Musculoskeletal, skin and connective tissues Flashcards

1
Q

20 - 50 female, 对称性、弥漫性肌肉痛 + insomnia + fatigue: Dx? Rx? 和 polymyalgia rheumatic, polymyositis 鉴别诊断

A

Fibromyalgia 纤维肌痛 病因不明 Rx:regular exercise + antidepressenets (TCAs, SSRIs) + anticonvulsants

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2
Q

> 50 yr, 双侧、对称性pain and stiffness in shoulders and hips, often with constitutional sym (night fever, malaise, weight loss): Dx? disease association? lab? Rx?

A

Dx: polymyalgia rheumatica 风湿性多肌病 disease association: temporal arteritis (50%颞动脉炎病人合并此病 - 怀疑颞动脉炎要马上给激素,防止blindness) lab: ↑ ESR, ↑ CRP, normal CK Rx: low-dose steroids

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2
Q

2nd MC skin cancer? cause? Cx? 好发部位?microscope? if metastasize? precursor disease (重要考点)? a variant disease? 其特点?

A

2nd MC skin cancer: SCC (squamous cell carcinoma) cause: sun, immunosuppression, arsenic exposure Cx: ulcerative lesions with frequent scale 好发部位: face, lower lip, ears, hands microscope: keratin “pearls” if metastasize: rarely, locally invasive, but may spread to lymph nodes precursor disease (重要考点): AK (actinic keratosis) 光化性角化病 a variant disease:keratoacanthoma 角化棘皮瘤 grow rapidly (4-6 wks), but may regress spontaneously over months

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3
Q

Acanthosis nigricans (AN): Cx? Disease association (最重要的考点!!)?

A

Cx: epidermal hyperplasia, symmetrical hyperpigmented, velvety thickening of skin, 好发部位在neck and axilla 腋窝 associated with: 2大类,分为良性、恶性: 1. benign AN: insulin resistance (DM, obesity, cushing syn) 2. malignant AN: visceral malignancy, especially GI and genitourinary neoplasm 如果是中老年病人,忽然出现黑色棘皮,一定要怀疑恶性肿瘤 最常见的肿瘤:gastric adenocarcinoma

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3
Q

Acetaminophen: 什么类型药? Mechanism? 可逆否?作用位点?inactivation位点? 是否解热?是否抗炎?Use? toxicity and the mechanism? antidote?

A

扑热息痛,和NSAID类似,都是可逆地抑制COX 作用位点: CNS inactivation位点: peripheral 是否解热? - yes (anti-pyretic) 是否抗炎?- No Use: use instead of aspirin in children to avoid Reye syn toxicity and the mechanism: hepatic necrosis; acetaminophen metabolite (NAPQI) depletes glutathione - form toxic tissue adducts in liver antidote: N - acetylcysteine (regenerate glutathione)

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4
Q

Albinism病因?黑色素细胞数目有无变化?

A

normal melanocyte #, with ↓ melanin production 病因: 1. ↓ tyrosinase activity 2. defective tyrosine transport 3. failure of neural crest cell migration during development ↑ risk of skin cancer

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4
Q

actinic keratosis: Cx? why important?

A

光化性角化病 Cx: small, rough (feel like “sand paper”) erythematous or red/brown papules on sun-exposed area; confined to epidermis premalignent to SCC! need to be monitored

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4
Q

acute gout drugs?

A
  1. NSAIDs: naproxen 萘普生; indomethacin 2. glucocorticoids (oral or intraarticular) 3. colchicine (stabilize microtubulin ; inhibit chemotaxis of leukocytes) GI side effects
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4
Q

Adalimumab: 什么类型药?同种药物还有什么? Mechanism? use? 要注意什么?

A

anti-TNFa McAb (同种药物还有Infliximab) use: IBD,RA, psoriasis, AS (ankylosing spondylitis) 要注意什么:prediposit for infection (reactive latent TB)

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5
Q

allergic contact dermititis: type ? hypersensitivity reaction major histological changes?

