Justin Shared "O Path Block 6" - 39 Picmonics

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O Path Block 6

Pemphigus Vulgaris
Pathophysiology
Autoantibodies against desmosomes
Symptoms
Flaccid Bullae on Skin
Oral Mucosa Involvement
Intraepidermal Acantholysis
Fluid Loss
Infection
Nikolsky Positive
Diagnosis
Biopsy showing Autoantibodies
Treatment
IVIG and Plasmapheresis
Oral Steroids
Immunosuppressants
2 mins
Bullous Pemphigoid
Linear Deposits of IgG
Affects Hemidesmosomes
Epidermal Basement Membrane
Tense Blisters on Skin
Nikolsky Negative
Contain Eosinophils
Rarely Affect Oral Mucosa
2 mins
Sturge Weber Syndrome
Pathophysiology
GNAQ Gene
Signs and Symptoms
Port Wine Stain
Arterial Venous Malformation
Leptomeningeal Angiomas
Intellectual Disability (Mental Retardation)
Seizures
Pheochromocytoma
Glaucoma
Tram-Track Calcifications
2 mins
Gardner Syndrome
Pathophysiology
Familial Adenomatous Polyposis (FAP) with Extraintestinal Benign Growths
Autosomal Dominant
APC Gene Mutation
Extraintestinal and Benign Manifestations
Desmoid Tumors
Nasal Angiofibromas
Osteomas
Congenital Hypertrophy of Retinal Pigment Epithelium (CHRPE)
Dental Abnormalities
Cutaneous Lesions
Adrenal Adenomas
Associated Malignancies
Thyroid Cancer
Duodenum/Periampullary Cancer
Pancreatic Cancer
Hepatoblastoma
Basal Cell Carcinoma of the Skin
Pathophysiology
Most Common Skin Cancer
2/3 of Cases Due to UV Light Exposure
Pink, Pearl-like Lesions
Rolled Borders
Telangiectasias
Commonly Upper Lip
Diagnosis
Shave Biopsy
Pallisading Nuclei
Treatment
Mohs Micrographic Surgery
2 mins
MEN 2B (Multiple Endocrine Neoplasia)
Pathophysiology
RET Gene
Autosomal Dominant
Signs and Symptoms
Medullary Thyroid Carcinoma
Pheochromocytoma
Marfanoid Body Habitus
Oral Ganglioneuromatosis
55 secs
Peutz-Jeghers Syndrome
Mechanism and Characteristics
Symptomatic Age: 10-30 Years
LKB1/STK11 Gene Mutation
Autosomal Dominant
Signs and Symptoms
Benign GI Hamartomatous Polyps
Mucocutaneous Hyperpigmentation
Positive Family History
Increased Risk of Colorectal Carcinoma
1 min
Neurofibromatosis Type 1
Pathophysiology
Autosomal Dominant
Chromosome 17
Signs and Symptoms
Neurofibromas
Optic Glioma
Lisch Nodules
Hamartomas
Cafe Au Lait Spots
Scoliosis
Pheochromocytoma
4 mins
Ehlers-Danlos Syndrome Types
Types
Types 1 and 2 - Classical
Type V Collagen Mutation
Joints and Skin
Type 3 - Hypermobility
Most Common
Tenascin X Deficiency
Joint Instability
Type 4 - Vascular
Type III Collagen Mutation
Vascular and Organ Rupture
2 mins
Plummer-Vinson Syndrome
Triad of Symptoms
Dysphagia
Esophageal Webs
Glossitis
Iron Deficiency Anemia
Association
Squamous Cell Cancer of Esophagus
1 min
Chronic Granulomatous Disease
Pathophysiology
Most Commonly X-linked Recessive
Defective NADPH Oxidase (Enzyme Complex)
Decreased Hydrogen Peroxide
Impaired Intracellular Killing in Phagocytes
Signs and Symptoms
Recurrent Purulent Skin and Lung Infections
Catalase Positive Organisms
Diagnosis
Negative Nitroblue Tetrazolium Test
DHR (Dihydrorhodamine) Flow Cytometry
Treatment
Antimicrobial Prophylaxis
TMP-SMX
Itraconazole
Interferon-Gamma
3 mins
HIV CD4+ 100
Signs and Symptoms
Aspergillus fumigatus
Cryptococcus neoformans
Candida albicans (Esophagitis)
Mycobacterium avium complex (MAC)
Cytomegalovirus (CMV)
Epstein-Barr virus (EBV) B-cell lymphoma
Toxoplasma gondii
Cryptosporidium
Bartonella henselae
5 mins
HIV CD4+ 200
Signs and Symptoms
Herpes Simplex Virus (HSV)
JC Virus
Progressive Multifocal Leukoencephalopathy (PML)
Pneumocystis Pneumonia
(HIV) Dementia
Histoplasmosis
3 mins
HIV CD4+ 500
Signs and Symptoms
Candida albicans (Oral Thrush)
Kaposi's Sarcoma (HHV-8)
Human Papillomavirus (HPV)
Epstein-Barr Virus (Oral Hairy Leukoplakia)
3 mins
Paget's Disease of Bone
Pathophysiology
Men > Age 40
Increased Osteoblast and Osteoclast activity
Mosaic Pattern
Signs and Symptoms
Increased Skull Diameter
Hearing Loss
Fractures
Bone Ache and Joint Pain
High Output Heart Failure
Diagnosis
Bone Scan
Normal Parathyroid hormone (PTH) and Calcium
Elevated ALP
Treatment
Bisphosphonate
Calcitonin
2 mins
Osteopetrosis (Marble Bone Disease)
Pathophysiology
Defective Osteoclast Resorption
Carbonic Anhydrase II Mutation
Lack of Acidic Environment
Symptoms
Fractures
Nerve Palsies
Diagnosis
Myelophthisic Anemia
Erlenmeyer Flask Bones
Normal ALP and PTH
Normal Calcium and Phosphorus
Bone-in-bone Appearance
Treatment
Bone Marrow Transplant
2 mins
Osteogenesis Imperfecta
Pathophysiology
Autosomal Dominant
Decreased Type I Collagen Production
Symptoms
Phenotypically Diverse
Brittle Bone Disease
Fractures from Minimal Trauma
Confused with Child Abuse
Hearing Loss
Dental Imperfections
Blue Sclera
1 min
Polycythemia Vera Disease
Pathophysiology
JAK2 Mutation leading to increased red blood cells
Tyrosine Kinase
Symptoms
Erythromelalgia
Pruritus
Often After Hot Shower
Headache
Treatment
Phlebotomy
Aspirin
1 min
Leukemia Assessment
Cause
Cause
Immature WBCs
Immature WBCs
Assessment
Assessment
Recurrent Infections
Recurrent Infections
Easy Bruising
Easy Bruising
Bleeding
Bleeding
Anemia
Anemia
Fatigue
Fatigue
Weight Loss
Weight Loss
Bone Pain
Bone Pain
Considerations
Considerations
May Have Normal or High WBC Count
May Have Normal or High WBC Count
2 mins
Hodgkin Lymphoma Pathophysiology
Pathophysiology
Reed-Sternberg Cells
Binucleate
Owl's Eyes Appearance
CD 15 and CD 30
B Cell Origin
Bimodal Age Distribution
2 mins

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