MUSCULOSKELETAL, SKIN, AND CONNECTIVE TISSUE- Pathology Flashcards
1
Q
Failure of longitudinal bone growth
A
Achondroplasia
2
Q
Which bone formation is affected in Achondroplasia?
A
Endochondral ossification
3
Q
Clinical presentation of Achondroplasia
A
Short limbs
4
Q
Memebranous ossification is affected in Achondroplasia
A
False, just endochondral ossification
5
Q
What is the result that mambranous ossification is not affected in Achondroplasia
A
Large head relative to limbs
6
Q
In Achondroplasia what inhibits chondrocyte proliferation?
A
Constitutive activation of fibroblast growth factor receptor (FGFR3)
7
Q
Which are the explanations of Achondroplasia?
A
> 85% of mutations occur sporadically and are associated with advanced paternal age, but the conditions also demonstrates autosomal dominant inheritance
8
Q
Common cause of dwarfism
A
Achondroplasia
9
Q
Do patients with Achondroplasia have normal life span and feritlity?
A
Yes
10
Q
What happens in Osteoporosis?
A
Trabecular (spongy) bone loses mass and interconnections despite normal bone mineralization and lab values (serum Ca2+ and PO4 3-)
11
Q
How is Osteoporosis diagnosis made?
A
By a bone mineral densitiy test (DEXA) with a T score of
12
Q
Pharmacologic cause of osteoporosis
A
By long term exogenous steroid use
13
Q
What can osteoporosis lead to?
A
Vertebral crush fractures
14
Q
Clinical findings of Vertebral crush fractures caused by osteoporosis
A
Acute back pain, loss of height, kyphosis
15
Q
Type I osteoporosis
A
Postmenopausal
16
Q
What explains Type I osteoporosis?
A
↑ bone resorption due to ↓ estrogen levels
17
Q
Distal radius fracture
A
Colles
18
Q
Common fractures of Type I osteoporosis
A
Femoral neck fracture, distal radius (Colles) fracture
19
Q
Type II osteoporosis
A
Senile Osteoporosis
20
Q
Who is mainly affected by senile osteoporosis?
A
Affects men and women > 70 years old
21
Q
Prophylaxis of Type II osteoporosis
A
Regular weight bearing exercise and adequate calcium and vitamin D intake throughout adulthood
22
Q
Treatment for Type II osteoporosis
A
Biphosphonates, PTH, SERMs (Selective Estrogen Receptor Modulators), rarely calcitonin; denosumab
23
Q
What is denosumab?
A
Monoclonal antibody against RANKL
24
Q
Marble bone disease
A
Osteopetrosis
25
What is Osteopetrosis?
Failure of normal bone resorption due to defective osteoclasts
26
Findings of Osteopetrosis
Thickened, dense bones that are prone to fracture
| Bone fills marrow space
27
What happens when bone fills marrow space in Osteopetrosis?
Pancytopenia, extramedullary hematopoiesis
28
What mutations could cause osteopetrosis?
Carbonic anhydrase II mutation
29
Cause of Osteopetrosis
Mutations impair of osteoclast to generate acidic envirmental necessary for bone resorption
30
What do X ray show in Osteopetrosis?
Bone in bone apperance
31
What could be a complication of Osteopetrosis?
Can result in cranial nerve impingment and palsies as a result of narrowed foramina
32
What is the treatment for Osteopetrosis?
Bone marrow transplant is potentially curative as osteoclasts are derived from monocytes
33
Disease caused by Vitamin D deficiency
Osteomalacia/ rickets
34
Vitamin D deficiency in adults
Osteomalacia
35
Vitamin D deficiency in children
Rickets
36
Pathophysiology of Osteomalacia/ rickets
↓ Vitamin D → ↓ serum calcium → ↑ PTH secretion → ↓ serum PO4 3-
37
What is the effect of Osteomalacia/ rickets
Due to defective mineralization/ calcification of osteoid → soft bones that bow out
38
How are osteoclast affected in Osteomalacia/ rickets?
Hyperactivity of osteoblasts → ↑ ALP (osteoblasts require alkaline enviroment)
39
Osteitis Deformans
Paget disease of bone
40
Explanation of Paget disease of bone
Caused by ↑ activity in both osteoblastic and osteoclatic activity
41
Characteristics of Paget disease of bone
Common, localized disorder of bone remodeling
42
How are Ca2+, phosphorus and PTH in Osteitis deformans?
Normal
43
Which lab is abnormal in Paget disease?
↑ ALP
44
Microscopic findings of Osteitis deformans
Mosaic pattern of woven and lamellar bone
45
Fractures seen in Paget disease
Long bone chlak stick fractures
46
Another characteristic of Paget disease of bone
↑ blood flow from ↑ arteriovenous shunts
47
What could be the result of ↑ blood flow from ↑ arteriovenous shunts in Osteitis deformas?
May cause high output heart failure
48
What risk is increased with Paget disease of bone?
Osteogenic sarcoma
49
X ray findings of Paget disease
Hat size can be increased
| Marked thickening of calvarium
50
Sensorial loss that could be seen in Paget disease
Hearing loss is common due to auditory foramen narrowing
51
Stages of Paget disease
Lytic
Mixed
Sclerotic
Quiescent
52
Who carries Lytic phase in Osteitis deformans?
Osteoclasts
53
Cells responsables of Mixed phase of Paget diease
Osteoclasts and Osteoblasts
54
This Osteitis deformans phase is carried by Osteoblasts alone
Sclerotic
55
How are cells affected in Quiescent phase of Paget diease?
Minimal osteoclast/ osteoblast activity
56
Alternative name for Avascular necrosis
Osteonecrosis
57
What happens in avascular necrosis?
Infarction of bone and marrow
58
Symptoms of avascular necrosis
Usually very painful
59
Causes of Avascular necrosis
Trauma, high dose corticosteroids, alcoholism, sickle cell
60
Most common site of Osteonecrosis
Femoral head
61
Why does avascular necrosis is common in femoral head?
