Australian Doctor Australian Doctor 3rd November 2017 | Page 15

Dermatology
Professor Dedee Murrell
Figure 1.
Figure 2.
Hair today
found type has carried the eponym for some time. 1
Still’s disease is uncommon. It begins at any time
after 18 months of age through to puberty. Post-
pubertal presentations are rare, but a variant —
adult-onset Still’s — is recognised.
In some cases there are few symptoms of arthri-
tis, presenting instead with predominantly systemic
disturbance including fever, pleural effusions, rash,
lymphadenopathy, splenomegaly or marked leuco-
cytosis and anaemia, making for a difficult diag-
nosis.
Previously this illness carried an appalling mor-
tality and morbidity, but today speedier diagnosis
and effective use of biological and non-biological
immunomodulants has largely tamed the disease.
Cases like Simon’s presenting with the late conse-
quences of juvenile onset disease are now fortu-
nately most uncommon.
Progress
In Simon’s case, the inflammation in the cervi-
A 45-YEAR-old man, otherwise well,
started noticing further receding of his
hair line, along with loss of hair volume
at the vertex of his scalp. He had an
older brother with hair loss who was on
simvastatin.
cal spine had caused fusion of the affected bones.
Simon’s rheumatologist mentioned that this is an
extremely rare presentation, “something which I
have only seen once or twice before” and arranged
for a neurosurgical opinion.
A decompressive laminectomy was recom-
mended. Significant changes were noted in the spi-
nal cord intra-operatively, suggesting that complete
resolution of symptoms was unlikely.
Simon subsequently underwent bilateral hip
replacements. However his persisting muscle spas-
ticity prevented successful rehabilitation.
Simon is now able to sit normally and comfort-
ably.
However, a combination of additional weight
gain, limited progress with post-operative rehab
and his existing motor deficits mean Simon is now
wheelchair-bound and requires nursing home
care. ●
THE QUIZ
Q. What is the most
likely diagnosis