4.21.2006 

Shock lecture

This excellent lecture was given by Dr. Broccard today. General notes from Dr. Broccard, comments and explanations from me.

Shock Notes

Neil A. Shah

4/21/06

Lecture by Alain Broccard, M.D.

Definition: An inability to adequately satisfy the oxygen demands of the tissue.

  • Oxygen delivery is determined by three variables: Hb, O2 sat and cardiac output.
    • Hb – If it is acutely low you cannot carry oxygen to your tissues (RBCs)
    • O2 sat – if your hemoglobin is not wed to oxygen it has no oxygen to deliver (respiration)
    • Cardiac output – Stroke volume x Heart Rate
      • Stroke volume affected by 3 variables
        • Preload – what is put into the heart to pump out
          • Preload = venous return
          • Determined by 3 things:
            • Mean capillary pressure – main determinant. This is the pressure in your total vasculature if you were to stop the heart. It is determined by the amount of blood (total blood volume) and the volume of the venous compartment (is it a big or small tube). If you put the same volume in a small bag the pressure would be higher, if you put the same volume in a large bag the pressure would be lower.
            • Right atrial pressure – if you've got something pushing back against the venous side then you can't fill the RA very well. Venous resistance – usually negligible.
        • Afterload – the resistance the heart must overcome to push any blood forward
          • Afterload problems causing shock are rare but include PE (giant clot you're trying to push against – like a cork in your CO) and a big valvular clot.
        • Contractility – how hard the heart beats
      • CO is the amount of O2 leaving the heart NOT the amount making it to target organs
  • Tissue perfusion
    • Tissue perfusion is determined by two variables: the Oxygen delivery (as above) and the mean arterial pressure (is it getting to the end organs).
    • CO can be excellent with crappy perfusion
      • For example – if you made an aorto-caval fistula your heart would be making great CO, you'd be oxygenating your blood well but you wouldn't be perfusing your end organs.
    • Poor tissue perfusion is due to either inadequate oxygen delivery or decreased perfusion pressure. Put another way, you essentially address oxygen delivery with measures to increase CO, but if that doesn't increase tissue perfusion you probably need to add pressors to increase perfusion pressure.
  • Clinical assessment of shock
    • Definition:
      • Change in BP from baseline (20% or 40 mmHg drop)
      • Altered mental status
      • Decreased urine output
    • Types
      • Cardiogenic
      • Hypovolemic
      • Distributive
    • Clinical features

Type

Cap refill/ Pulse Pressure

Neck Veins / Lung crackles

Cardiogenic

Long / Weak

Inc. or NL

Hypovolemic

Long / Weak

Down / Neg

Distributive

Short/ Fine

Down / Neg

      • Since the brain and kidney are oxygen dependant and sensitive to changes in input they are very good for clinical assessment of tissue perfusion. Altered mental status and oliguria/anuria are good clinical markers of tissue hypoperfusion.
    • Swan-Ganz catheter data by shock type

Type

RAP

PCWP

CO

SVR

Mixed venous O2

Cardiogenic

Nl or Up

Nl or Up

Down

Up

Down

Hypovolemic

Down

Down

Down

Up

Down

Distributive

Down

Down

Nl or Inc.

Down

Nl or Up

      • Mixed venous O2
        • Defined as O2 in pulmonary arteries
        • Determined by two things:
          • Input oxygen to tissue
          • Extraction of oxygen by tissue
          • Normal value is ~70%
        • Why is it normal or increased in distributive shock?
          • In distributive shock the cells themselves are sick, usually from cytokine poisoning, and they are abnormally using oxygen.
          • There may also be shunting in the microcirculation leading to less O2 being used.
    • Lab values
      • Lactate can be a helpful indicator of tissue hypoperfusion especially a trend.
  • Treatment of shock
    • Five big things
      • Get access
      • Give fluid – the only contraindication would be LV failure where you risk increasing pulmonary edema.
      • Give oxygen
      • Consider intubation
        • Does the patient have difficulty guarding their airway due to AMS?
        • Also, is the work of breathing using oxygen that otherwise would be benefiting target tissues?
          • Normally you use ~5% of your CO to supply muscles driving respiration.
          • Severe tachypnea can lead to up to 50% of CO being wasted on the work of breathing.
          • Ventilation takes away the work of breathing from the CO demand thus leaving more for other organs.
      • Is the shock due to medical or surgical problems?
        • For example bleeding can be medical (pt with INR of 12) or surgical (pt with GSW that lac'd the femoral artery). Both will have hypovolemic shock from their hemorrhage but both will require different initial treatment.
    • Pressors
      • Don't get scared. Keep these simple things in mind:
        • Variables you can change: heart rate, preload, afterload, BP and contractility.
        • Giving volume (normal saline) will change preload.
        • Increasing blood pressure (MAP) will improve organ perfusion and this is done with pressors.