Due to inssuficiency of medial circumflex artery
62
What is the main problem of osteoporosis?
↓ Bone mass
63
Characterized by dense, brittle bones
Osteopetrosis
64
When is Ca2+ ↓ in Osteopetrosis?
In severe, malignant disease
65
Which lab alteration is seen in Paget disease?
↑ ALP
66
Abnormal "mosaic" bone architecture
Paget disease
67
Lab characterisitics of Osteomalacia/ rickests
↓ Serum CA2+
↓ PO4 3-
↑ ALP
↑ PTH
68
How are the bones in Osteomalacia/ rickests
Soft bones
69
When you see Serum ↑ CA2+, ↑ PO4 3-, Normal ALP, ↓ PTH. you suspect of....
Hypervitaminosis D
70
Causes of Hypervitaminosis D
By over supplementation or granulomatous disease (sarcoidosis)
71
How is Osteitis Fibrosa Cystica classified?
1º Hyperparathyroidism
| 2º Hyperparathyroidism
72
"Brown tumors" of bone
Osteitis Fibrosa cystica
73
Why does Osteitis Fibrosa cystica present as "Brown tumors" of bone?
Due to fibrous replacement of bone, subperiostal thining
74
↑Serum CA2+
↓ PO4 3-
↑ ALP
↑ PTH
1º Hyperparathyroidism
75
↓ Serum CA2+
↑ PO4 3-
↑ ALP
↑ PTH
2º Hyperparathyroidism
76
Cause of 1º Hyperparathyroidism
Idiopathic or parathyroid hyperplasia, adenoma, carcinoma
77
When is 2º Hyperparathyroidism seen?
Often as compensation for End Stage Renal Disease (ESRD) (↓ PO4 3- excretion and production of activated vitamin D)
78
Why End Stage Renal Disease (ESRD) causes 2º Hyperparathyroidism?
↓ PO4 3- excretion and production of activated vitamin D
79
Primary Benign bone tumors
Giant cell tumor
| Osteochondroma
80
Age of apperance of Giant cell tumor of bones
20-40 years old
81
Where is commonly seen Giant cell tumor of bones?
Epiphyseal end of long bones
| Often around knee
82
How is Giant cell tumor of bones consider?
Locally agressive benign tumor
83
Apearance on X ray of "Soap bubble"
Giant cell tumor
84
Hystology of Giant cell tumor of bone
Multinucleated giant cells
85
Bening bone tumor that causes Exostosis
Osteochondroma
86
What is exostosis?
Is the formation of new bone on the surface of a bone, because of excess calcium forming
87
Characteristic of Osteochondroma
Mature bone with cartilaginous cap
88
How often Osteochondroma progresses to Chondrosarcoma?
Rarely
89
Primary Malignant tumors of Bone
Osteosarcoma
Ewing sarcoma
Chondrosarcoma
90
Second most common primary malingnant bone tumor
Osteosarcoma
91
Most common primary malingnant bone tumor
Multiple myeloma
92
Alternative name for Osteosarcoma
Osteogenic Sarcoma
93
Predisposing factors to Osteosarcoma
Paget disease of bone, bone infarcts, radiation, familial retinoblastoma, Li Fraumeni syndrome
94
Which form of Li Fraumeni syndrome predisposes to Osteosarcoma?
Germline P53 mutation
95
Where is the common site of apperance of Osteosarcoma?
Metaphysis of long bones, often around knee
96
Codman triangle is seen on X ray on this pathology of bone
Osteosarcoma
97
How is Codman triangle formed?
From elevation of periostum
98
Which X ray pattern can Osteosarcoma present?
Codman triangle or sunburst pattern
99
How is Osteosarcoma consider?
Aggressive
100
How is Osteosarcoma treated?
With surgical en bloc resection (with limb salvage) and chemotherapy
101
How are lesion seen on X ray in Osteosarcoma?
Lucent lesion
102
What is seen in Osteosarcoma on MRI?
Heterogeneous mass with periosteal elevation
103
Who commonly present Ewing sarcoma?
Boys
104
Where does Ewing sarcoma commonly appear?
In diaphysis of long bones, pelvis, scapula, and ribs
105
Hystology of Ewing sarcoma
Anaplastic small blue cell malignant tumor
106
How is Ewing sarcoma consider?
Extremely aggressive with early metastases
107
Which is the good point of Ewing sarcoma?
Responsive to chemotherapy
108
How is the Ewing sarcoma apperance on bone?
"Onion Skin"
109
Which gene translocation is associated to Ewing sarcoma?
t (11;22) translocation
110
How common is Chondrosarcoma seen?
Rare
111
How is Chondrosarcoma consider?
Malignant, cartilaginous tumor
112
Who are at higher risk to present Chondrosarcoma?
Men 30-60 years old
113
Where is Chondrosarcoma often located?
Pelvis, spine, scapula, humerus, tibia, or femur
114
How can Chondrosarcoma appears?
May be of primary origin or from osteochondroma
115
Evolution of Chondrosarcoma
Expansile glistening mass within the medullary cavity
116
Etiology of Osteoarthritis
Mechanical-joint wear and tear destroys articular cartilage
117
Joint findings of Osteoarthritis
Subchondral cysts, sclerosis, osteophytes (bone spurs), eburnation, Herbenden nodes, and Bouchard nodes
118
What are Herbenden nodes?
Are hard or bony swellings that can develop in the distal interphalangeal joints (DIP)
119
What are Bouchard nodes?
Bouchard's nodes are hard, bony outgrowths or gelatinous cysts on the proximal interphalangeal joints
120
Does Osteoarthritis presents with metacarpophalangeal (MCP) joints involvement?
No
121
Predisposing factors for Osteoarthritis
Age, obesity, joint deformity, trauma
122
What is Eburnation?
