Peau et oncologie cutanée Flashcards

1
Q

What are the main functions of the skin (4)

A
  • Protection (UV, mechanical, chemical, thermal)
  • Immunologic (barrier to microorganisms)
  • Metabolic (vitamin D synthesis)
  • Thermoregulation
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2
Q

What is the embryological origin of the epidermis

A

Ectoderm

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3
Q

What is the embryological origin of the dermis

A

Mesoderm

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4
Q

What is the embryological origin of melanocytes

A

Neural crest

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5
Q

What is the embryological origin of Langerhans cells

A

Mesenchymal origin

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6
Q

How long does it take for keratinocyte differentiation to occur

A

28-45 days from basal layer to cornified layer

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7
Q

What 4 cells are found in the epidermis

A
  • Keratinocytes
  • Melanocytes
  • Langerhans cells
  • Merkel cells
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8
Q

What 3 cells are found in the dermis

A
  • Fibroblasts
  • Macrophages
  • Mast cells

aussi occasionnellement: lymphocytes

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9
Q

What appendage cells are found in the epidermis

A

None

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10
Q

What appendage cells are found in the dermis

A
  • Hair follicles
  • Sebacious glands
  • Eccrine and Appocrine sweat glands
  • Meissner’s corpuscules
  • Pacinian corpuscules
  • Ruffini endings
  • Bulb of Krause
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11
Q

What is the function of eccrine glands

A

Thermoregulation

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12
Q

What is the function of apporcine glands

A

Sweat

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13
Q

What is the function of Langerhans cells

A

Immunity (antigen response)

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14
Q

Cell for:
Light Touch
Dynamic 2-point discrimination

A

Meissner’s corposcule

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15
Q

Cell for:
Vibration
Deep pressure

A

Pacinian corpuscules

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16
Q

Cell for:
Sustained pressure
Hot temperature

A

ADD

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17
Q

Cell for:
Cold temperature

A

Bulb of Kraus

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18
Q

What are the 5 layers of the epidermis

A

Mneumonic: ‘’ Come Lets Get Sun Burned’’

Stratum corneum
Stratum lucidum
Stratum granulosum
Stratum spinosum
Stratum basale

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19
Q

What are the 2 layers of the dermis

A
  • papillary layer
  • reticular layer
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20
Q

What type of collagen is primarily found in the papillary and reticular layers of the dermis