Pressor

Alpha (vasoconstriction)

Beta 1 (+ chronotropy (HR) and + inotropy (contractility)

Beta 2 (vasodilation)

Notes

___Dopamine

+

+

NONE

Most arrhythmogenic; metabolized to NE

___NorEpi

++

++

NONE

Almost never wrong to use this.

____Epi

++

+++

NONE

More arrhythmogenic than NE, but still good.

___Phenylephrine

++

NONE

NONE

Not helping heart. Not used alone.

___Dobutamine

NONE

+

+

Don't use alone.

        • If the chart doesn't work here is the order: Dopamine, norepinephrine, epinephrine, phenylephrine, dobutamine.
        • Notes: Dopamine - most arrhythmogenic, metabolized to NE; NE - almost never wrong to use this; Epi - more arrhythmogenic than NE, but still good
        • Phenylephrine
          • Often used with dobutamine
          • Can be used with epi/norepi to get more vasoconstriction so you can back off on the beta effects of these two meds.
        • Dobutamine
          • If pt has a fixed CO, like in a PE, you can kill the patient with this agent alone. Why? Well, they can only pump so much b/c there is a big occlusion that's decreasing the volume that the LV gets to see and pump to the body. If you dilate the vasculature you've just killed your MAP and now your end organs aren't going to see any of that small CO that your heart is making.
          • Very useful if:
            • You've increased volume.
            • You've increased BP
            • You're still not getting good end organ perfusion.
            • Here the B2 effects can cause vasodilation @ the target organs à better tissue perfusion.
  • Example clinical scenario – putting it all together
    • Septic patient comes in with BP 75/50 and a CVP of 3.
    • Shock type?
      • Distributive. This patient has big time dilation secondary to cytokines and they're third spacing all kinds of fluid. Their effective circulating volume, the volume available to carry oxygen and be pumped by the heart and go to end organs, is low.
    • Step 1
      • Give fluid. You dump in 4L of NS.
      • CVP increases to 12 (normal 8-12) and BP comes up to 85/50.
      • Pt still AMS and anuric.
    • Step 2
      • Try to increase MAP to increase end organ perfusion
      • Give norepi (tighten down the arteries which should increase the BP)
      • CVP to 10, MAP to 65 (better), mixed venous O2 50% (not bad)
      • Lactate still 3 (ULN = 2.1)
      • Check the HCT and Hb is 12 – no need to transfuse.
    • Step 3
      • You've given volume (inc. preload), the heart is working just fine in terms of contractility and heart rate and you have a fair MAP. Unfortunately you're still not perfusing end organs well. What can you do?
      • Give dobutamine. The vasodilation effects will help perfuse end organs and you're not dealing with a situation where the pt has a fixed CO.
      • Patient looks better.

4.17.2006 

Aneurysmal Subarachnoid Hemorrhage

This summary is of the Suarez et al review in NEJM Jan 26, 2006. It's great and I'd highly recommend reading it.