A degenerative process of bone commonly found in patients with osteoarthritis or non-union of fractures
123
Clinical presentation of Osteoarthritis
Pain in weight bearing joints after use (eg at the end of the day), improving with rest
124
How does Knee cartilage loss begins in Osteoarthritis?
Medially ("bow legged") Noninflammatory
125
Which systemic symptoms are found in Osteoarthritis?
None
126
Treatment for Osteoarthritis
NSAIDs, intra-articular glucocorticoids
127
Etiology of Rheumatoid arthritis
Autoimmune- inflammatory destruction of synovial joints
128
Who mediates inflammatory destruction in Rheumatoid arthritis?
By cytokines and type III and type IV hypersensitivity reactions
129
Joint findings of Rheumatoid arthritis
Pannus formation in joints (MCP, PIP), subcutaneous rheumatoid nodules (fibrinoid necrosis), ulnar deviation of fingers, subluxation, Bakers cyst (in poploteal fossa)
130
Which joint are not included in Rheumatoid arthritis?
No DIP involvement (Distal interphalangeal joint)
131
Predisposing factors for Rheumatoid arthritis
Females > males
132
How many patients of Rheumatoid arthritis present with + rheumatoid factor?
80%
133
Antibodies of Rheumatoid factor
Anti- IgG antibody
134
Which is the most specific marker of Rheumatoid arthritis?
Anti-cyclic citrullinated peptide antibody
135
Which factor is very associated to Rheumatoid arthritis?
HLA-DR4
136
Classical presentation of Rheumatoid arthritis
Morning Stiffness lasting > 30 minutes and improving with use, symmetric joint involvement, systemic symptoms
137
Systemic symptoms of Rheumatoid arthritis
Fever, fatigue, pleuritis, pericarditis
138
Treatment for Rheumatoid arthritis
NSAIDs, glucocorticoids, disease modifying agents
139
Examples of disease modifying agents when treating Rheumatoid arthritis
Methotrexate, sulfasalazine, TNF- α inhibitors
140
Characteristics of a Rheumatoid arthritis joint damage
Bone and cartilage erosion
Increased synovial fluid
Pannus formation
141
Characteristics of a Osteoarthritis joint damage
```
Thickened capsule
Slight synovial hypertrophy
Osteophyte
Ulcerated cartilage
Sclerotic bone
Joint space narrowing
Subchondral bone cyst
```
142
Findings on X ray of the knee on Osteoarthritis
Joint space narrowing and sclerosis
143
Possible hand manifestation of Rheumatoid arthritis
Boutonniere deformitis of PIP joints with ulnar deviation
144
Autoimmune disorder characterized by destruction of exocrine glands
Sjogren syndrome
145
Which glands are mainly affected in Sjogren syndrome?
Exocrine (especially lacrimal and salivary)
146
Who are mainly affected by Sjogren syndrome?
Females 40-60 years old
147
Findings of Sjogren syndrome
Xerophthalmia
Xerostomia (Decrease Salivary production)
Bilateral Parotid enlargement
148
Which antibodies are present in Sjogren syndrome?
Antinuclear antibodies: SS-A (anti-RO and/or SS-B (anti-La)
149
How can Sjogren syndrome be acquired?
Can be a primary disorder or a secondary syndrome associated with other autoimmune disorders (eg. rheumatoid arthritis)
150
Complications of Sjogren syndrome
Dental carie, mucosa-associated lymphoid tissue (MALT) lymphoma
151
How can a MALT lymphoma be pressented in Sjogren syndrome?
May present as unilateral parotid enlargement
152
Explain Xerophthalmia in Sjogren syndrome... which is the risk?
Decreased tear production and subsequent corneal damage
153
What is gout?
Acute inflammatory monoarthritis caused by precipitation of monosodium urate crystals in joints
154
What is associated to gout?
Hyperuricemia
155
What causes hyperuricemia in gout?
Underexcretion of uric acid
| Overproduction of uric acid
156
Percentage of undersecretion of uric acid causing hyperuricemia in gout
90% of patients
157
Percentage of Overproduction of uric acid causing hyperuricemia in gout
10% of patients
158
Causes of undersecretion of uric acid causing hyperuricemia in gout
Largely idiopathic
159
What can excacerbates undersecretion of uric acid causing hyperuricemia in gout?
By certain medications (eg. thiazide diuretics)
160
Causes of Overproduction of uric acid causing hyperuricemia in gout
Lesch Nyhan syndrome, Phosphoribosyl pyrophosphate (PRPP) excess, ↑ cell turnover, (eg. tumor lysis syndrome), von Gierke disease
161
Characteristics if crystals in Gout
Crystals are needle shapped and - birefrigent
162
What does - birefrigent crystals means?
Yellow under parallel light, blue under perpendicular light
163
In whom is it more common gout?
In males
164
Symptoms of gout
Asymmetric joint distribution. Joint is swollen, red and painful
165
Classic manifestation of Gout
Is painful Metatarsophalangeal Joint of the big toe (podagra)
166
What is tophus?
Localized deposition of crystalline monosodium urate monohydrate in peripheral tissues
Are pathognomonic for the disease gout
167
Where are Tophus commonly found in Gout paitents?
Often on external ear, olecranon bursa, or Achiles tendon
168
When can an acute attack of gout tends to occur?
After a large meal or alcohol consuption
169
Which is the explanation of Alcohol causing gout?
Alcohol metabolites compete for same excretion sites in kidneys as uric acid, causing ↓ uric acid secretion and subsequent buildup in blood
170
Treatment for Acute Gout
NSAIDs (eg. indomethacin), glucocorticoids, colchicine
171
For Chronic gout, what helps to prevent it?
Xanthine oxidase inhibitors
172
Example of Xanthine oxidase inhibitors for chronic gout
eg. Allupirinol, Febuxostat
173
How are tophi seen in microscope in Gout disease?
Aggregates of urate cystals surrounded by inflammation
174
How is Pseudogout presented?
With pain and effusion in a joint
175
What causes Pseudogout?