A

Papillary: type 3
Reticular: type 1

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21
Q

What is the primary type of collagen found in bones

A

Type 1

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22
Q

What is the primary type of collagen found in tendons

A

Type 1

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23
Q

What is the primary type of collagen found in hyalin cartillage

A

Type 2

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24
Q

What is the primary type of collagen found in arteries

A

Type 3

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25
What is the ratio of type 1:3 collagen in the adult skin
4:1
26
What is the ratio of type 1:3 collagen in hypertrophic scars And what is its composition
2:1 Well-organized collagen fibers with myofibroblast
27
What are common areas for hypertrophic scarring
Areas of high tension: knees, ankles
28
What is the ratio of type 1:3 collagen in keloids and what is its composition
3:1 Disorganized hypocellular, hick collagen fibers, no myofibroblasts
29
Common area for keloids
Ches, earlobs, upper arms
30
What is the ratio of type 1:3 collagen in feotal wounds
1:3
31
What is the precursor of tropoelastin
Elastin
32
What cells confer stretch and elastic recoil to the skin
Elastin
33
What are the types of receptors found in the skin
- mechanoreceptors - thermoreceptors - chemoreceptors - nociceptors
34
List 5 skin changes that occur with aging
- flatenning of dermal-epidermal junction - less layers of keratinocytes - decreased melanocyte density - decreased number of Langerhans cells - dermal atrophy - enlargement of fibroblasts - increase in ground substance - increase in tyep 3 collagen - decreased elastic fibers ADD JANIS P. 219 PICTURE
35
Actinic changes (photoaging) is clinically evidenced by?
Rhytids Increased skin laxity Pigmentary mottling
36
What is the pathophysiology of Ehlers-Danlos Syndrome
abnormal collagen cross linking
37
What are some clinical findings of Ehlers-Danlos?
- hypermobile joints - thin, friable skin - redundant perioccular skin (epicanthal folds, wide nasal bridge) - increased predisposition to hypertrophic scarring - POOR wound healing
38
What are findings of Cutis Laxa (Elastolysis)
- hypoelastic skin - premature aging - NORMAL wound healing - ventral hernias - cardiopulmonary issues - GI issues
39
What are findings of Pseudoxanthoma Elasticum
- **cobblestone yellowish plaques** - calcification and degeneration of elastic fibers - NORMAL wound healing - occular problems - cardiac anomalies
40
What are findings of Progeria (Hutchinson-Gilford Syndrome)
- autosomal recessive - **premature aging** - poor wound healing - baldness - increased skin laxity - loss of subcutaneous fat - growth retardation - premature death - craniosynostosis - micrognathia - prominent ears
41
What are findings of Werner's Syndrome
- autosomal recessive - premature aging - **scleroderma-like skin** - hyper/hypo pigmentation of skin - microangiopathy - diabetes - cataract - high pitched voice
42
Mechanism of action of silicone for scars
Hydration and occlusion leading to decreased fibroblast activity Scar warming leading to increased collagenase activity
43
Injectable projects for scar treatment, + 1 side effect for each
Corticosteroid (depigmentation) 5-Fluorouracil (ulceration) Bleomycin (skin and pulmonary fibrosis) Fat grafting
44
Keloid recurrence rate after 1) Excision alone 2) excision + steroid injection 3) Excision + postop radiation therapy
1) Excision alone: 50-80% 2) excision + steroid injection: 40-50% 3) Excision + postop radiation therapy: 10-30%
45
What is the radiation protocol for keloid scars
Three fractions starting post-op day 2 with a total dose of 15-30 Gy
46
Mechanism of allograft
Gets vascularized and is then rejected day 10
47
Phases of skin graft take
1) Imbibition 24-48h 2) Inosculation 48-72h 3) Revascularisation 4-6 days
48
Common causes fo skin graft failure
Shear Fluid collection: seroma/hematoma Infection Inadequate perfusion from recipient site
49
Explain primary contracture of skin graft
immediate contracture Results of retained elastin in dermis FTSG
50
Explain secondary contracture of skin grafts
Contraction in healed grafts Result of myofibroblast activity STSG
51
Adv and disadv of STSG
Adv: Higher survival Reharvest Greater availability of donor Disadv: Secondary contracture Poor color/texture match Hair growth not possible
52
Adv and disadv of FTSG
Adv: Can close donor site Better color/texture match More robust reinnervation Disadv: Lower survival Need to close donor site limits size of harvest
53
Time to repeithialize donors of STSG
14-21 days
54
What is the most common form of skin cancer
Basal cell carcinoma
55
What is the most common malignancy of the eyelid
