This patient with end-stage renal disease developed skin changes after an imaging procedure. The thickening and induration of the skin on the arms is characteristic of nephrogenic systemic fibrosis (NSF), a rare systemic fibrotic disorder associated with exposure to gadolinium-containing MRI contrast agents in patients with kidney disease. NSF causes fibrosis of the skin, joints, and internal organs. The FDA recommends avoiding gadolinium contrast for patients with renal insufficiency due to the risk of developing NSF.

1. Dr. Ankur Banik
PGT
Dept Of General Medicine
BMCH

5. • Heberden’s nodes
• Rheumatoid nodules
• Jaccoud’s arthropathy
• Pseudogout tophi
• Erosive arthropathy of
rheumatoid arthritis

6.  Although classically described in rheumatic fever,
Jaccoud’s arthropathy has also been described in SLE
and other diffuse connective-tissue diseases.
 In Jaccoud’s arthropathy, the deformities 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 rheumatoid arthritis.
 The mainstay of the management of Jaccoud’s
arthropathy includes physical therapy and the use of
orthotic devices.

8.  A 40-year-old Caucasian man presented to the ER with severe pain
in his right hip, which came on suddenly after tripping on a
pavement. He denied falling and was unable to bear weight.
Peripheral neuropathy had been diagnosed in the past when he
developed bilateral foot drop, paraesthesia of his feet and erectile
dysfunction. The patient was in severe pain and his right leg was
shortened and externally rotated. The quadriceps were wasted
bilaterally, his feet were in splints for his foot drop and there were
multiple raised erythematous papules on his skin. The greater
trochanter was tender and he was unable to move the right hip. The
left leg moved normally. Heart sounds were normal and chest was
clear on auscultation. There was obvious splenomegaly but no other
abdominal abnormality. Neurological examination of the cranial
nerves and upper limbs was normal. Tone in the lower limbs was
increased bilaterally. It was not possible to fully assess power due to
the patient's hip pain, however, power was grade five bilaterally in
the upper limb and grade four in the left leg. Lower limb reflexes
were normal. Plantars were downward bilaterally. Sensation was
reduced in the L4-S1 region. Vibration sense, temperature,
nociception and 2-point discrimination were impaired in this
region.

9.  Radiographs of the hip revealed a pathological
comminuted intertrochanteric fracture of the proximal
right femur . No additional lesions were found on skeletal
survey. MRI showed a destructive lesion in the proximal
femur, expanding the bone with a well-defined sclerotic
margin. An enlarged spleen (17.4 cm) was also evident on
CT. Urine was negative for Bence–Jones protein. Serum
immunofixation was consistent with immunoglobulin Gλ
monoclonal gammopathy. Laboratory analysis revealed a
normal full blood count, urea and electrolytes, liver
function and thyroid function tests. Plasma glucose and
haemoglobin A1c were normal. Other Blood tests revealed
hypotestosteronaemia and hyperprolactinaemia.
 Diagnosis?

11.  The diagnosis of POEMS syndrome was made by a combination
of history and examination findings. The presence of a
pathological fracture, splenomegaly, skin changes,
prolactinaemia, erectile dysfunction and peripheral neuropathy
in a young male suggested a multisystem disorder. The diagnosis
of multiple myeloma seemed unlikely given that no oligoclonal
bands were seen on immune electrophoresis, urine Bence–Jones
protein was negative and calcium was normal. In addition,
classic multiple myeloma has never been reported in association
with POEMS.The presence of peripheral neuropathy made the
diagnosis of myeloma less likely as 50% of patients with
osteosclerotic myeloma have peripheral neuropathy, while this
occurs in only 1–8% of patient with multiple myeloma.MGUS
and Waldenstrom's macroglobulinaemia both result in the
presence of an M protein on electrophoresis but, unlike POEMS,
they are not associated with symptoms of end organ damage
such as anaemia and organomegaly.