  • Subarachnoid Hemorrhage
    • Definition: Bleeding into space occupied by CSF, under the dura and on-top of the pia mater in the CNS.
    • Types
      • Traumatic
      • Non-traumatic
        • Aneurysmal (80%)
          • Poor prognosis
        • Non-aneurysmal (20%)
          • Pretty good prognosis
  • Aneurysmal subarachnoid hemorrhage (hereafter SAH)
    • Epidemiology
      • SAH is 2-5% of all new strokes
      • 20-30,000 people affected in US/year
      • Incidence increases with age
        • Mean age 55
      • Women > Men
      • Blacks > Whites
    • Prognosis
      • 46% will have long term cognitive defects
      • 1/3 of pts will require lifelong care
      • 51% case fatality rate
        • Most within first two weeks
        • 25% within first 24 hours
      • Larger aneurysms are at higher risk of popping
      • Posterior circulation aneurysms have a worse prognosis
      • Modifiable risk factors
        • Cigarette smoking
        • Hypertension
        • Cocaine use
        • ETOH
      • Genetic risk factors (connective-tissue d/o)
        • PCKD
        • Ehlers-Danlos
        • PXE
        • FMD
      • Factors associated with poor outcome
        • LOC
        • Age
        • Amount of blood or biventricular hemorrhage
        • Bob is 100 years old, obtunded and bleeding all into his dome-piece – Bob is in bad shape
    • Grading systems
      • World Federation of Neurological Surgeons
        • GCS + Clinical score
          • Gr 1 = GCS 15 + No motor deficit
          • Gr 2 = GCS 13-14 + No motor deficit
          • Gr 3 = GCS 13-14 + Motor deficit
          • Gr 4 = GCS 7-12 +/- Motor deficit
          • Gr 5 = GCS 3-6 +/- Motor deficit
      • Head CT grading scale
        • SAH + Intraventricular hemorrhage
          • Gr 0 = absent / absent
          • Gr 1 = minimal / absent
          • Gr 2 = minimal / present in both lat ventricles
          • Gr 3 = Thick (filling a cistern or fissure) / Absent
          • Gr 4 = Thick / present in both ventricles
    • Diagnosis
      • Clinical s/sx
        • Sudden onset of "worst headache of my life"
        • Nausea / vomiting
        • Neck pain
        • Photophobia
        • Loss of consciousness
        • May have sentinel headaches preceding the SAH by a few weeks
      • Physical exam
        • Retina – hemorrhages
        • Neck – meningismus
        • Neuro – decreased LOC, focal neuro signs
      • Common misdiagnoses
        • 50% of SAH are misdiagnosed initially (including my patient)
        • Common incorrect DX = migraine, tension h/a
      • Diagnositic workup
        • Head CT without contrast is first choice (thin cuts through base of brain to pickup small bleeds)
          • Good for DX the bleed but ALSO good for finding edema/signs of vasospasm (primary complication)
          • Sensitivity decreases with time
            • 100% @ 12 hours
            • 93% @ 24 hours
            • 50% @ 7 days
        • LP
          • LP should be performed if CT is negative/equivocal and you have a high pre-test probability
          • LP findings
            • Elevated opening pressure
            • Elevated red cell count not diminishing with tube #
        • Gold standard test – digital subtraction cerebral angiography (CT angio may be equivalent)
        • Obtain MRI @ 7-14 days
    • Treatment
      • Stabilize (airway, CV function)
      • Goals
        • Prevent rebleeding
        • Prevent vasospasm
      • Preventing rebleeding
        • Medical management
          • Maintain BP WNL with labetalol or nicardipine
            • After aneurysm is controlled HTN is OK
          • Treat hyperglycemia
          • Treat hyperthermia
          • Prophylax with pneumoboots against DVT
            • Add heparin after surgery
          • Treat pain with narcotics
          • Peripheral calcium channel blockers
            • Nimodipine 60mg q4h x 21 days
        • Surgical intervention
          • Key point – earlier is better
          • Clipping
            • This is superior in terms of preventing rebleeding
          • Coiling
            • Generally older patients are better off this way
      • Complications
        • Vasospasm
          • Complicates 46% of cases
          • Best predicted by amt of blood on initial CT
          • Diagnosis
            • Transcranial Doppler
              • Perform Qday or QOD
              • High blood flow rate
            • Serial CT scans
          • Treatment
            • Triple H therapy
              • Hypervolemia
              • Hypertension
              • Hyperviscocity (not really used)
        • Hydrocephalus
          • Complicates 20% of cases
          • Treatment
            • Ventriculostomy
        • Seizures
          • Occurs in 1/3 of patients
          • Untested as to whether prophylaxis is of benefit
            • Consider lorazepam with phenytoin load
          • 20% of sz are nonconvulsive
        • Medical complications
          • Pulmonary edema
          • Cardiac arrhythmias
          • E-lyte disturbances
            • SIADH
            • Cerebral salt wasting