A

type IV histological changes: spongiosis 棘细胞水肿

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6
Q

Allopurinol抗痛风的机制?同样的药物还有? 如果用于lymphoma, leukemia机制?要注意什么?

A

Allopurinol抗痛风机制:inhibit XO (xanthine oxidase) 同样的药物还有Febuxostat。急性发作时是禁忌症(应用NSAIDs) 如果用于lymphoma, leukemia机制: prevent tumor lysis-caused irate nephropathy 要注意如果和azathioprine, 6-MP联用抗肿瘤,会↑ 这两种药物concentration: (both metabolized by XO), 所以如果联用,一定要降低抗肿瘤药物的剂量!!!!

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8
Q

Ankylosing spondylitis: Cx? XR? HLA (class I or II?) 累及其他的器官表现?

A

Cx: chronic dull back pain, morning stiffness, can be relieved by over-counter pain killer; 一生中不断缓解和复发 XR: “bamboo spine”- vertebral fusion HLA-B27 (class I; class II are DR, DP, DQ) 除了脊柱之外常累及: 1) respiratory: pain limits chest expansion - hypoventilation: 病人随访中必须监测chest expansion 2) cardiac: ascending aortitis ⇒ dilation of aortic ring, aortic insufficiency - AR (aortic regurgitation) 3) eye: uveitis: pain in the eyes, blurred vision, 结膜充血

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8
Q

Aspirin: Mechanism? 作用可逆否? Clinical use? Toxicity? 对respiratory center作用?

A

Mechanism? 作用可逆否: covalently acetylate cyclooxygenases (both COX1 and COX2), ⇒ ↓ TXA2, PGs Clinical use: 1. low dose (< 300 mg/day): anti-platelet aggregation (↑ bleeding time until new platelets are made: ~ 7 d; no change in PT, PPT) 小剂量aspirin用于prevention of transient ischemic attack, MI, stroke [对于PE不如wafarrin有效,对于DVT不如heparin有效】 2. intermediate dose (300 - 2400 mg/d): anti-pyrectic, analgesic 3. high dose (> 2400 mg/day): anti-inflammation Toxicity: 1. in adults: gastric ulcer, tinnitus (CN VIII), chronic use caused GI bleeding, nephritis, acute renal failure 2. to treat kids for viral infection: Reye syn stimulate respiratory center⇒ hyperventilation ⇒ respiratory alkalosis

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9
Q

atopic dermatitis又名? 诊断hallmark? associated with diseases?

A

atopic dermatitis又名eczema 湿疹 诊断hallmark: intense pruritis, can’t Dx without it 常在5-6岁(甚至婴儿期起病),start on the face in infancy, and often appears in the antecubital fossae thereafter; 终身反复, often associated with other atopic diseases (asthma, allergic rhinitis - UW 考点)

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9
Q

biphosphonates (Alendronate, other -dranotes) 什么类型药? Mechanism? clinical use? toxicity? 如何预防?

A

什么类型药:pyrophosphate analogues Mechanism: inhibit osteoclast activity by binding to hydroxyapatite in bone clinical use: osteoporosis, 恶性高钙血症, Paget disease toxicity - corrosive esophagitis; osteonecrosis of the jaw 如何预防- pts take lot of water and remain upright for 30 min;

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10
Q

Dx: fever + generalized erythematous rash + sloughing of the upper layers of the epidermis that heals completely; seen in newborns and children

A

Dx: Staphylococcal scalded skin syndrome exotoxin destroys keratinocyte attachments in the stratum granulosum (颗粒层,皮肤从外到里第3层), ⇒ destroy the epidermal dermal junction

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10
Q

blistering skin disorders有哪两种? 区别: 1. 严重程度? 2。Ab against? 3. oral mucosa involvement? 4. 荧光染色location? pattern? 5. Nikolsky sign positive/negative?

A

PA vs. bullous pemphoid, see “notes for printing”

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10
Q

Dx: multiple types of skin lesions (macules, papules, vesicles, “target lesions”-targets with rings and a dusky center showing epithelial disruption) associated with?