BCC
56
What is the predominant location of basal cel carcinomas
80% of BCCs are in the head and neck
57
Name 5 risk factors for basal cell carcinoma
- Sun exposure (#1) - Fitzpatrick skin type - Advanced age - Immunosupression (AIDS, organ transplant) - Arsenic - Hydrocarbons - Genetic mutations (PTCH and p53)
58
What are the genetic mutations associated with BCC
- PTCH (patch gene codes for SHH pathway) - p53 gene - p-450 and glutathione (in truncal BCC)
59
What is the pathogenesis of albinism
Defective production of melanin from tyrosine Type 1: tyrosinase-related oculocutaneous albinism with AFFECTED tyrosinase activity Type 2: tyrosinase-related oculocutaneous albinism with NORMAL tyrosinase activity
60
What is the inheritance pattern of Gorlin Syndrome (Nevoid basal cell syndrome)
Autosomal dominant 9q22.3-q31
61
What are findings associated with Gorlin's Syndrome (Nevoid basal cell syndrome)
** Dx need 2 major or 1 major and 2 minors** Major: - multiple BCCs (>2 or 1 BCC before 20yrs old) - odontogenic keratocysts of the jaw histologically proven - >3 palmar and plantar pits - calcification of falx cerebri - bifid ribs - 1st degree relative Minor - cleft lip/palate - macrocephalie - hypertelorism - broad nasal root - pectus excavatum - scoliose - tumeur ovaire ou médulloblastome
62
What is the inheritance pattern of Xeroderma Pigmentosum
Autosomal recessive
63
List 6 features of xeroderma pigmentosum
Dentition anormale arthralgies retard cognitif télangiectasies photosensibilité multiple epithelial malignancies
64
Name the syndrome/disease - impaired DNA repair - intolerance to UV radiation - multiple epithelial malignancies
altération dans la réparation de l'ADN entrainant intolérance aux rayon UV
65
What is the risk of malignant transformation of Nevus sebaceus of Jadassohn
10-15% risk of malignant transformation to BCC
66
Nevus sebaceus of Jadassohn predisposes to which type of malignancy
BCC
67
Name the lesion - present at birth on scalp or face - well circumscribed - hairless yellow-ish plaque - verrucous and nodular transformation in puberty - malignant risk of 10-15%
Nevus sebaceus of Jadassohn
68
List features of Nevus sebaceous of Jadassohn
- present at birth on scalp or face - well circumscribed - hairless yellow-ish plaque - verrucous and nodular transformation in puberty - malignant risk of BCC of 10-15%
69
What % of BCCs will recur within 5 years
30-50%
70
Define 7 criteria for high versus low risk BCC
Locations: Low risk: <2cm thrunk and extremities Hight risk: >=2cm trunk and extremities and any size for head, nack, hands, pretibial and anogenital area Poorly (high) versus well (low) defined borders Recurrrent (high) versus primary (low) lesion +/- immunosuprression Site of prior RT Subtypes: aggressive growth pattern (high) vs nodular or superficial (low) Perineural involvement
71
What is the overall metastasis risk of basal cell carcinoma
<0.1%
72
What are the main sites of metastasis for BCCs
- lymph nodes - lungs - bones
73
Name the 6 types of BCC Which is most common and most agressive
- nodular (50-60%) - nodular ulcerative - spreading - ulcerative - infiltrative - pigmented - morpheaform or sclerosing (most agressive) ADD PHOTO JANIS P.256
74
Where do basal cell carcinomas originate from
Pluripotent epithelial cells of the epidermis and hair follicles at the dermoepithelial junction
75
What is the most common histologic type of BCC
Nodular (50-60%)
76
What is the most aggressive form of BCC
Mopheaform (high incidence of +ve margins after excision)
77
What is the goal of treatment in management of cutaneous malignancies
Cure tumor with preservation of function and cosmesis
78
List 6 non-surgical treatment options for BCC
- Radiation - Imiquimod - 5 F-U - CO2 laser - Photodynamic therapy (PDT) - Cryosurgery *superficial therapies are reserved for patients who are not candidates for surgery or radiation*
79
List 3 surgical treatment options for BCC
- Curretage and electrodesiccation (for low risk) - Mohs surgery - Surgical excision with margins (Gold standard)
80
% des BCC qui sont histologiquement mixte
40%
81
Name 4 indications for Mohs surgery in the context of BCC
- reccurent tumor - cosmetically sensitive area (nose, eyelid, periauricular) - aggressive BCC (morpheaform, micronodular) - poorly differenciated margins - other tumors (ie. SCC with perineural invasion, dermatofibrosarcoma, microcystic adnexal carcinoma)
82
List 2 adjuvant therapies for management of BCC
- Radiation - Vismodegib (SHH pathway inhibitor) - last resort, surtout si métastase
83
List adverse events associated with Vismodegib
- Muscle spasms - Alopecia - Loss of taste - Weight loss - Fatigue
84
What is the cure rate of BCC using Mohs
99% cure rate
85
What are the recommended margins for BCC