4.16.2006 

How to read an EKG at 2:30am

How to read an EKG at 2:30am – by Neil Shah

  • Draw the rubric – I do this on every EKG I read. It should catch the major stuff that any MD should be looking for. There are weirder things that cardiologists can catch, but to me I just want to make sure my pt/crosscover won't crump right now.
    • Reg / Irreg
    • Rate
    • Rhythm
    • Intervals: PR, QRS, QT
    • Axis: RVH/LVH/Hemiblock
    • P waves: RAE/LAE
    • ST changes
    • T waves
    • Q waves
    • Other/Clinical correlate – Is pt on dig (Dali sign)? Chance of a PE (S1Q3T3)? Peaky T waves? Pacer spikes causing a long QRS?
  • It takes 5-10 minutes to methodically run through this when you first start out but after a few dozen EKGs you should be able to rip through one in 1-2 minutes.
  • Key take home point: narrow QRS means the impulse/irregularity is ATRIAL while wide QRS means the impulse/irregularity is VENTRICULAR.
  • Explanations
    • Regular v. irregular
      • Is there a P before every QRS and a QRS after every P?
        • Yes = Sinus rhythm à Calculate rate (300 / # big boxes between QRS complexes)
        • No = Why?
          • Irreg irreg - See rhythm section below
          • Reg irreg – See heart block section below
    • Rate
      • If regular you can pick any two beats, if irregular you'll need to calculate an "average" or count multiple QRS intervals and divide.
      • Bradycardia < 60 BPM
      • Tachycardia > 100 BPM
      • Normal = 60 – 100 BPM
    • Rhythm
      • Sinus rhythm – the electrical impulse originates in the pacemakers cells of the SA, goes to the AV node, waits, then moves through the Purkinje system. Repolarization (T wave) occurs normally.
      • Tachycardia – look at the QRS complexes
        • Narrow QRS – the signal originates from the atria therefore the problem is atrial
          • Regular narrow-complex tachycardias
            • Sinus tachycardia – Heart is working properly, just fast. P waves should be clearly visible before each QRS.
            • Paroxysmal superventricular tachycardia (PSVT) – P waves usually buried. ~140 BPM; try carotid massage which should restore NSR.
            • Atrial flutter (Aflut) – Sawtooth/sinusoidal baseline with regular number of P waves between QRS.
            • Paroxysmal atrial tachycardia (PAT) – Difficult to tell from PSVT – carotid massage won't terminate this one. Also, PR intervals are variable (variable conduction delay).
          • Irregularly irregular narrow-complex tachycardias
            • Atrial fibrillation (Afib) – Squiggly baseline with irreg. irreg. QRS. Ventricular response can be slow or fast. If fast it is termed Afib with rapid ventricular response (RVR).
            • Multifocal atrial tachycardia (MAT) – irreg. irreg. rhythm with 3 distinct P-wave morphologies; this is the fast version of Wandering Atrial Pacemaker (they're the same thing, one is just fast)
        • Wide QRS – the signal is from the ventricles (bad!)
          • Regular wide-complex tachycardias
            • Ventricular tachycardia (Vtach) – 3 or more PVCs in a row, or a regular, wide-complex tachycardia. In reality it is not regular, but it looks regular.
              • Uniform – all the beats looks the same. This is less bad.
              • Polymorphic – beats looks different. This is usually assoc. with ACS and therefore bad.
            • Accelerated idioventricular rhythm – Mentioned here, but it is NOT a tachycardia. No P-waves, rate is normal, all QRS are monomorphic and wide. Benign.
          • Irregular wide-complex tachycardias
            • Ventricular fibrillation (Vfib) – Often a degeneration from Vtach. This is a terminal rhythm – shock it immediately. The tracing just looks like a squiggly, low-amplitude mess (like afib minus the QRS complexes).
            • Torsades – Degeneration of long QT (usually the "hypo" set of electrolyte disturbances or drugs). Looks like Vtach except that the amplitudes increase and decrease in a spiraling pattern. This is treated differently that Vtach so distinguish it.
      • Bradycardia
        • Can be due to heart block or simply sinus bradycardia (think resting EKG of a marathon runner)
    • Intervals
      • PR
        • 0.12 < Normal < 0.2 (one big box)
        • Short - < 0.12
          • Wolff-Parkinson-White (WPW) – Look for a slurred upstroke "Delta wave" to the QRS. Also, QRS will be widened. WPW patients are predisposed to Afib and PSVT – both of which can be bad as the rhythm can degenerate into Vtab/Vfib.
          • Lown-Ganong-Levine (LGL) – Think WPW but without QRS widening. Same predisposition to arrhythmias.
        • Long – heart block
          • All PR intervals are uniformly long, no orphan P waves
            • 1st degree heart block – can be normal, no worries
          • P-waves w/out QRS (when you see a gap in the QRS complexes look for these)
            • Mobitz type I second degree block (Wenckebach) "short, long, longer, drop" – don't worry much