12.  Also known as Crow–Fukase syndrome, peculiar
progressive polyneuritis syndrome or Takatsuki syndrome,
POEMS syndrome is a rare multisystem syndrome
associated with plasma cell dyscrasia that consists of the
following features: polyneuropathy, organomegaly,
endocrinopathy, monoclonal gammopathy and skin
changes.
 Peak incidence occurs at age 50–60, and progressive
peripheral neuropathy is the predominant feature.
 The mechanism by which plasma cells cause POEMS
syndrome is not fully understood, but it is thought to
involve elevations in vascular endothelial growth factor
(VEGF), interleukin 6 (IL6), tumour necrosis factor-α and
IL1β

13. The most recent criteria for the diagnosis of POEMS syndrome
require that all of the following be present:
 polyneuropathy
 a monoclonal plasma-cell proliferative disorder
 one or more of the following major criteria:
 sclerotic bone lesions
 Castleman's disease
 elevated VEGF levels
 one or more of the following minor criteria:
 organomegaly (enlarged spleen, liver or lymph nodes)
 extravascular volume overload (oedema, pleural effusion or ascites)
 endocrinopathy (adrenal, pituitary, gonadal, parathyroid); diabetes
or hypothyroidism alone are insufficient
 skin changes
 papilloedema
 Thrombocytosis or polycythaemia

14.  There are no randomised controlled trials of treatment in
POEMS syndrome, owing to its rarity.Conventional treatments
for demyelinating neuropathy, such as steroids, intravenous
immunoglobulin and plasma exchange, are ineffective;
treatment must be aimed at the haematological disorder.
 Prednisolone and alkylating agents are the most commonly used
 hematopoietic stem cell transplantation
 Radiotherapy or surgical excision of osteosclerotic lesions that
are localized may temporarily (and sometimes permanently)
lead to a remission of symptoms
 Given that VEGF plays a central role in the symptoms of POEMS
syndrome, some have tried bevacizumab (Avastin), a
monoclonal antibody directed against VEGF.

16.  A 26 year old female presented with chief complain of
proximal muscle weakness & pain with swelling & rash
in the face.

18.  Dermatomyositis (DM) is a connective-tissue disease
characterized by inflammation of the muscles and the
skin. While DM most frequently affects the skin and
muscles, it is a systemic disorder that may also affect the
joints, the esophagus, the lungs, the heart.
 An autoimmune etiology is supported by an association
with other autoimmune or connective tissue disease with
various auto antibodies.
 Some cases of dermatomyositis actually "overlap" (i.e. co-
exist with) other autoimmune diseases such as Sjögren's
syndrome, lupus, scleroderma, or vasculitis.

19.  The main symptoms include skin rash and
symmetric proximal muscle weakness which may
be accompanied by pain.
 The pain may resemble the type experienced after
strenuous exercise. Some dermatomyositis
patients have little pain, while in others , the pain
may be severe.
 Skin findings occur in dermatomyositis but not in
PM and are generally present at diagnosis.

21.  Clinically suspected case of of DM is confirmed by
serum muscle enzyme (CK),EMG finding & muscle
biopsy.

22. Cross section of a muscle biopsy show atrophy of the fibers at the
periphery of the fascicle(perifascicular atrophy)

23. Drug used in the management of DM:
 Prednisolone
 Methotrexate
 Mycophenolate
 Intravenous immunoglobulin
 Azathioprine
 Cyclophosphamide
 Rituximab

24.  The heliotrope rash is a violaceous eruption on the
upper eyelids and in rare cases on the lower eyelids
as well, often with itching and swelling (most
specific, though uncommon).
 Shawl (or V-) sign is a diffuse, flat, erythematous
lesion over the back and shoulders or in a "V" over
the posterior neck and back or neck and upper
chest, which worsens with UV light.
 Gottron's sign is an erythematous, scaly eruption
occurring in symmetric fashion over the MCP and
interphalangeal joints .