4mm (small, face and other low risk) **10mm high risk (large, trunk and extremities)
86
What is the cure rate of BCC treated with C&E
Overall 74% 96-100% for tumors <2mm
87
What are the contra-indications to cryosurgery in BCC management
- tumors deeper than 3mm - cold intolerance - morpheaform type - recurrent BCC - cosmetically sensitive area
88
What should you warm your patients about when using cryosurgery to treat BCCs
- prolonged edema (4-6 weeks) - permanant pigment loss
89
What are the indications for radiation in patients with BCCs
- non surgical candidate - >60 yrs old - tumor up to 15mm in high risk location - tumor up to 20mm in intermediate risk location
90
What are the limitations of punch biopsies
Cannot assess lesion dept beyond the punch
91
Why are shave biopsies not recommended in the context of melanomas
Cannot assess lesion dept at all
92
In which layer of the skin is melanoma in situ confined to
Basal Layer of Epidermis
93
What is the margin of choice for a melanoma in situ of the face
5mm
94
Name 3 considerations in the long term management of Gorlins
- Frequent physicals exam - Nicotinamide (B3) - Sun protection
95
Pour quelle pathologie cutanée la radiotx est-elle contre-indiquée et pourquoi?
syndrome de Gorlin Radiotx met plus à risque de cancer de peau
96
What is a sebaceous nevus
Naevus of Jadassohn Hamartomatous sebaceous glands proliferation Most commonly on scalp Present at birth
97
What is the expected evolution of neavus sebaceous during childhood
Growth proportional to child development until adolescence. Turns into a veracious lesion in adolescence
98
Name 4 different diagnosis to neavus sebaceous
Epidermal naevus Sebaceous naevus Linear psoriasis Sebaceous carcinoma Cutis aplasia
99
What is the most frequent malignant transformation of sebaceous naevus
BCC
100
Name 2 structures that can lead to malignant transformation
- Apocrine glands - Eccrine glands - Hair follicles
101
Name 4 risk factors for Merkel carcinoma
UV radiation Immunosupression Advanged age Merkel cell polyomavirus infection
102
Describe Merkel carcinoma lesions characteristics
Painless Firm Shinny Flesh colored or erythematous nodule Rapidly growing
103
What is the most appropriate treatment option for Merkel carcinoma
1-2cm Down to fascia Sentinel lymph node biopsy
104
What adjuvant therapy should be used in the management of Merkels carcinoma
Radiation therapy (proven to reduce recurrence rates)
105
Name 2 imaging modalities that you would use to stage Merkel carcinoma
PET-Scan TAP-Scan +/- MRI
106
Where do SCC arise from
Cellules malpighienne de la membrane basale de l’épiderme
107
Types of SCC
Verrucous Ulcerative Marjolins ulcer Subungeal
108
Lifetitme risk of malignant degeneration of burn scar
Marjolain Ulcer: 2%
109
Name 8 risk factors for squamous cell carcinoma
Fitzpatrick Type 1-2 Sun exposure HPV HSV Immunosuppression Prior radiation Chronic wound Advanced age
110
Name characteristics of low risk (LR), high risk (HR) and very high risk (VHR) SCC
## Footnote NCCN 2024
111
5 Pre-malignant lesion of SCC and % of malignant transformation of known
Actinic keratosis (1-10%) Maladie de Bowen (10%) Leukoplakia (15%) Érythroplasie de Queyrat (30%) Keratoacanthoma Papulose bowenoid ## Footnote Leukoplakie: patche blanchâtre a/n muqueuse orale Queyrat: Bowen génital ou oral Bowen: SCC in situ
112
What is Bowens disease of penis glans, vulva or oral mucosa, what is its % of malignant transformation
Erythroplasia of Queyrat 30%
113
Name 2 premalignant lesions associated with SCC of the lip
Leucoplakia (15% malignant transformation) Actinic Cheilitis
114
Areas of metastasis of SCC
Lungs Liver Brain Skin Bone
115
Name 2 elements on pathologie that would increase the risk of reccurence of SCC
-Adenoide, adenosquameux ou carcinosarcomateux -Dept >2mm -Perineural invasion -Lympho-vascular invasion -Poor differenciation
116
Medical treatment of SCC
Radiation Brachytherapy Oral retinoids Topical 5-FU and imiquimod (pre malignant or SCC in situ) Chemotherapy (carbotaxol or cisplatin) Immunotherapy (cemiplamab or pembrolizumab)
117
Treatment of low risk SCC
* Wide local excision with 4-6mm margins (if positive margins must do Mohs) * Mohs * Radiaton therapy (if patient refuses surgery)
118
Treatment of HR and VHR SCC
* Consider SLNB if multiple high risk features or if recurrent * Mohs * Standard excision wit "wider" surgical margins * RT for non surgical candidates
119
2 Indications for SLNB SCC
Recurrent OR Multiple HR features
120
Management d’un SCC sous-unguéal
Amputation à l’IPD
121
FU pour un SCC maladie localisée (NCCN 2024)
Low Risk: q3-12mois x 2 ans puis q6-12 mois x 3 ans puis q1 an à vie high risk: q3-6mois x 2 ans puis q6-12 mois x 3 ans puis q1 an à vie very high risk: q3-6mois x 2 ans puis q6 mois x 3 ans puis q6-12 mois à vie