A

Dx: erythema multiforme associated with: 1. infection - mycoplasma pneumoniae, HSV 2. drugs (sulfa, beta-lactams, phenytoin) 3. cancers 4. autoimmune

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10
Q

Dx: multiple plaques with collarette scale (蜀黍红疹), “herald patch” followed days later by “X’mas tree” distribution?

A

Pityriasis rosea 玫瑰糠疹 self-resolving in 6-8 wks

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12
Q

Dx: progressive symmetric proximal muscle weakness (常考的描述是“can’t comb hair, can’t raise from chair, 说明累及shoulders and hips):最可能的诊断? activation of which immune cells? release cytokines?

A

Dx: polymyositis endomysial inflammation with CD8+ Th1 cells: activate macrophage to release IL-1, TNFa

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13
Q

Dx: proximal muscle weakness (can’t comb hair, can’t clim up stairs) + autonomic sym (dry month, diplopia, impotence)? associated with which disease? 病因?和什么疾病鉴别?

A

Dx: Lambert-Eaton myathenic syndrome (LEMS) associated with small lung cancer (i.e.: CXR 可见mass in upper lung) 病因: autoantibodies to presynaptic Ca2+ channel ⇒ ↓ Ach release (improve with muscle use) 和什么疾病鉴别: MG (myasthenia gravis) - ab against Ach postsynpatic receptors, worsen with muscle use, associated with thymoma, sym can be reversed with AchE inhibitors)

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14
Q

Dx: pruritic papules, bullae on elbow; 和什么病鉴别? which Ab deposit at where? disease association?

A

dermatitis herpetiforms 和PV (pemphigus vulgaris), bullous pemphipgoid鉴别 IgA deposited at tips of dermal papillae; associated with celiac disease

16
Q

Dx: skin rash (骨关节突出部位) + progressive muscle weakness? Lab? Rx?

A

dermatomyositis malar rash, Gottron papules (手指骨关节的红斑)-特征性病变 Heliotrope rash (upper eye lids或者眶周红斑) -特征性病变 CD4+ T cells Lab: ↑ CK (-特征性病变), anti-Jo-1 (-特征性病变), anti-SRP (specific but not characteristic), anti-Mi-2 Rx: steroids

17
Q

Dx: white, painless plaques on the tongue, can’t be scraped off caused by which virus? in which population of pts?

A

Dx: hairy leukoplakia 毛状白斑 caused by EVB occur in HIV+ pts

18
Q

Dx: yo male, fever, malaise, macropapular rash on palms and soles?病原体? screen test? 确诊test?

A

Dx: 二期梅毒 病原体:Treponema pallidum screen test: RPR(VDRL) test: add pt’s serum to a mix of cardiolipin + lecithin + cholesterol: flocculation (= aggulation) occurs 用于筛查,不特异 确诊test: FTA-ABS

19
Q

erythema nodosum Cx? Disease association

A

结节性红斑 Cx: painful, inflammatory lesions, 多发于腿部 extremely non-specific findings, associated病实在太多了!! sarcoidosis, coccidiodomycosis, histoplasmosis, TB, streptococcal infection, leprosy, Crohn disease

20
Q

Etanercept: 什么类型药? Mechanism? use? 要注意什么?

A

依那西普 TNFa decoy receptor (fusion protein of TNGa receptor + IgG1 Fc) use: RA, psoriasis, AS (ankylosing spondylitis) 要注意什么:prediposit for infection (reactive latent TB)

21
Q

febuxostat: 什么类型药? Mechanism?

A

gout prevention 机制和allopurinol 类似,inhibit XO (xanthine oxidase)

22
Q

Gout的2种原因?和pseudogout的鉴别?

A

Gout : 90%: underexcertion of uric acid - 原发性较多,也可能由medication加重(thiazide diuretics) 10%: overproduction of uric acid, caused by: Lesch-Nyhan, von Gierke (糖原储积病,G-6-phophatase deficiency), PRPP, ↑ cell turnover (tumor lysis syn) birefringent偏振光: gout: negative (crystals are yellow under parallel light; blue under perpendicular light) pseudo gout:positive; crystals are blue under parallel light;

24
Q

Infectious arthritis: MCCs?