By deposition of calcium pyrophosphate crystals within the joint space
176
What is seen in Pseudogout on X ray?
Chondrocalcinosis
177
Characteristics of Crystals seen in Pseudogout
Forms Basophilic, rhomboid crystals that are weakly positively birefringent
178
Which joints are mainly affected in Pseudogout?
Large joints (classically the knee)
179
Who are mainly affected by pseudogout?
>50 years old; both sexes affected equally
180
Diseases associted to Pseudogout
Hemochromatosis, hyperparathyroidism and hypoparathyroidism
181
Treatment for sudden Gout
NSAIDs
182
In case of Severe attacks of Gout what is recommended?
Steroids and Colchicine
183
Comparing Gout and Pseudogour, what is the difference between their crystals?
Gout- Crystals are yellow when parallel to the light
| Pseudogout- Crystals are blue when parallel to the light
184
Common agents that cause Infectious arthritis
S. aureus, Streptococcus and Neisseria gonorrhoeae
185
Charactetristics of Gonococal arthritis
Is a STD that presents as migratory arthritis with asymmetric pattern
186
How is the joint affected by Infectious arhtritis?
Swollen, red, and painful
187
Main Findings of Infectious arthritis
STD
Synovitis (eg. knee)
Tenosynovitis (eg. Hand)
Dermatitis (eg Pustules)
188
Arhritis without rheumatoid factor (no anti IgG antibody)
Seronegative spondyloarthropathies
189
Seronegative spondyloarthropathies
```
PAIR
Psoriatic arthritis
Ankylosing spondylitis
Inflammatory bowel disease
Reactive arthritis
```
190
Which factor is strong associated to Seronegative spondyloarthropathies?
HLA-B27 (gene that codes for HLA MHC class I)
191
In whom does Seronegative spondyloarthropathies occurs more often?
In males
192
Joint pain and stiffness associated with psoriasis
Psoriatic arthritis
193
Characterisitics of Psoriatic arthritis
Asymmetric and patchy involvement
194
Possible characteristic of fingers in Psoriatic arthritis
Dactylitis
195
What is Dactylitis?
"sausage fingers"
196
What is seen on X ray in Dactylitis in Psoriatic arthritis?
"pencil in dup" deformity in x ray
197
How many patients with Psoriatic arthritis present Dactylitis?
Seen in fewer than 1/3 with psoriasis
198
Chronic Inflammatory disease of spine and sacroiliac joints
Ankylosing spondylitis
199
Reuslts of Chronic Inflammatory disease of spine and sacroiliac joints
Ankylosis (stiff spine due to fusion of joints), uveitis, and aortic regurgitation
200
X ray characteristic seen in Ankylosing spondylitis
Bamboo spine (Vertebral fusion)
201
Inflamatory bowel diseases
Crohn disease and Ulcerative colitis
202
Which seronegative disease is also associated to Inflammatory bowel disease?
Ankylosing spondylitis or peripheral arthritis
203
Alternative name for Reactive Arthritis
Reiter Syndrome
204
Classic Triad of Reiter Syndrome
Conjuntivitis and anterior uveitis
Urethritis
Arthritis
205
What do patients with Reactive Arthritis say?
"Can't see, can't pee, can't bend my knee"
206
Causes of Reactive Arthritis or Reiter Syndrome
Post GI (Shigella, Salmonella, Yersinia, Campylobacter) or Chlamydia infections
207
Classic presentation: Rash, joint pain, and fever
Systemic lupus erythematous
208
Classic presentation of Systemic lupus erythematous
Rash, joint pain, and fever
209
Who suffer more often Systemic lupus erythematous?
In a female of reproductive age and African descent
210
Wart-like vegetations on both sides of valve in Systemic lupus erythematous
Libman Sacks endocarditis
211
What are Libman Sacks endocarditis?
Wart-like vegetations on both sides of valve in Systemic lupus erythematous
212
Kidney finding in Systemic lupus erythematous
Lupus nephritis
213
Which type of hypersensitivity reaction is Lupus nephritis?
Type III hypersensitivity reaction
214
Types of Lupus nephritis
Nephritic
| Nephrotic
215
Characteristic of nephritic Lupus nephritis
Diffuse proliferative glomerulonephritis
216
Characteristic of nephrotic Lupus nephritis
Membranous glomerulonephritis
217
Main characteristics of Systemic Lupus erythematosus
```
RASH OR PAIN
Rash
Arthritis
Soft tissues/serositis
Hemathologic disorders (cytopenias)
Oral/ nasopharyngeal ulcers
Renal disease, Raynaud phenomenom
Photosensitivity / Positive VDRL/RPR
Antinuclear antibodies
Immunosuppressants
Neurologic disorders (eg. seizures, psychosis)
```
218
Characteristics of Rash in Systemic Lupus erythematosus
Malar or discoid
219
Which lab tests are postive in Systemic Lupus erythematosus?
VDRL/ RPR
| Antinuclear antibodies
220
Common causes of death in Systemic Lupus erythematosus
Cardiovascular disease
Infections
Renal disease
221
Which antibodies may be positive for Systemic Lupus erythematosus?
```
Antinuclear antibodies (ANA)
Anti-dsDNA
Anti-Smith
Antihistone
Anticardiolipin
```
222
How are ANA consider for Systemic Lupus erythematosus?
Sensitive, not specific
223
If Anti- dsDNA are positive in SLE, how does it translates?
Specific, poor prognosis (renal disease)
224
How are Anti Smith antibodies are consider for SLE?
Specific, not prognostic
225
To whom are Anti Smith antibodies directed to?
Against snRNPs
226
When are Antihistones antibodies present in SLE?
Sensitive for Drug indiced lupus
227
When are Anticardiolipin antibodies false positive when thinking of SLE?
On test for syphilis, prolonged PTT (paradoxycally, increased risk of arteriovenous thromboembolism)
228
What is affected in SLE by immune complex formation?
↓ C3, C4 and CH50
229
Why are C3, C4 and CH50 ↓ in SLE?