              • Esoteric note: You can't distinguish between Mobitz I and II if you've got 2:1 conduction.
            • Mobitz type II second degree block with fixed PR interval "regular, regular, drop, regular, regular, drop" – worry a bit
            • 3rd degree AV block – take another sheet of paper and mark off all the P waves on it. They should be regular and march along at their own pace. Now, do the same for the QRS complexes (which are wide) – they are also regular. The rates are completely different. Since the QRS is wide we know it's ventricular. Basically the atria and ventricles are not talking to each other and are beating at their own rates. This is uniformly bad. Vtach is a form of 3rd degree block.
      • QRS
        • Normal < 0.10 (2.5 small boxes)
        • Long – bundle branch block
          • RBBB – look for an RSR' ("bunny ears" or, more commonly, a "volcano") in V1/V2.
          • LBBB – look for a "blunt top," "bunny ears" or "volcano" in V5/V6. LAD may also be present
          • Esoteric note: You cannot diagnose ventricular hypertrophy in the ventricle that is blocked (i.e. LBBB means you can't call LVH)
      • QT
        • Normal < 0.40 (correct for rate – the faster the HR the faster the heart needs to repolarize and the shorter the QT gets; inversely slow HR means a longer QT. This is one number where the automated machine calculation is useful IMO)
        • Long – Most likely a drug toxicity or electrolyte abnormality. May be congenital. Can degenerate into Torsades.
    • Axis (the quick way)
      • Normal: Net QRS deflection is upright in I and AVF the axis is normal.
      • LAD: Net positive in I and negative in AVF
        • Left anterior hemiblock – Should r/o LVH first as these look identical (no ST changes, No QRS prolongation, no T changes)
        • Left ventricular hypertrophy (LVH) – Normally the precordial leads follow a net S to net R progression. This is b/c the normal heart has a left-axis secondary to the large left ventricle. Think of LVH as an accentuation of the normal S->R progression from V1 to V6. Strict criteria are: R in V5 > 26 OR R from V5/6 + S from V1/2 > 35 OR AVL > 13
      • RAD: Net negative in I and positive in AVF
        • Left posterior hemiblock – Should r/o RVH first. DX when there is RAD w/out other explanation.
        • Right ventricular hypertrophy (RVH) – Normally there is S to R progression in the precordial leads. In RVH this is distorted b/c the RV is putting out a larger signal than it should. Criteria: The R wave in V1 is > than the S wave (V1 is a net R wave – it should be a net S wave normally).
    • P waves – look at lead II
      • Normal: Less than 2.5mm deflection.
      • Right atrial enlargement (RAE) – PEAKY P-wave "RAE is REALLY tall" > 2.5mm.
      • Left atrial enlargement (LAE) – LONG P-wave "LAE is LONG" that is one box longer than it should be. There are V1 criteria, but just looking for funkiness in II to dx RAE/LAE is quicker.
    • ST changes (infarction)
      • Most people go straight here when looking at an EKG. That's fine, you can scan them first incase your patient is tombstoning, but don't forget to look at the rest of the EKG systematically. This becomes doubly important when there are no ST changes.
      • ST elevation
        • Criteria: Greater than one box of elevation of the ST segment in any lead. Differentiate this from J-point elevation (benign). In true ST elevation the S wave and T wave are slurred together unlike in J-point elevation where the S and T waves are distinct.
        • Grouping leads is helpful for localizing the infarct
          • II, III, AVF – Inferior
          • I, AVL, V5, V6 – Left lateral
          • V1, V2, V3, V4 – Anterior
        • "Tombstone sign" – lots of ST elevations that look like tombstones all over the EKG. Given this morbid name because your patient is having a massive MI right now.
      • ST depression
        • Usually a sign of ischemia, not infarction. These may occur during a stress-test for example (usually signaling the end of the test)
    • T waves
      • Normal – T waves are upright.
      • Inverted T waves – these can be normal or abnormal. Asymmetric inverted T-waves are more often pathologic. Flipped T waves can be found in patients with prior MI, angina/ischemia and infarction. I use them as a gauge of whether I should be looking for something else – "Hmm, pan-flipped T-waves in the precordial leads – could this be RV strain from a PE?"
    • Q waves
      • Normal – less than one box and can occur in the limb leads. NEVER COUNT Q WAVES IN AVR. Actually, you're probably best off not looking at AVR unless you're a cardiologist.
      • Abnormal – more than one box occurring anywhere on the EKG. Usually grouped (anterior leads, lateral leads…). Q waves indicate a prior MI but they take time to develop so you won't see new ones in an acute MI.