A

In US: gonococcal arthritis是最常见的感染性关节炎!!! “STD” = synovitis 滑膜炎,tenosynovitis 腱鞘炎,dermatitis presents as migratory arthritis, asymmetric; affected joint is swollen, red and painful S. aureus, Streptococcus, Neisseria gonorrhoeae

25
Q

Infliximab: 什么类型药? Mechanism? use? 要注意什么?

A

anti-TNFa McAb use: IBD,RA, psoriasis, AS (ankylosing spondylitis) 要注意什么:prediposit for infection (reactive latent TB)

26
Q

Lichen Planus: Dx: 6P? Mucosal involvement manifest? patho见什么细胞浸润皮肤什么部位? Disease association

A

Dx: 6P: pruritic, purple (在黑皮肤上不一定是紫色),polygonal, planner papule or plaque Mucosal involvement manifest: reticular white lines (Wickham striae) patho见什么细胞浸润皮肤什么部位: sawtooth infiltration of lymphocytes at dermal-epidermal junction Disease association: hepatitis C

27
Q

MC skin cancer? cause? Cx? microscope? if metastasize?

A

BCC (basal cell carcinoma) cause: sun exposure Cx: pink, pearly nodules, central crusting or ulceration; non-healing ulcers, or scaling plaque microscope: nests of basaloid cells; palisading (栅栏样的) nuclei if metastasize: almost never; only locally invasive

29
Q

myositis ossificans 骨化性肌炎 什么病?常见部位?

A

metaplasia (组织转化) of skeletal muscle to bone following trauma 常见部位:upper or lower extremity present as suspicious mass, 或者XR偶然发现

31
Q

osteopetrosis: defecit in? X-ray表现?对骨髓、颅神经的影响?potential Rx?

A

deficit: mutations (eg: carbonic anhydrase II 碳酸酐酶) ⇒ osteoclasts deficit!! can’t reabsorb bones X-ray表现: thickened, dense bones prone to fracture (“bone in bone” 对骨髓、颅神经的影响: fill BM ⇒ pancytopenia, extra medullary hematopoiesis; if narrowed foramina: can result in cranial nerve impingement and palsies potential Rx: BMT

32
Q

osteoporosis: 哪种骨受累?Dx? Types, 各自骨折最容易发生部位, 以及损伤血管? Rx?

A

哪种骨受累:trabecular (spongy) bone loses mass and interconnections; [normal bone mineralization: so normal lab values: serum Ca2+, PO3-正常】 Dx: DEXA (bone mineral density test) with a T-score < 2.5 Types: Type 1: postmenopause, due to ↓ E2; MC seen in femoral neck fracture 【损伤medial femoral circumflex A, distal radius fracture; Type II: senile osteoporosis, in > 70-yr old, MC in spine [compression fracture] Rx: - prophylaxis: regular weight-bearing exercise, adequate calcium and VitD intake Rx with biphsphonates, PTH, SERMs, denosumab (McAB against RANKL)

34
Q

Paget disease: Patho: which cell(s) involved? Stages of disease? Lab? Cx? 累及部位? Biopsy可见? ↑ risk for which cancer?

A

bone remodeling deficit, common Patho: which cell(s) involved: both osteoclasts and -blasts, 主要是osteoclast activity ↑, too much bone absorption ⇒ bone pain Stages of disease: (先破后立) - lytic: osteoclasts - mixed: osteoclasts + osteoblasts - sclerotic: osteoblasts quiescent: minimal osteoblasts/osteoclast activity Lab: normal serum Ca2+, PO3-, PTH ↑ ALP, ↑ activity of M-CSF, RANK-L (to stimulate osteoclasts) Cx: bone pain, long bone chalk-stick fractures; can affect skull - hearing loss Biopsy可见: mosaic pattern of woven and lamellar bone, multinucleate cells (破骨母细胞), ↑ risk for which cancer: osteogenic sarcoma

35
Q

Probenecid:什么类型药? Mechanism? 要注意什么?

