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clinical clues apparent on examination can expand or narrow the differential diagnosis substantially , even when the patient is seen first for management of a life - threatening condition in the intensive care unit ( icu ) .
we present a female patient with presumed chronic inflammatory demyelinating polyneuropathy ( cidp ) in whom recognition of hypertrichosis , clubbing and papilledema suggested poems ( polyradiculoneuropathy , organomegaly , endocrinopathy , m - protein and skin abnormalities ) syndrome .
a 35-year - old female patient was seen by the icu consult services for management of impending respiratory failure .
she had presented with cough , sputum , worsening limb weakness and orthopnea for one - week .
weakness was symmetrical in all four limbs and she was unable to move any of her limbs across the bed .
she was unable to roll to one side or lift her head off the pillow .
she had presented with insidious progressive weakness of all limbs 7 months prior to the current symptoms to another hospital .
cerebrospinal examination was acellular and showed raised proteins ( 1.3 g / dl ) , without any oligoclonal bands .
human immunodeficiency virus enzyme linked immunosorbent assay ( elisa ) , venereal disease research laboratory antibodies and antinuclear antibodies by elisa were negative .
she was initiated on 2 g / kg intravenous immunoglobulins monthly , along with 0.5 mg / kg steroids , 1.5 mg / kg azathioprine and osteoporosis prevention therapy .
weakness remained static till one - week prior to the time of her current presentation .
she had no previous history of smoking , alcohol or other drug abuse or animal exposure .
there was also no relevant family history . on examination , she was afebrile , normotensive , with a respiratory rate of 40 cycles / min and pulse rate of 106 beats / min .
there was evidence of accessory muscle use , with flaring of alae nasi and paradoxical movement of the diaphragm on inspection .
general examination also showed bilateral pitting pedal edema to the level of the knee , grade 2 pandigital clubbing , diffuse skin hyperpigmentation with mucosal sparing and hypertrichosis [ figure 1 ] .
neurological examination confirmed normal mental status and muscle weakness : power in both lower limbs was graded 1/5 and in the upper limbs was 2/5 , according to the medical research council ( mrc ) scale .
arterial blood gas showed respiratory academia due to acute respiratory acidosis ( ph 7.3 , pao280 mm hg on 4 l / min oxygen , paco250 mm hg , hco328 meq / l ) . computed tomography of the head was normal .
bilateral lower limb venous doppler did not show any evidence of venous thrombosis and quantitative d - dimers ( immunoturbidometry , lister metropolis ) were negative .
she was unable to complete a forced vital capacity maneuver or perform breath hold required for respiratory muscle testing .
sniff measurements and ultrasonography of diaphragmatic muscles were not performed given the obvious paradoxical movements clinically .
composite clinical photographs showing grade 3 clubbing ( left ) , with severe distal weakness and a correctible claw - hand deformity and hypertrichosis ( right ) summary of clinical investigations in the index patient she was initiated on parenteral piperacillin - tazobactam , azithromycin , enoxaparin , oxygen at 4 l / min ( approximate fio20.4 ) with bilevel noninvasive ventilation ( niv ) ( bipap 14/4 cmh2o , resmeds9 vpap auto ) .
plasmapheresis with 2 l exchanges using 5% albumin replacement was started on alternate days for possible worsening of cidp and was continued for six sessions .
the simultaneous features of clubbing , hypertrichosis and papilledema along with progression of weakness despite treatment prompted further evaluation .
radiographs of the skull , pelvis , and spine [ figure 2 ] showed osteosclerotic lesions over the right femur and spine .
ultrasound examination showed splenomegaly ; there was no evidence of hepatomegaly , enlarged nodes or ascites .
g / l ) with serum albumin levels of 2.43 g / dl ( normal 4 - 6 g / dl ) .
free lambda chain levels were elevated at 29.60 mg / l ( normal , 5.71 - 26.3 mg / dl ) with normal free kappa chain value of 11 mg / l ( normal 3.3 - 19.4 mg / l ) .
serum 2-microglobulin was 3550 ng / ml ( normal 670 - 2143 ng / ml ) .
bone marrow biopsy from the right iliac crest showed normocellular marrow , absence of lymphoid aggregates or plasma cell rimming and megakaryocyte hyperplasia .
composite radiographs of the pelvis and hips ( left ) showing a sclerotic lesion over the right femur and thoraco - lumbar spine ( right ) , showing another lesion over the lumbar spinous processes ( black arrows )
poems syndrome is a rare paraneoplastic syndrome because of an underlying plasma cell disorder ( pcd ) .
the pathogenesis of poems syndrome involves -chain producing monoclonal plasma cell clone from the sclerotic bony lesions .
vascular endothelial growth factor ( vegf ) levels are high but may not be the sole pathogenic mechanism , as anti - vegf therapy has shown inconsistent results .
the diagnosis of poems syndrome is made by the dispenzieri criteria [ table 2 ] .
the differential diagnosis includes vasculitis , connective tissue disease , monoclonal gammopathy of undetermined significance , smoldering multiple myeloma ( mm ) , mm or solitary plasmacytoma .
the composite clinical and laboratory findings [ table 1 ] differentiates poems syndrome from these other disorders . plasma vegf level of greater than 200 pg / ml has a specificity of 95% and sensitivity of 68% in support of diagnosis of poems syndrome . this entity can be distinguished from other pcds by the prominent neuropathy , minimal to absent renal disease , thrombocytosis and organomegaly .
diagnostic criteria for the poems * syndrome treatment is directed at abolishing the underlying plasma cell clone . in patients without diffuse marrow involvement and/or > 3 skeletal lesions , radiation to the isolated sclerotic lesions
half of nonresponders or progression occurs within 12 months of radiation . in those with disseminated bone marrow involvement , chemotherapy with melphalan - dexamethasone , lenalidomide , and autologous stem cell transplantation
thalidomide and bortezomib have activity in poems syndrome but have potential to worsen the neuropathy .
the index patient presented with findings of advanced peripheral and phrenic nerve neuropathy as a result of the delay of 18 months prior to diagnosis .
she was managed with local radiotherapy to proximal right femur and lumbosacral area ( 40 gy , 2 gy / cycle , total 20 cycles ) , thromboembolism prophylaxis , limb and respiratory rehabilitation , percutaneous gastrostomy feeds and nocturnal bilevel niv .
she improved objectively 6 weeks after completion of radiotherapy with upper limb power improving to 3/5 and lower limb power to 2/5 and absent paradoxical respiratory movements .
she remains on follow - up at 6 months with clinical assessment monitoring the weakness , sensory loss and pulmonary function testing by spirometry .
we present a female patient who was referred for management of respiratory failure . she was being evaluated and managed as worsening chronic inflammatory demyelinating polyneuropathy with type 2 respiratory failure . 
 initial examination showed hypertrichosis , clubbing and papilledema along with severe distal and proximal motor - predominant weakness with impending respiratory failure . 
 she was managed with noninvasive ventilation ( niv ) and plasmapheresis awaiting diagnostic investigations . 
 immunofixation showed an  m band  and free lambda chain levels were elevated . 
 radiographs showed the classic osteosclerotic lesions of poems ( polyradiculoneuropathy , organomegaly , endocrinopathy , m - protein and skin abnormalities ) syndrome . 
 six weeks after commencing radiotherapy to the osteosclerotic lesions , the patient responded favorably and remains off nocturnal niv support .