4.13.2006 

Cutaneous manifestations of internal disease

This summary started from a table in Habif 4th Ed (Clinical Dermatology). I've added links and comments out that should be helpful (most eMedicine articles have pictures)

  • Telangiectasias
  • Alopecia mucinosa
    • Malignancy
    • Notes: Patch of papules with follicular prominence. The scalp/face is typically boggy. Usually these pt's present with red, finely scaly patches evolving to plaques that are refractory to topical treatment. Repeat biopsies often necessary for early diagnosis. Scalp finding with skin lesions should raise high index of suspicion.
  • Amyloidosis
    • Skin findings
      • Smooth macroglossia
      • Shiny, waxy papules that are red/purple typically around the eyes, nasolabial folds and chin. Also may be found in intertriginous areas. Keyword: "pinch purpura" – minor trauma of the skin leads to purpura
    • Associated malignancy
    • Notes
      • Amyloid can be primary, secondary or malignancy associated. When it's found with multiple myeloma (these pt's have lytic bone lesions, typically lumbosacral, pee out Bence Jones protein – plasma cells on blood smear) it has the same distribution as primary amyloidosis
  • Acanthosis nigricans
    • Skin findings
      • Hyperpigmented, hyperkeratotic skin folds (typically the back of the neck and axillae)
    • Associations
      • Benign – insulin resistance (MOA is thought to be increased insulin acting on keratinocytes leading to hyperproliferation)
      • Malignant
    • Associated malignancies
    • Notes
      • Thought to be a paraneoplastic insulin-like secretion that causes the hyperproliferation of the keratinocytes. In 1/3 of pt's the skin manifestations will appear in advance of otherwise evident ca recurrence. Take home point – if a non-insulin resistant / non-obese / non-diabetic patient presents with acanthosis nigricans you need to do a thorough GI adenoca workup.
  • Acrokeratosis neoplastica (Bazex's Syndrome)
    • Skin findings:
    • Associated malignancies
    • Notes
      • There is a X-linked hereditary Bazex's Syndrome which should be distinguished from the malignancy associated acrokeratosis neoplastica. The skin changes precede other cancer manifestations. This is a zebra - ~150 cases in the literature.
  • Bloom's Syndrome
    • Skin findings
      • Malar erythema
    • Associated malignancies
      • These syndromic patients have 200+ times the risk of the general population to develop cancers. Most common ones are:
      • Acute leukemia
      • Gastric adenoca
      • Lymphoma
    • Notes
      • AR syndrome typically in Ashkenazi Jews. Syndromic looking kids: short, high pitched voice, small for age, history of multiple URIs, with skin findings +/- chelitis.
      • http://www.emedicine.com/derm/topic54.htm
  • Carcinoid syndrome
  • Cowden's Disease
    • Skin findings (present in 90+% of patients)
      • Multiple hamartomas affecting the face, typically around the mouth, or oral mucous membranes. May also present with acral keratoses (verrucous papules 1-5mm) on the dorsum of the hands/feet.
    • Associated malignancies
      • Breast carcinoma
      • Follicular thyroid carcinoma
    • Notes
      • These patients will develop cancer. They need a multi-specialist care team to handle the numerous issues that can develop with this syndrome. Have a very high index of suspicion if the skin findings are present in a macrocephalic or retarded patient.
      • http://www.emedicine.com/derm/topic86.htm
  • Dermatomyositis
    • Skin findings (40% present with only these)
      • Heliotrope rash – violaceous hue around eyes. Apparently it looks like a flower.
      • Gottron's papules – red polygonal papules covering the knuckles of the hands
      • Calcinoses – hard, whiting papules/tumors
      • Photodistributed erythema – shawl sign, dorsal hand erythema
      • Nailfold telangiectasias – you'll need a dermatoscope to see these
    • Associated malignancies
      • Breast
      • GI
      • GU
      • Ovarian
      • Lung
    • Notes
  • Erythema gyratum repens
    • Skin findings