Due to immune complex formation
230
Treatment for SLE
NSAIDs, steroids, immunosupressants, hydroxychloroquine
231
Characterized by immune mediated, widespread noncaseating granulomas and Elevated ACE levels
Sarcoidosis
232
What is Sarcoidosis?
Characterized by immune mediated, widespread noncaseating granulomas and Elevated ACE levels
233
In whom is Sarcoidosis more common?
In black females
234
Clinical findings of Sarcoidosis
Often asymptomatic except for enlarged lymph nodes
235
On incidental CXR you find bilateral hilar adenophaty and/or reticular reticular opacities, and the patient is asymptommatic, what do you suspect of?
Sarcoidosis
236
What are the CXR possible findings on Sarcoidosis?
Bilateral hilar adenophaty and/or reticular reticular opacities
237
What is associated to Sarcoidosis?
With restrictive Lung disease (interstitial fibrosis), erythema nodosum, lupus pernio, Bell palsy, uveitis
238
Possible Microscopic findings in Sarcoidosis
Epithelioid granulomas containing microscopic Schaumann and asteroid bodies
239
Lab findings in Sarcoidosis
Hypercalcemia
240
Why is Hypercalcemia present in Sarcoidosis?
Due to ↑ α hydroxylase- mediated vitamin D activation in macrophages
241
Treatment for Sarcoidosis
Steroids
242
Which are the clinical findings of Polymyalgia rheumatica?
Pain and stiffness in shoulders and hips, often with fever, malaise and weight loss
243
How is the muscular weakness in Polymyalgia rheumatica?
Does not cause muscular weakness
244
In whom is it more common seen Polymyalgia rheumatica?
In women > 50 years old
245
What patholgy is associated to Polymyalgia rheumatica?
Temporal (giant cell) arteritis
246
Whcih labs may be present in Polymyalgia rheumatica?
↑ ESR, ↑ C- reactive protein, normal CK
247
Which is the treatment for Polymyalgia rheumatica?
Rapid response to low dose corticosteroids
248
In which patients is more often seen Fibromyalgia?
Most commonly seen in females 20-50 years old
249
Chronic, widespread musculoskeletal pain associated with stiffness, paresthesias, poor selep and fatigue
Fibromyalgia
250
Main findings in Fibromyalgia
Chronic, widespread musculoskeletal pain associated with stiffness, paresthesias, poor selep and fatigue
251
Which is the treatment for Fibromyalgia?
Treat with regular exercise, antidepressants (TCAs, SNRIs), and anticonvulsants
252
Progressive symmetric proximal muscle weakness, characterized by endomysial inflammation
Polymyositis
253
What is Polymyositis?
Progressive symmetric proximal muscle weakness, characterized by endomysial inflammation
254
What kind of inflammation is present in Polymyositis?
Endomysial inflammation with CD8+ Tcells
255
What is most often involved in Polymyositis?
Shoulders
256
Clinical findings of Dermatomyositis
Similar to polymyositis, but also involves malar rash, Gottron papules, heliotrope rash, "shawl and face" rash, "mechanics hands"
257
What is heliotrope rash?
Erythematous periorbital
258
What risk is increased in patients with Dermatomyositis?
Occult malignancy
259
Type of inflammation seen in Dermatomyositis
Perimysial inflammation and atrophy with CD4+ T cells
260
LAb findings on Polymyositis and Dermatomyositis
↑ CK, + ANA, + anti Jo- 1, + anti SRP, + anti-Mi antibodies
261
Treatment for Polymyositis and Dermatomyositis
Steroids
262
Neuromuscular junction disease
Myasthenia gravis
| Lambert Eaton myasthenic syndrome
263
Most common Neuromuscular junction disorder
Myasthenia gravis
264
Uncommon Neuromuscular junction
Lambert Eaton myasthenic syndrome
265
Which kind of antibodies are present in Myasthenia gravis?
Autoantibodies to postsynaptic ACh receptor
266
Which kind of antibodies are present in Lambert Eaton myasthenic syndrome?
Autoantibodies to presynaptic Ca2+ channel → ↓ ACh release
267
This disease is manifested with Ptosis, diplopia and weakness
Myasthenia gravis
268
How do symptoms worsen in Myasthenia gravis?
With muscle use
269
Clinical symptoms on Myasthenia gravis
Ptosis, diplopia, and weakness
270
Pathologies associated to Myasthenia gravis
Thymoma, thymic hyperplasia
271
Which is the treatment for Myasthenia gravis?
AChE inhibitor
272
What is the benefit of AChE inhibitors drug for Myasthenia gravis?
Reversal of symptoms
273
Presented with proximal muscle weakness, autonomic symptoms (dry mouth, impotence)
Lambert Eaton myasthenic syndrome
274
How do symptoms improve in Lambert Eaton myasthenic syndrome?
Improve with muscle use
275
Clinical presentation of Lambert Eaton myasthenic syndrome
Presented with proximal muscle weakness, autonomic symptoms (dry mouth, impotence)
276
How is the benefit of AChE inhibitors when used for Lambert Eaton myasthenic syndrome?
Minimal effect
277
Pathology associated to Lambert Eaton myasthenic syndrome
Small cell lung cancer
278
Metaplasia of skeletal muscle to bone following muscular trauma
Myositis ossificans
279
What is Myositis ossificans?
Metaplasia of skeletal muscle to bone following muscular trauma
280
Where is Myositis ossificans more often seen?
In upper or lower extremity
281
How is Myositis ossificans may be presented on radiography?
May present as suspicious "mass" at site of known trauma or as incidental finding on radiography
282
Excessive fibrosis and collagen deposition throughout the body
Scleroderma
283
What is Scleroderma?
Systemic sclerosis
284
Dermatologic findings in Scleroderma
Sclerosis of skin, manifestating as puffy and taunt skin with absence of wrinkles
285
Most common cause of death in Scleroderma
Sclerosis of Pulmonary system
286
Other possible findings of Scleroderma
Sclerosis of renal, pulmonary, cardiovascular and GI system
287
Who are mainly affected by Scleroderma?
