Oct 27 2019 Flashcards
Name and Associated condition
Dermatitis Herpetiformis
(found on extensor surfaces of elbows, knees, back and buttocks)
Intensely pruritic, erythematous papules, vesicles adn bullae.
Gluten-sensitive enteropathy (celiac disease)
What is Acanthosis nigracans and associated conditions?
Increased pigmentation especially in axilla
Insulin resistance and GI malignancy
What are multiple skin tags associated with?
Insulin resistance
Pregnancy
Chron disease (when found perianal)
Name and associated condition?
Porphyria cutanea tarda
Blistering lesions on skun exposed areas, can progress to hypertrichosis, hyperpigmentation and sclerodermatous thickening.
Associated with Hepatitis C
What skin conditions are associated with Hepatitis C
Porphyria cutanea tarda, Cutaneous leukocytoclastic vasculitis and Lichen planus
Name and associated condition?
Cutaneous Leukocytoclastic Vasculitis (Palpablue purpura)
Hepatitis C (Caused by secondary cryoglobulinemia)
Name and associated condition?
Molluscum contagiosum virus (benign single or multiple rounded, dome shaped, pink, waxy papules 2-5mm)
HIV
What skin conditions are associated with HIV?
Sudden-onset, severe psoriasis
Recurrent herpes zoster
Disseminated molluscum contagiosum
Severe seborrheic dermatitis
What skin conditions are associated with GI malignancy?
Explosive onset multiple, itchy sevorrheic keratoses
Acanthosis nigricans
Name and associated conditions?
Severe seborrhic dermatitis
HIV and Parkinsons
What is sudden onset of multiple, itchy seborrheic keratosis associated with?
GI malignancy
Name and associated condition?
Pyoderma gangrenosum
Inflammatory bowel disease
What causes anemia in celiac?
Iron deficiency
What will a biopsy of dermatitis herpetiformis show?
DIF of perilesional skin shows granular IgA deposits in ther dermal papillae
Patient with elevated MCHC, microcytosis anemia with spherocytes. Differential and w/u?
Most likely are AIHA or HS
DAT or Coombs can help differentiate (positive in AIHA)
eosin-5-maleimide binding (flow cytometry) or acidified glycerol lysis test can confirm HS.
How do you treat Hereditary Spherocytosis (HS)?
RBC transfusion for symptomatic anemia, folic acid supplements, splenectomy for severe cases
What are the common and less common causes of peripheral spherocytes?
AIHA, HS
Clostridial sepsis, snake bites, G6PD deficiency, MAHA
What are the indications for irradiated RBC transfusion?
BMT recipients
Acquired or congenital cellular immunodeficiency
Blood components donated by first or second degree relatives
What are indications for leukoreduced PRBC?
Chronically transfused patients
CMV seronegative at risk pt (AIDS, Transplant)
Potential transplant recipients
Previous febrile non-hemolytic transfusion reaction
What are indications for Washed PRBC?
IgA deficiency
Complement-dependent AIHA
Continued allergic reactions with PRBC despite anti-histamines
What is the most common form of Diffuse Parenchymal lung disease and its associated histopath?
IPF, Usual interstitial pneumonia (UIP)
Common age of onset of IPF?
50-70
HPI of IPF?
>6 month dry cough that disturbs daily activities
DOE
No history of potential cuases of fibrosis
~50% have a smoking history
Objective findings in IPF?
Velcro early inspiratory crackles with base predominance
~50% clubbing
Decreased lung volumes on CXR
Bibasilar septal line thickening with reticular changes and bronchiectasis when more severe
Honeycombing, cystic changes and traction bronchiectasis prominent in bases and subpleural areas on HRCT
May have s/s of Right CHF
What is required to diagnose IPF?
>6month cough
Imaging findings consistent with UIP
No known cause
Lung biopsy not necessary if imaging is consistent
Natural progression of IPF?
Progressive
Median survivial of 3-5 years post diagnosis
Stepwise decline, periods of stability with acute declines (may be triggered or idiopathic)
Treatment of IPF?
Manage comorbidities (obesity, CHF, deconditioning, OSA, Pulmonary HTN)
Supplemental oxygen
Pulmonary rehab for those with deconditioning
Nintedanib and pirfenidone
Lung transplant - early referral appropriate
If not a transplant candidate recommend against mechanical ventilation and referr to palliative
How do nintedanib and pirfenidone work? Common side effects?
Target fibroblasts (considered central in progression of IPF)
Not curative, delay progression
Diarrhea, elevated liver associated enzymes, increased risk of MI for Nintedanib
Diarrhea, nausea, headache, dyspepsia/GERD, anorexia, weight loss for pirfenidone
What did the trials show for nintedanib?
INPULSIS 1 trial (2014) -Decreased FVC decline (114 vs 239) did not reduce time to first exacerbation
INPULSIS 2 trial (2014) -Decreased FVC decline (113 vs 207) did not reduce time to first exacerbation
TOMORROW trial (2011) -reduced decline in FVC and acute exacerbations
What did the trail for pirfenidone show?
ASCEND trial (2014) - Reduced risk of progression by 30%, lower progression of FVC >10% 16.5% vs 31.8%.
SP3 trial (2010), CAPACITY 1 & 2 (2010) reduce the FVC decline and progression-free survival (no survival benefit).
Risk factors for AAA?
Risk factors for expansion and rupture?
Age >60
Male gender 6:1
HTN
Cigarettes
White
Atherosclerosis
FH of AAA
===================================
>5.5 cm
rate of expansion >= 0.5 cm every 6 mo or 1cm/yr
Active cigarette smoking (highest rate of AAA rupture)
Diagnosis of AAA?
AP diameter >3.0cm
75% asymptomatic
Screening guidelines for AAA?
Men 65-75 who have smoked at least 100 cigs
select screening in Men who have never smoked
Surveillance for AAA?
<=4cm every 2-3 years (rupture risk <0.5%/yr)
4.1-5.4cm 6-12 months (rupture risk 0.5-10%)
>5.5cm repair (rupture risk >10%) should have CTA or MRA for surgical planning.