      • Very distinct erythematous "wood-grain" rash on the torso that rapidly expands (~1cm a day). It looks like some kind of tribal tattoo.
    • Associated malignancies
      • Lung
      • Breast
      • GU/GI/Prostate
    • Notes
      • 80% of these patients have a malignancy. It is postulated that cutaneous presentation of tumor antigens creates the characteristic rash. You need to go looking for a malignancy as the overwhelming majority of these patients present with this rash prior (up to 5 years) to overt malignancy. Like acanthosis nigricans it can be associated with non-malignant conditions like CREST syndrome, lupus and TB.
      • http://www.emedicine.com/derm/topic134.htm
  • Gardner's Syndrome
    • Skin findings
      • Multiple epidermoid cysts (these may get secondarily infected)
      • Cutaneous osteomas
    • Associated malignancy
      • Adeno CA of the colon – 100% of pts will get malignant degeneration of polyps
    • Notes
      • This is an AD genetic disorder. Treat patients with prophylactic colectomy to prevent 100% rate of malignant degeneration. Be suspicious and get a scope if pt presents with multiple epidermoid cysts, FHx of colon CA.
      • http://www.emedicine.com/DERM/topic163.htm
  • Glucagonoma syndrome
    • Skin findings
      • Necrolytic migratory erythema (NME) – annular cyclical rash. Begins as erythematous patches typically in the lower abdomen/perineum, blisters, erodes, crusts and heals in 10 days.
    • Associated malignancy
      • Glucagon secreting alpha cell tumor of the pancreas
    • Notes
      • May be associated with severe hepatic cirrhosis that leads to a high level of glucagon in the blood (liver should break it down) or cystic fibrosis (high enteroglucagon produced by crypt cells in intestine d/t nutrient malabsorption).
      • http://www.emedicine.com/derm/topic168.htm
  • Hypertrichosis lanuginose acquisita
    • Skin findings
      • "malignant down" – final, fetal hair (lanugo) found on the face (typ. Forehead, ears) but can be found on the trunk.
    • Associated malignancies
      • Lung
      • Breast
      • GI/GU/lymphoma
    • Notes
  • Acquired ichthyosis
    • Skin findings
      • Generalized fish-like, polygonal scale that spare the flexural areas
    • Associated malignancies
      • Hodgkin's
      • Non-Hodgkin's lymphoma
      • Breast/GU
    • Notes
      • Distinguish from hereditary ichthyosis. This finding can be associated with non-malignant chronic conditions such as sarcoid, thyroid d/o and kidney failure; autoimmune (dermatomyositis, SLE).
      • http://www.emedicine.com/derm/topic678.htm
  • Leser-Trélat sign
    • Skin findings
      • Numerous eruptive seborrheic keratoses gradually increasing in size and number. These typically appear over 3-6 months.
    • Associated malignancies
      • Colon
      • Breast
    • Notes
      • Since SKs are common in the elderly it can be difficult to distinguish if these are eruptive. The SKs themselves are benign. Of the cutaneous findings associated with malignant disease this one has the weakest evidence. Some have questioned if this sign should even be associated with malignancies.
      • http://www.emedicine.com/derm/topic915.htm
  • Muir-Torre Syndrome
    • Skin findings
      • Sebaceous adenoma (most common) – yellow papules on the face (typically in a malar/chin distribution – think acne)
      • Sebaceous carcinoma – typically on the eyelid; tumor +/- ulceration.
      • Keratoacanthoma – rapidly growing red papule with a central keratotic plug and telangiectasias on the sides. May be confused with a BCC; also may harbor a SCC at the base.
    • Associated malignancies
      • Typically proximal colorectal cancers
      • Breast
      • Others
    • Notes
  • Paget's disease of the breast
    • Skin findings
      • Erythematous, scaly often indurated plaque on the nipple of the breast; may have serous/bloody weeping
    • Associated malignancy
    • Notes
      • Must address breast malignancy. One should scrape/bx the lesion to make the diagnosis as SCC in situ can look similar. BX/scrapings must show Paget cells.
  • Extramammary Paget's disease
    • Skin findings
      • Eroding, erythematous, crusty plaques in the anogenital region