75% female
288
2 major types of Scleroderma
Diffuse scleroderma
| Limited scleroderma
289
Characteristics of Diffuse scleroderma
Widespread skin involvement, rapid proggression, early visceral involvement
290
Which antibodies may be present in Diffuse scleroderma?
Anti Scl-70 antibody
291
What kind of antibody are Anti Scl-70 antibody?
Anti-DNA topoisomerase I antibody
292
What is the limited scleroderma?
Limited skin involvement confined to fingers and face
293
Which Scleroderma is related to CREST syndrome?
Limited scleroderma
294
What is CREST syndrome?
```
Calcinosis
Raynaud phenomenom
Esophageal dysmotility
Scletodactyly
Telangiectasia
```
295
Which antibodies are associated to CREST syndrome?
antiCentromere antibody
296
Dermatologic lesions
```
Macule
Patch
Papule
Plaque
Vesicle
Bulla
Pustule
Wheal
Scale
Crust
```
297
Flat lesion with well-circumscribed change in skin color
Macule
298
Macule >1 cm
Patch
299
What is a papule?
Elevated solid skin lesion
300
Papule >1cm
Plaque
301
Small fluid containing blister
Vesicle
302
What is Bulla?
Large fluid containing blister > 1 cm
303
Vesicle containing pus
Pustula
304
What is wheal?
Transient smooth papule or plaque
305
Flaking off of stratum corneum
Scale
306
What is Crust?
Dry exudate
307
When are macule seen?
Frecle, labial macule
308
When is Patch seen?
Large birhtmark (congenital nevus)
309
Skin lesion acne
Papule
310
Types of diseases presented with papule
Mole (nevus), acne
311
Type of skin lesion of Psoriasis
Plaque
312
When are vesicles seen?
Chicken pox (Varicella), shingles (Zoster)
313
When is Bulla seen?
Bullous pemphigoid
314
Pathology associtated to Pustule
Pustular psoriasis
315
Skin lesion of Hives (urticaria)
Wheal
316
Patholgies with Scale
Eczema, psoriasis, Squamous Cell Carcinoma
317
Skin lesion associated to Impetigo
Crust
318
What is Hyperkeratosis?
↑ thickness of stratum corneum
319
Examples of situations with Hyperkeratosis
Psoriasis, calluses
320
Hyperkeratosis with retention of nuclei in stratum corneum
Parakeratosis
321
Which pathology includes Parakeratosis?
Psoriasis
322
What is Spongiosis?
Epidermal accumulation of edematous fluid in intercellular spaces
323
Example of disease that has spongiosis?
Eczematous dermatitis
324
Separation of epidermal cells
Acantholysis
325
Which pathology presents with acantholysis?
Pemphigus vulgaris
326
What is Acanthosis?
Epidermal hyperplasia (↑ spinosum)
327
Pathology with Acancthosis
Acanthosis nigricans
328
Pigmented skin disorders
Albinism
Melasma (chloasma)
Vitiligo
329
What is the problem in albinism?
Normal melanocyte number with ↓ Melanin production
330
What causes ↓ Melanin production in Albinism?
Due to ↓ Tyrosinase activity or defective tyrosine transport
331
What is another cause of Albinism?
By failure of neural crest cell migration during development
332
What risk is increased for Albinism?
Skin cancer
333
Hyperpigmentation associated with pregnancy ("mask of pregnancy")
Melasma (chloasma)
334
Situations associated to Melasma
Pregnancy or OCP use
335
Clinical characteristics of Vitiligo
Irregular areas of complete depigmentation
336
Cause of Vitiligo
By autoimmune detruction of melanocytes
337
Pathologies with Verrucae
```
Warts
Epidermal hyperplasia
Hyperkeratosis
Koilocytosis
Condyloma acuminatum
```
338
Who causes warts?
By HPV
339
Characteristics of Warts
Soft, tan-colored, cauliflower like papules
340
Verrucae on Genitals
Condyloma acumunatum
341
Common mole
Melanocytic nevus
342
How is Melanocytic nevus consider?
Benign, but melanoma can arise in congenital or atypical moles
343
Skin lesion of intradermal nevi
Papula
344
Skin lesion of Junctional nevi
Flat macules
345
Alternative name for Urticaria
Hives
346
Skin lesion in Urticaria
Pruritic wheals
347
When are wheals form in Hives?
After mast cell degranulation
348
Characteristics of Urticaria
Superficial dermal edema and lymphatic channel dilation
349
Alternative name for Freckles
Ephelis
350
What is the main point of Freckles?
Normal number of melanocytes, ↑ melanin pigmentation
351
Alternative name for Eczema
Atopic Dermatitis
352
How is Eczema consider?
Pruritic eruption
353
Wheres in Atopic Dermatitis commonly found?
On skin flexures
354
Wat else is associated to Atopic Dermatitis?
With other atopic diseases (asthma, allergic rhinitis)
355
How is the usual evolution of Atopic Dermatitis?
Ussually starts on the face in infancy and often appears in the antecubital fossae thereafter
356
Hypersensitivity reaction in Allergic contact dermatitis
Type IV hypersensitivity reaction that follows exposure to allergen
357
Sites where Allergic contact dermatitis occurs
At site of contact
358
Common causes of Allergic contact dermatitis
Nickel, poison ivy, neomycin
359
Skin lesions characteristics on Psoriasis
Papules and plaques with silver scaling
360
Where does Psoriasis often occur?
Especially on knees and elbows
361
Hystologicy characteristics in Psoriasis
Acanthosis with parakeratosis scaling
| ↑ stratum spinosum, ↓ stratum granulosum
362
What is parakeratosis?
Nuclei still in stratum corneum
363
Which sign is present in Psoriasis?
Auspitz sign
364
What is Auspitz sign?
