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Dr. Ankur Banik PGT Dept Of General Medicine BMCH

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Dr. Ankur Banik


Dept Of General Medicine


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• Heberden’s nodes• Rheumatoid nodules• Jaccoud’s arthropathy• Pseudogout tophi• Erosive arthropathy of

rheumatoid arthritis

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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’sarthropathy includes physical therapy and the use of orthotic devices.

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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.

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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.


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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 oligoclonalbands 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.MGUSand 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.

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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

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The most recent criteria for the diagnosis of POEMS syndromerequire 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

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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.

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

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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'ssyndrome, lupus, scleroderma, or vasculitis.

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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.

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

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

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Drug used in the management of DM:




Intravenous immunoglobulin




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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 .

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Mitral stenosis



Pulmonary embolism


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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

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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

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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 proximalto the embolus and

the collapse of the distal vasculature creating the appearance of a sharp cut off on chest radiography.

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V/Q scanning

MDCT angiography : investigation of choice

Pulmonary Angiography : Gold standard

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

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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.

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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 skin and 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

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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.

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

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Actinic elastosis


Nephrogenic systemic fibrosis

Porphyria cutanea tarda

Uremic frost

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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.

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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 fibrosingdermopathy

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.

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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.

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