    • Associated malignancies
      • 25% assoc. with adenoCA of rectum/anus.
    • Notes
      • Paget cells on bx. Since these rectal cancers can be slow growing, pt may present with extramammary Paget's disease a decade before malignancy would otherwise manifest. Sigmoidoscopy would likely be prudent in these pts.
  • Palmoplantar keratoderma (PPK)
  • Paraneoplastic pemphigus
    • Skin findings
      • Painful mucosal erosions +/- erosive stomatitis and blistering
    • Associated malignancies
      • Thymomas
      • Lymphoid malignancies
      • Sarcomas
    • Notes
  • Peutz-Jeghers Syndrome
    • Skin findings
      • Pigmented macules on the lips and oral mucosa.
    • Associated malignancies
      • AdenoCA of the stomach, duodenum and colon
    • Notes
  • Sipple's Syndrome (MEN IIB)
    • Skin findings
      • Multiple mucosal neuromas
      • Can't find picture
    • Associated malignancies
      • MEN IIB – pheochromocytoma and medullary thyroid CA
    • Notes
  • Sweet's Syndrome
    • Skin findings
      • Erythematous plaques/nodules that evolve vesicles/bullae/pustules. BX: Non-infectious neutrophilic dermatosis (no orgs). Pt's also have fever, elevated ESR and elevated WBC on CBC.
    • Associated malignancies
      • 20% of cases are assoc. with malignancy
      • Hematologic malignancies are most common (AML)
    • Notes
  • Urticaria pigmentosa (disseminated)
    • Skin findings
      • Pigmented macules/patches that become urticarial when rubbed (+ Darier's sign).
    • Associated malignancies
      • Hematologic malignancies
    • Notes
      • Majority of UP patients have no malignancy. These pts will all typically have elevated histamine levels. Frequently sx can be alleviated with oral antihistamines. Some pts may have systemic mastocytosis and thus may require more extensive treatment. All UP pts deserve a CBC +/- smear to look for thrombocytopenia (splenic sequestration?), anemia or leukocytosis. Other w/u may include skeletal bone survey and GI w/u depending on presenting s/x. If CBC anomalies are found pt may deserve a bone marrow bx.
      • http://www.emedicine.com/derm/topic258.htm
  • von Hippel-Lindau
    • Skin findings
      • Angiomas
        • Papules composed of dilated capillaries – typical cherry angioma
        • May also present with other types of angiomas
      • Also 50% will have retinal findings (tortuous vessels/angiomas)
    • Associated malignancies
      • Pheochromocytoma
      • Hypernephroma
    • Notes
      • VHL has a multitude of systemic issues. The finding with the most yield is the ocular exam as 50% of pts will have ocular findings. Pts may also have capillary malformations in their cerebellum, cysts in their kidneys and pancreas.
      • http://www.emedicine.com/PED/topic2417.htm
  • Von Recklinghausen's neurofibromatosis (NF-1)
    • Skin findings
      • Café au lait macules 6 or more in number > 1.5 cm
      • Neurofibromas – soft papules that display a "buttonhole" sign (pushing on them makes them go "into" the skin)
      • Lisch nodules in the iris (hamartomas)
    • Associated malignancies
      • Malignant neurilemoma
      • Astrocytoma
      • Pheochromocytoma
    • Notes
      • These pts usually present at a young age with parental concern over their "brown spots." If they have multiple pigmented macules that don't yet meet criteria they should be followed closely. Pts should have BPs and optho exams yearly in addition to general physicals. MRI imaging of the brain to look for astrocytomas/malignant neurilemomas should be undertaken.
      • http://www.emedicine.com/DERM/topic287.htm
  • Wiskott-Aldrich Syndrome
    • Skin findings
      • Eczematous rash in typical eczema distribution (flexural areas of extremities)
      • May have purpura related to thrombocytopenia
    • Associated malignancies
      • Lymphomas
      • Leukemias
    • Notes
      • This is an immune deficiency disease. These pts tend to succumb to infection, bleeding or cancer. Should be suspected in infants with ecchymoses, thrombocytopenia, repeated infections and eczematous rash.
      • http://www.emedicine.com/PED/topic2443.htm