Pinpoint bleeding spots from exposure of dermal papillae when scales are scraped off
365
What is associated to Psoriasis?
Nail pitting and Psoriatic Arhtitis
366
Skin lesions seen in Seborrheic keratosis
Flat, greasy
367
Hystological characteristics of Seborrheic keratosis
Pigmented squamous epithelial proliferation with keratin filled cysts (horn cyst)
368
Looks of Seborrheic keratosis
"Stuck on"
369
Where do lesions of Seborrheic keratosis occur?
On head, trunk, and extremities
370
Common benign neoplasm of older people
Seborrheic keratosis
371
Which sign is possible in Seborrheic keratosis?
Leser Trelat sign
372
What is Leser Trelat sign?
Sudden apperance of Multiple seborrheic keratoses
373
What does Leser Trelat sign in Seborrheic keratosis indicate?
An underlying malignancy (eg. GI, lymphoid)
374
Infectious skin disorders
Impetigo
Cellulitis
Necrotizing fasciitis
Staphylococcal scalded skin syndrome
375
How is impetigo consider?
A very superficial skin infection
376
Who are the main organisms that cause impetigo?
S. aureus or S.pyogenes
377
How contagious is Impetigo?
Highly contagious
378
Apperance of Impetigo
Honey colored crusting
379
Variant of impetigo
Bullous impetigo
380
Who causes bullous impetigo?
S. aureus
381
Acute, painful, spreading infection of dermis and subcutaneous tissues
Cellulitis
382
Clinical presentation of Cellulitis
Acute, painful
383
Skin layers affected by cellulitis
Spreading infection Dermis and subcutaneous tissues
384
Main bacterias that cause Cellulitis
S. aureus
| S. pyogenes
385
How does Cellulitis often starts?
With a break in skin from trauma or another infection
386
What is Necrotizing fasciitis?
Deeper tissue injury, usually from anaerobic bacteria or S. pyogenes
387
Main agents that cuase Necrotizing fasciitis
Usually from anaerobic bacteria or S. pyogenes
388
Clinical finding in Necrotizing fasciitis
Results in crepitus
389
What explains Crepitus in Necrotizing fasciitis?
Methane and CO2 production
390
How are the bacterias of Necrotizing fasciitis consider?
As "Flesh eating bacterias"
391
What does Necrotizing fasciitis causes?
Bullae and a purple color to the skin
392
Pathophysiology of Staphylococcal scalded skin syndrome
Exotoxin destroys keratinocyte attachments in the stratum granulosum only
393
What is the differecne between Staphylococcal scalded skin syndrome vs Toxic epidermal necrolysis?
Toxic epidermal necrolysis, destroys the epidermal junction
394
Characterized by fever and generalized erythematous rash with sloughing of the upper layers of the epidermis that heals completely
Staphylococcal scalded skin syndrome
395
Findings of Staphylococcal scalded skin syndrome
Characterized by fever and generalized erythematous rash with sloughing of the upper layers of the epidermis that heals completely
396
Group of age when Staphylococcal scalded skin syndrome appears
Newborns and children
397
White, painless plaques on the tongue that cannot be scraped off
Hairy leukoplakia
398
Causant agent of Hairy leukoplakia
EBV
399
In which patients is hairy leukoplakia seen?
HIV positive patients
400
Blistering skin disorders
```
Pemphigus vulgaris
Bollous pemphigoid
Dermatits herpetiformis
Erythema multiforme
Stevens Johnson syndrome
```
401
How is Pemphigoid vulgaris consider?
Potentially fatal autoimmune disorder
402
What is the cause of Pemphigoid vulgaris?
Autoimmune disorder with IgG antibody against desmoglein
403
What is desmoglein?
Component of desmosomes
404
Findings of Pemphigoid vulgaris
Flacid intraepidermal bullae
405
What causes Flacid intraepidermal bullae in Pemphigoid vulgaris?
By acantholysis
406
How are keratinocytes in stratum spinosum connected?
By desmosomes
407
Which study helps to make the diangosis of Pemphigoid vulgaris? What do we see?
Immunofluorescence reveals antibodies around epidermal cells in a reticular (net-like pattern)
408
Structure involved in Pemphigoid vulgaris, other than skin?
Oral mucosa
409
Sign present in Pemphigoid vulgaris
Nikolsky sign
410
What is Nikolsky sign?
Separation of epidermis upon manual stroking of skin
411
Between Pemphigoid vulgaris and Bullous pemphigoid, which is less severe?
Bullous pemphigoid
412
What is the problem in Bullous pemphigoid?
Involves IgG antibody against hemidesmosomes
413
Skin layer affected in Bullous pemphigoid
Epidermal basement membrane
414
Clinical findings on Bullous pemphigoid
Tense blisters containing eosinophils
415
Does Bullous pemphigoid affect oral mucosa?
No
416
Study for Bullous pemphigoid.... and what does it shows?
Immunofluorescence reveals linear pattern at epidermal dermal junction
417
How is Nikolsky sign in Bullous pemphigoid?
Negative
418
Skin lesion of Dermatitis herpetifomis
Pruritic papules, vesicles and bullae
419
Where is Dermatitis herpetifomis often seen?
Elbows
420
Deposits of IgA at the tips of demal papillae
Dermatitis herpetiformis
421
What is the probleme Dermatitis herpetifomis?
Deposits of IgA at the tips of demal papillae
422
Which pathology is associated to Dermatitis herpetifomis?
Celiac disease
423
What is associated to Erythema multiforme?
Infections, drugs, cancers, and autoimmune disease
424
Infections associated to erythema multiforme
Mycoplasma pneumoniae, HSV
425
Drugs associated to erythema multiforme
Sulfa drugs, β lactams, phenytoin
426
Types of lesions on Erythema multiforme
Macules, papules, vesicles and target lesions
427
Characteristics of target lesions in Erythema multiforme
Looks like targets with multiple rings and a dusky center showin epithelial disruption
428
Skin disease with target lesions
Erythema Multiforme
429
Characterized by fever, bulla formation and necrosis, sloghing of skin and a high mortality rate
Stevens Johnson syndrome
430
Lesions seen in Stevens Johnson syndrome
Typically 2 mucous membranes are involced and skin lesions may appear like targets as seen in erythema multiforme
431
Which is the main event associated to Stevens Johnson syndrome?