29.  Mitral stenosis
 Pneumonia
 Pneumothorax
 Pulmonary embolism
 Sarcoidosis

30.  Hampton’s hump, seen here on the left side of the
chest in this radiograph, is a peripheral wedge-shaped
opacification abutting the pleura, signifying
pulmonary infarction distal to a pulmonary embolism.
The patient had a thrombus in the left main
pulmonary artery.
 The majority of pulmonary emboli do not result in
infarction of the distal lung, presumably because of
vascular collateralization.
 Infarction is most likely to occur in patients with large
pulmonary emboli or underlying lung disease

32.  Hampton’s hump(peripheral wedge shaped opacity)
 Westermark sign(peripheral oligemia)
 Knuckle sign or Palla’s sign (enlarged rt. Descending
pulmonary artery)
 Fleishner’s sign: elevated hemidiaphragm
 Melting Sign : infarct may show rapid clearing (melts
like snow) with treatment in contrast to pneumonic
consolidation

33.  represents a focus of oligemia
(vasoconstriction) seen distal to
a pulmonary embolism.
 The sign results from a
combination of:
 The dilation of the
pulmonary arteries proximal
to the embolus and
 the collapse of the distal
vasculature creating the
appearance of a sharp cut off
on chest radiography.

34.  V/Q scanning
 MDCT angiography : investigation of choice
 Pulmonary Angiography : Gold standard

36.  A 40 year old gardener presented with intensely itchy
erythematous, serpiginous skin eruptions as shown in
the figure. Diagnosis?

38.  Cutaneous larva migrans (CLM) is a skin disease in
humans, caused by the larvae of various nematode
parasites of the hookworm family(Ancylostomatidae).
 The most common species causing this disease is
Ancylostoma braziliense. These parasites live in the
intestines of dogs, cats and wild animals and should
not be confused with other members of the hookworm
family for which humans are definitive hosts, namely
Ancylostoma duodenale and Necator americanus.

39.  The filariform larvae can burrow through intact skin
that comes into contact with soil or sand that is
contaminated with feces.
 the larvae are only able to penetrate the upper dermis
of the skinand thus create the typical worm like
burrows visible underneath the skin.
 These parasites apparently lack the collagenase
enzyme required to penetrate through the basement
membrane deeper into the skin

40.  Oral agents include albendazoleand ivermectin.
 Another agent which can be applied either topically or taken by
mouth is thiabendazole.
 Topical freezing agents, such as ethylene chloride or liquid
nitrogen,applied locally can freeze and kill the larvae, but this
method has a high failure rate because the larvae are usually
located away from the site of the visible skin trails. Additionally,
this is a painful method which can cause blistering and/or
ulceration of the skin and it is therefore not recommended.
 Some local anti-itch products(i.eCalamine lotion).
 Wearing shoes in areas where these parasites are known to be
endemic offers protection from infection.In general, avoiding
exposure of skin to contaminated soil or sand offers the best
protection.

42.  What is the diagnosis for this patient with end-stage
renal disease who developed skin changes as follows
after an imaging procedure?

44.  Actinic elastosis
 Calciphylaxis
 Nephrogenic systemic fibrosis
 Porphyria cutanea tarda
 Uremic frost

45.  Nephrogenic systemic fibrosis (NSF) or
nephrogenic fibrosing dermopathy
(NFD)is a newly described rare and serious
systemic syndrome that involves fibrosis of
skin, joints, eyes, and internal organs in
patients with renal insufficiency after
exposure to gadolinium-containing
magnetic resonance contrast agents.

46.  There is thickening of the skin of the arm, most
prominent over the flexures of the wrists and
elbows and the area between the thenar and
hypothenar prominences.
 These are typical features of nephrogenic fibrosing
dermopathy
 NFD is characterized by diffuse, cutaneous,
hyperpigmented, brawny induration,
erythematous or skin-colored plaques with
irregular borders with ameboid projections, firm
discrete papules, and subcutaneous nodules.
 It appears to be associated with exposure to
gadolinium used to perform magnetic resonance
imaging.

47.  The functional consequences of nephrogenic
systemic fibrosis are often devastating and may be
fatal.
 Since nephrogenic systemic fibrosis develops after
exposure to gadolinium-containing magnetic
resonance contrast agents in some patients, the
FDA recommended avoiding such agents in
patients with renal insufficiency.
 The mechanism by which nephrogenic systemic
fibrosis develops after gadolinium exposure
remains unknown.