A

丙磺舒, 抗痛风(预防和慢性治疗)急性发作时是禁忌症(应用NSAIDs) Mechanism: inhibit reabsorption of uric acid in PCT; also inhibit secretion of penicillin

36
Q

psoriasis: 皮肤哪(几)层厚度变化? microscopic change? associated with diseases? Rx?

A

↑ stratum spinosum (棘层变厚,第四层);↓ stratum granulosum (颗粒层【第三层】变薄) parakeratotic scaling; pinpoint bleeding when scales are scraped off associated with diseases: nail pitting, psoriatic arthritis Rx:

38
Q

Reactive arthritis (Reiter syn): Cx? HLA? Cause?

A

post GI infection or Chlamydia (衣原体)感染, 例如腹泻后2周出现关节炎症状:symmetric large joint arthritis, 关节炎培养无细菌(sterile synovial fluid on joint aspiration) Cx: classic triad (Can’t see, can’t pee, can’t bend my knee) 1.Conjunctivitis and anterior uveitis 葡萄膜炎 2.urethritis 3. arthritis HLA-B27 病原体:SSYCC Shigella, Salmonella, Yersinia, Campylobacter 弯曲杆菌, Clamydia

39
Q

Sarcoidosis: Cx? CXR? patho? Ca2+变化,什么原因?哪些immune cells involved?

A

Cx: african American + bilateral hilar adenopathy on CXR, patho 见noncaseating granulomas —— 考题里绝对是诊断为sarcoidosis!! Ca2+ ↑ , caused by activated macrophages synthesized too much 1, 25-VitD ⇒ suppress PTH secretion Immune cells: Th1 cell-mediated immune reaction: these T cells secrete IL-2 and INFƔ

40
Q

Scleroderma (systemic sclerosis) 病因?Cx? 好发人群?分型及ab association?

A

病因:excessive deposition of fibrosis and collagen throughout the body Cx: 1. 皮肤表现:puffy and taut skin, without wrinkles 2.其他器官:sclerosis of renal, pulmonary (MCC of death), cardiovascular, GI 好发人群: females 2 types: 1) diffuse scleroderma: rapid progression + early visceral involvement, associated with anti-Scl-70 Ab (anti-DNA topoisomerase I ab) 2) limited scleroderma: CREST, more benign course associated with anti-Centromere Ab (C for CREST)

41
Q

seronegative spondyloarthropathies: definition? HLA association? 包括哪些疾病?

A

definition: rheumatoid factor negative (rheumatoid factor: anti-Ig IgM antibody) HLA association: HLA-B27 (encodes HLA MHC class I) 包括哪些疾病: PAIR 1. psoriatic arthritis: t bend my knee

43
Q

Sjögren’s syndrome: 什么病?特征?好发人群? Cx? Ab markers有哪些?腮腺的表现? complications?

A

Autoimmune, destruction of exocrine glands, especially lacrimal and salivary glands 好发人群: 40-60 females Cx: 1. xerophthalmia (↓ tear ⇒ damage to cornea) 眼干燥 2. xerostomia ((↓ saliva) 口腔干燥 3. bilateral parotid enlargement Ab markers有anti-nuclear Ab : SS-A (anti-Ro), SS-B (anti-La) 腮腺: bilateral enlargement (和并发症里MALT lymphoma的单侧腮腺肿大区别) complications: 1)dental caries 2) MALT lymphoma (mucosa-associated lymphoid tissue); may present as unilateral parotid enlargement

44
Q

Skin biopsy 见stellar cells with intracytoplasmic granules (shape: tennis racquet): what are these cells? express which MHC? co-stimulatory molecule? derived from ? lineage?

A

Langerhans cells (dendritic cells, APC) express MHC II co-stimulatory molecule: B7 derived from myeloid lineage

46
Q

SLE: Cx? 哪种免疫细胞介入? MCC for death? 心脏、肾脏累及的表现? Rx?