Adverse drug reaction
432
More severe form of Stevens Johnson syndrome
Toxic epidermal Necrolysis
433
When is Stevens Johnson syndrome categorized as Toxic epidermal Necrolysis
With > 30 % of the body surface area involved
434
Miscellaneous skin disorders
```
Acanthosis nigricans
Actinic keratosis
Erythema nodosum
Lichen planus
Pityriasis rosea
Sunburn
```
435
Epidermal hyperplasia causing symmetrical, hyperpigmented, velvety thickening of skin
Acanthosis nigricans
436
Where dos acanthosis nigricans especially occurs?
On neck or in axilla
437
Situations associated to Acanthosis nigricans
With hyperinsulinemia (eg. diabetes, obesity, Cushing syndrome) and visceral malignancy (eg. gastric adenocarcinoma)
438
How is Actinic keratosis consider?
Premalignant lesions
439
What causes Actinic Keratosis?
caused by sun exposure
440
Skin lesions of Actinic Keratosis
Small, rough, erythematous or browinish papules or plaques
441
What risk is increased with Actinic Keratosis?
Squamous cell carcinoma is proportional to degree of epithelial dysplasia
442
Painful inflammatory lesions of subcutanoeus fat
Erythema nodosum
443
Where is Erythema nodosum commonoly found?
Usually on anterior shins
444
Causes of Erythema nodosum
Often idiopathic, but can be associated to sarcoidosis, coccidioidomycosis, histoplamosis, TB, streptococcal infections, leprosy, and Crohn disease
445
Clinical manifestation of Lichen Planus
```
6 Ps of Lichen Planus
Pruritic
Purple
Polygonal Planar Papules
Plaques
```
446
How does mucosal involvement is manifested in Lichen Planus?
Manifests as Wickham striae
447
What are Wickham striae?
Reticular white lines
448
Microscopic findings in Lichen Planus
Sawtooth infiltrate of lymphocytes at dermal epidermal junction
449
Pathology associated with Lichen Planus
Hepatitis C
450
Lesions evolution in Pityriasis rosea
"Herald path" followed days later by "Christmas tree"
451
Types of dermal lesions in Pityriasis rosea
Multiple plaques with collarette scale
452
Treatment for Pityriasis rosea
Self resolving in 6-8 weeks
453
What is a Sunburn?
Acute cutaneous inflammatory reaction due to excessive UV irradiation
454
Cellulary what are the effects of Sunburn?
Causes DNA mutations, indicing apoptosis of keratinocytes
455
Which UV rays are dominant in tanning and photoaging?
UVA
456
Which UV rays are seen in sunburns?
UVB
457
Skin pathologies that sunburns can lead to...
Can lead to impetigo and skin cancer (basal cell carcinoma, squamous cell carcinoma, and melanoma)
458
Skin cancers
basal cell carcinoma
squamous cell carcinoma
melanoma
459
Most common skin cancer
Basal cell carcinoma
460
Where is basal cell carcinoma found?
In sun exposed areas
461
Prognosis of Basal cell carcinoma
Locally invasive, but almost never metastasizes
462
Skin lesions foun in basal cell carcinoma
Pink, pearly nodules, commonly with telangiectasia, rolled bordersand central crusting or ulceration
Or scaling plaque
463
When ypu see a nonhealing ulcers with infiltrating growth, you should suspect of...
Basal cell carcinoma
464
Histology characteristics pf Basal cell carcinoma
Basal cell tumors have "Palisading" nuclei
| Revelas nest of basaloid cells in dermis
465
Second most common skin cances
Squamous cell carcinoma
466
Factors associated to Squamous cell carcinoma
Excessive sexposure to sunlight, immunosuppression, and ocasionally arsenic exposure
467
Common sites of Squamous cell carcinoma aperance
On face, lower lip, ears and hands
468
How is squamous cell carcinoma consider?
Locally invasive, but may spread to lymph nodesand will rarely metastasize
469
Clinical findings in Squamous cell carcinoma
Ulcerative red lesions with frequent scale
470
What is associated to Squamous cell carcinoma?
Chronic draining sinuses
471
Histopathology of Squamous cell carcinoma
Keratin pearls
472
Precursor to squamous cell carcinoma
Actinic keratosis
473
Clinical aperancer of Actinic keratosis
A scaly plaque
474
Variant of Squamous cell carcinoma that grows rapidly (4-6 weeks) and may regress spontaneously over months
Keratoacanthoma
475
Common skin tumor with significant risk of metastasis
Melanoma
476
Which is the tumor marker seen in Melanoma?
S-100 tumor marker
477
Who have higher risk of Melanoma?
Fair skin persons
478
What is associated to Melanoma apperance?
With sunlight exposure
479
What factor correlates with risk of Metastasis of Melanoma?
Depth of tumor
480
Characteristics of Melanoma
```
ABCDE
Asymmetry
Border irregularity
Color variation
Diameter > 6 mm
Evolution over time
```
481
4 types of Melanoma
Superficial spreading melanoma
Nodular melanoma
Lentigo maligna melanoma
Acrolentiginous melanoma
482
Gene activated in Melanoma
Often driven by activating mutation in BRAF kinase
483
Primary treatment for Melanoma
Is excision with appropriately wide margins
484
Which situations of Melanoma could be treated with medication?
Metastatic or unresectable melanoma
485
Which gene seem to be mutated in patients with Metastatic or unresectable melanoma?
BRAF V600E mutation
486
Drug used for Metastatic or unresectable melanoma
Vemurafenib
487
What is Vemurafenib?
BRAF kinase inhibitor
