Description
The patient was a middle aged female. She was propped up in
bed, using four pillows. She was short and obese. The obesity was mainly truncal
with thin upper and lower limbs in comparison to her trunk.
On examination of her head, the head appeared large and the
shape of the head was rounded in appearance. This change in size and shape
seemed to be due to deposition of fat.
Her nose was beaked in shape; the skin over it was tight.
She had nasal prongs on and they were delivering oxygen.
There were multiple telangiectasiae over her malar region.
Her mouth appeared small and her lips were small as well. There was perioral
puckering of the skin.
On examination of the arms, the discrepancy between the
size of the arms and the size of the trunk was noted once more.
The patient’s hands looked small and they appeared narrow.
No nail fold capillary dilatation was seen. The skin over the fingers was shiny,
smooth, tight and bound to the underlying bone. This was so severe that there
were flexion contractures of the fingers of the right hand. The fingers were
narrow and tapered.
Over the extensor aspect of her left elbow, there were
multiple superficial, white, hard nodules, which were attached to the overlying
skin.
On examination of function of her hands there was marked
impairment of function more on the right than the left side.
Further examination revealed no elevation of the JVP, no
parasternal heave, an ejection systolic murmur was heard in the aortic area and
this was conducted to the neck. The second heart sound was of normal intensity
and single. There was no diastolic murmur.
Bilateral fine, late inspiratory crepitations were heard
over both lung bases.
Analysis
♥
The patient had central obesity with matchstick upper and lower
limbs. This suggests that the patient is on steroids.
(Page 66, 87)
ª
The moon face seen on examination of the head reinforces the
initial impression that the patient is on steroids.
(Page 73)
¨
The beak shaped nose suggests the patient has systemic sclerosis.
(Page 142)
§
The nasal prongs delivering oxygen suggests the patient has
dyspnoea. In systemic sclerosis this could mean the patient either has pulmonary
fibrosis, pulmonary hypertension, cardiac failure or pericardial effusion.
(Page 560)
♥
The malar telangiectasiae, small mouth and perioral puckering
reinforce our view that the patient has systemic sclerosis.
(Page 552,553)
ª
The thin arms reinforce our view that the patient is on
steroids.
¨
The change in size and shape of the hands reinforce the diagnosis
of systemic sclerosis. (Page 554)
§
The skin changes and the changes in the fingers continue to
reinforce the diagnosis. (Page 555)
♥
The finding of calcinosis reinforces the diagnosis further.
(Page 554)
ª
The poor function of the hands reveals that the disease is severe
causing functional impairment of the locomotor system.
(Page 530)
¨
The fact that the JVP is not elevated, there is no parasternal
heave, no loud second sound, no diastolic murmur excludes pulmonary hypertension
and cardiac problems accounting for dyspnoea. (Page
197,203,206,211)
§
The finding of a n ejection systolic murmur conducted to the neck
suggests coincidental aortic stenosis. (Page 210,
213)
♥
Fine end inspiratory crepitations heard bilaterally suggest the
patient has pulmonary fibrosis. (Page 264)
Diagnosis
ª
Systemic sclerosis with functional impairment of the locomotor
system and pulmonary fibrosis
¨
Iatrogenic Cushing’s syndrome
Systemic sclerosis
Cushing's syndrome