A

Cx: RASH OR PAIN Rash Arthritis Soft tissues/serositis Hematologic disorder (cytopenias) Oral /nasopharyngeal ulcers Renal disease, Raynauld phenomenon Photosensitivity,Positive VDRL/RPR Antinuclear Abs (ANA-sensitive but not specific, anti-dsDNA - specific, poor prognosis, indicating renal disease; ) Immunosuppressants (↓ C3, C4, CH50 due to immuoplex formation) Neurologic disorders (seizures, psychosis) 哪种免疫细胞介入: Th1 (例如Fas gene mutation, Th1 over activated) MCC for death: 1. Cardiovascular diseases 2. infection 3. renal disease 心脏累及的表现: 1. pericarditis, 胸膜炎 【pain radiates to back, shoulders; inspiration加重,relived by sitting up] 2.Libman-Sacks endocarditis: wart-like vegetations on both sides of valve 肾脏累及的表现: lupus nephritis (type III hypersensitivity) 1. Nephritic - diffuse proliferative glomerulonephritis 2. neprhotic - membranous glomerulonephritis Rx: NSAIDs, steroids, immunosuppressants, hydroxychloroquine

47
Q

specific COX2 inhibitor? why use selective inhibitor against COX2? clinical use? COX2有什么特点?

A

celecoxib 考昔 nonspecific COX inhibitors (NSAIDs) cause GI bleeding and renal damage celecoxib does not have the corrosive effects on the GI lining; spare platelet functions (TXA2 production is NOT dependent on COX-1) Used in RA and osteoarthritis, or pts with gastritis or ulcers COX2有什么特点:72 KDa enzyme, inducible, very specifically expressed in inflammatory cells and vascular endothelium; almost undetectable in other tissues !

48
Q

Stevens-Johnson syn: Cx: Association? If > 30% skin involved: Dx?

A

Cx: fever + bulla formation and necrosis + sloughing of skin [high mortality] 一般至少有2种mucous involved If > 30% skin involved: Dx for toxic epidermal necrolysis

49
Q

sunburn中 UVA dominant in which cases? wavelength? protected by? UVB? wavelength? protected by?

A

UVA dominant in: tanning, photoaging; 320 - 400 nm, protected by avobenzone 阿伏苯宗 UVB: sunburn; 290- 320 nm, protected by PABA (para-aminobenzoic acid) 对-氨基苯甲酸 Zinc oxide protects against both

50
Q

urticaria [er:ti’karia]: patho?

A

荨麻疹 mast cell degranulation superficial dermal edema, lymphatic channel dilation

51
Q

very painful skin infection lesion + honey-colored crusting, or bullae: Dx? 病原体?- 这些病原体还引起什么常见的皮肤病? 是否传染? 最常见病原体的virulence factor, 其作用?

A
  1. Dx: impetigo [impi’taigou] 脓疱病 caused by S. aureus or S. progenies highly contagious,在皮肤表浅处感染 2. S. aureus or S. progenies如果在dermis and subcutaneous tissues感染, often starts with a break in skin from trauma or another infection: cellulitis 蜂窝织炎 3. S. aureus or S. progenies如果在皮肤深处感染,还常常引起necrotizing fasciitis 坏死性筋膜炎 最常见病原体S. aurues的virulence factor: protein A 其作用: bind the Fc portion of IgG ⇒ impaired complement activation (↓ C3b), opsonization, phagocytosis
52
Q

最容易转移的皮肤癌? tumor marker? Cx? gene mutation? Rx?

A

melanoma: associated with sun exposure; light skin: ↑ risk [“Greys’ anatomy”里Izzy case] tumor marker: S-100 Cx: “ABCDE” Asymmetry Border irregularity Color variation Diameter > 6 mm Evolution over time gene mutation: BRAF kinase Rx: 1) excision with wide margins 2) if can’t resect or metasize + BRAF V600E (Val ⇒ Glutamic acid) mutation: Vemurafenib (BRAF kinase inhibitor)

53
Q

确诊RA需要什么Ab?

A

Not RA!!! anti-CCP ab (cyclic citrullinated peptides)