supplementation in patients with
• Which of the following is the most likely
cause of a painless swelling seen in this
• Coronary angiography
• Median nerve compression
• Gout flare
• Rheumatoid arthritis
• Bullous pemphigoid
• Pseudoaneurysms occur infrequently as a
complication of transradial coronary angiography.
• Predisposing factors include the use of a large
catheter sheath, multiple punctures at the site, the use
of antiplatelet and antithrombotic agents, inadequate
hemostasis or postprocedure compression, and
vascular-site infection. The patient underwent surgical
excision of the pseudoaneurysm and suture of the
radial artery (next slide).
• She remained well at follow-up visits both 1 week and
2 months after the procedure, with preserved radial
arterial pulse and no residual swelling.
• What is the name for this finding that is
reducible on physical exam?
• Heberden’s nodes
• Rheumatoid nodules
• Jaccoud’s arthropathy
• Pseudogout tophi
• Erosive arthropathy of rheumatoid arthritis
• In Jaccoud’s arthropathy, the deformities are reducible and
result mainly from soft-tissue abnormalities, such as laxity of
ligaments, fibrosis of the capsule, and muscular imbalance,
rather than from destruction of the bone of joints, as occurs in
• the condition characterised clinically by 'reversible' joint
deformities such as swan neck, thumb subluxation, ulnar
deviation, along with an absence of articular erosions on a
• JA was initially described in patients with rheumatic fever
(RF), but as this disorder has become rare the main clinical
entity associated to JA at present is systemic lupus
erythematosus (SLE). JA has also been described in other
connective tissue diseases, infections and neoplasia. In
general, its prevalence in either SLE or RF is around 5%.
• The etiopathogenic mechanisms of JA are not
known, but some authors have suggested an
association with hypermobility syndrome.
• Presently, the therapy for JA is conservative and
based on the use of non-hormonal anti-inflammatory
drugs, low doses of corticosteroids, methotrexate
• The role of surgery through either the realignment of
soft tissue around the joint--or more aggressive
procedures such as arthrodesis, silastic implant and
arthroplasty--needs to be proven.
A 1-year-old girl presented with a history of growth failure, mild
anemia, hypophosphatemia, hypovitaminosis D, glucosuria, and
proteinuria. She underwent diagnostic bone marrow aspiration,
• Bone marrow revealed an increased proportion
of macrophages containing polygonal crystals
(20%; normal range, 2 to 5%), which are
pathognomonic for cystinosis
• Cystinosis is a lysosomal storage
disease characterized by the abnormal
accumulation of the amino acid cystine.
• It typically follows an autosomal
recessive inheritance pattern. Cystinosis is the
most common cause of Fanconi syndrome in the
pediatric age group.
• There (are) three distinct types of cystinosis each
with slightly different symptoms: nephropathic
cystinosis, intermediate cystinosis, and non-
nephropathic or ocular cystinosis.
• The signs and symptoms of intermediate
cystinosis are the same as nephropathic
cystinosis, but they occur at a later age.
Intermediate cystinosis typically begins to affect
individuals around age twelve to fifteen.
Malfunctioning kidneys and corneal crystals are
the main initial features of this disorder. If
intermediate cystinosis is left untreated, complete
kidney failure will occur.
• People with non-nephropathic or ocular cystinosis
do not usually experience growth impairment or
kidney malfunction. The only symptom is
photophobia due to cystine crystals in the cornea.
• Cystinosis is normally treated with a drug
called cysteamine (brand name Cystagon).
• The administration of cysteamine can reduce the intracellular
cystine content. Cysteamine concentrates inside the
lysosomes and reacts with cystine to form both cysteine and a
cysteine-cysteamine complex, which are able to leave the
lysosomes, When administered regularly.
• Cysteamine eye drops remove the cystine crystals in the
cornea that can cause photophobia if left unchecked.
• Patients with cystinosis are also often given sodium citrate to
treat the blood acidosis, as well as potassium and phosphorus
• A 70-year-old woman who presented to
the clinic for a routine examination was
found to have a 6-cm mass on her hard
palate. She reported that the mass had
been present since birth.
She reported that the mass had been present since
birth. Occasionally, she found that the area was hard to
clean and that entrapped food gave her halitosis, but
otherwise she was asymptomatic.
Palatal tori are seen in approximately 20 to 30% of the
population and are more common in females than in
males. Frequently, these bony outgrowths are
incidental findings on routine oral examinations, and
there is often considerable variation in clinical
The lesion in this patient was unchanged over a 2-year
period of follow-up. Surgical correction was offered, but
the patient declined because the lesion was
• A 9-year-old boy with a history of a lung
lesion presented to the emergency
department with fever and chest pain. ..
A chest radiograph (Panel A) showed a large
opacity in the right lung, abutting the minor
fissure and displacing it downward
Computed tomography of the chest revealed a large
heterogeneous mass with scattered areas of low attenuation,
suggesting mucous impaction, and a single large systemic artery
(Panel B, arrow) providing vascular supply from the descending
aorta. No air bronchograms were evident within the mass.
• Pulmonary sequestration is a congenital
disorder characterized by anomalous lung
tissue that lacks normal communication with
the tracheobronchial tree,
which increases the likelihood of infection.
• The patient underwent a right upper
• The patient recovered without complications
and since the operation has had no further
• An 83-year-old man was evaluated for a 1-
year history of a pruritic, progressively
worsening migratory rash, with associated
weakness and a 5-kg weight loss. He had
a 30-pack-year history of smoking; he had
stopped smoking 45 years earlier. …
Erythema gyratum repens
On examination, he
had erythematous skin
lesions that appeared
in concentric, raised,
(Panel A). The rash
affected mainly the
trunk and proximal
A computed tomographic scan revealed a
pulmonary mass measuring 59 by 43 mm (Panel B).
Bronchoscopy with biopsy revealed squamous-cell
carcinoma of the lung
Erythema gyratum repens is a rare syndrome
typically associated with an underlying
It occurs most frequently in conjunction with
lung cancer and next most frequently with
esophageal and breast cancers.
It may regress with treatment of the cancer.
Treatment with gemcitabine was initiated for
this patient, but he died 3 months after
diagnosis, after only one infusion; the rash
had not resolved.
• A 40-year-old man presented with a 5-
week history of headaches and blurred
vision in both eyes, with no other medical
• He reported having no chest pain or
shortness of breath.
• A physical examination was notable for a
blood pressure of 210/130, and the
visual acuity was 20/200 in both eyes.
Retinopathy in malignant
blurring (long arrow),
(short arrow), a
macular “star” with
in both the right eye
(Panel A) and the left
eye (Panel B)
The patient was admitted to the hospital and given
Patients with malignant hypertension, such as our
patient, have a diastolic blood pressure of 130 mm
Hg or higher in association with ocular pathologic
These ocular findings can also be caused by blood
dyscrasias, systemic lupus erythematosus,
neuroretinitis, sarcoidosis, or eclampsia.
Changes in the retina may result in permanent
loss of vision.
This patient was given atenolol, amlodipine, and
enalapril for the high blood pressure.
One month later, the fundus showed clinically
Two months after presentation, the patient's blood
pressure was normal (128/80 mm Hg), and his
visual acuity had improved to 20/60.