122
FU pour SCC avec maladie régionale (NCCN 2024)
q2-3mois x 1 an puis q2-4 mois x 1 an puis q4-6 mois pour 3 ans puis q6-12 mois à vie imagerie (IRM) au besoin si e/p n'est pas suffisant pour suivre l'étendu de la maladie ou si risque de récurrence gg
123
Melanoma risk factors (NCCN 2024)
Age > 50 Homme **Prédisposition phénotypique** Nevus dysplasique Atypical mole syndrome Fitzpatrick 1-2 Cheveux yeux, yeux bleus **Histoire personnelle** Coup de soleil multiple Lésion pré-maligne: KA, NMSC Immunosuppression (VIH, transplant) Xeroderma pigmentosum **Prédisposition génétique** Histoire familiale positive Mutations: BRAC, PTEN, MITF **Facteurs environnementaux** Utilisation de lit de bronzage Habiter dans un climat de soleil ou altitude près de l'équateur Exposition chronique au soeil
124
4 Lésions mélanocytaires pré-malignes
Névus dysplasique (10%) Mélanome in situ Lentigo malin (Hutchison freckle) (5%) Névus mélanocytaire congenital (1-10%)
125
Atypical mole syndrome criteria et risque de transfo maligne ## Footnote autres noms: B-K mole syndrome, FAMMM familial atypical multiple mole melanoma
* More than 100 nevi measuring 6-15mm * One or more measuring >8mm * One or more with atypical features ## Footnote 10% de transfo maligne
126
Types of pigmented lesion that can be confused with melanoma
Junctional nevi Compound nevi (Halo nevus is subtype) Intradermal nevi Blue Nevi Spitz nevus Lentigo Seborrheic keratosis
127
Where are melanocytes normally located
Basalis layer of epidermis
128
nommer 2 différences entre un CMN et un nevus mélanocytaire acquis
CMN: invasion du derme réticulaire profond et des annexes cutanées acquis: se limite au derme papillaire et n'envahit pas les annexes cutanées
129
Where are melanocytes located in congenital melanocytic neavus
Deep reticular dermis (can invade appendages, nerves and vessels)
130
Name 2 important prognostic findings on pathology for melanoma other than tumor depth
Ulceration Mitotic rate Microscopic satelitosis
131
Melanoma growth patterns/types
Superficial spreading melanoma Nodular Lentigo maligna Acral-lentginous melanoma Desmoplastic melanoma Amelanotic melanoma Noncutaneous melanoma Ocular melanoma
132
Precursor of lentigo maligna
Hutchinson freckle
133
Characteristic of acral melanoma
melanonychia Usually flat with irregular border Long radial growth
134
Describe T1-T4 for melanoma
## Footnote nombre de mitose n'est plus important
135
Name 4 risk factors for malignant transformation in CMN (transformation to melanoma)
3 or more nevi Location on the trunk >20cm contour irrégulier | aussi jeune âge 3-5 ans ? ## Footnote janis 2022
136
Name 2 characteristics of neurocutaneous melanosis
CNS involvement >2 small or 1 large
137
qu'est-ce que la neuromélanose cutanée
invasion mélanocytaire des méninges et du parenchyme cérébral associé à un CMN
138
7 caractéristiques cliniques d'un CMN qui se transforme en mélanome
■ Rapid increase in size ■ Irregularity of border ■ Development of asymmetry ■ Variation of color within the nevus ■ Development of satellite lesions ■ Changes in texture ■ Bleeding, pain, itching
139
What is the preferred diagnosis modality for neurocutaneous melanosis
MRI ( at 4 months of age avant que le cerveau soit myélinisé pour bien voir les dépôt de mélanine)
140
Name 2 reasons why we usually proceed with surgical excision of neavus sebaceous
Aesthetic consideration (cause of alopecia) Risk of malignant transformation
141
Name 3 possible tumoral transformations of sebaceous neavus
Sebaceous carcinoma SCC BCC (most common) Microcystic adnexal carcinoma
142
Name 3 reconstruction options for a posterior scalp defect that cannot be closed primarily
2nd intension Skin graft Tissue expansion Local rotation flap
143
What is the significant of a melanoma recurrent a few cm away from the primary lesion?
Metastasis in transit
144
Name 3 possible DDx for a cutaneous lesion on the finger with bony erosion?
Osteomyelitis Marjolin ulcer BCC Osteosarcome
145
What are 2 cutaneous findings associated with Gorlins other than BCCs
- palmar or plantar pits - facial milia - epidermal cysts
146
Name 2 squeletal findings associated with Gorlins
- calcification of falx cerebri - bifid ribs - scoliosis - syndactylie - sprengel deformity (scapula)
147
What is the % risk of malignant transformation in congenital melanocytic nevi
small = 1 % giant = 5-10%
148
Nommer 5 indications d'IRM pour les CMN
3 CMN ou plus 1 GCMN CMN du midline CMN au visage Sx neuro ## Footnote c'est indications correspondent aussi aux facteurs de risques de mélanose neurocutanée
149
Name 2 surgical considerations in the management of CMN
- Serial excisions (depending on size) - Excision at young age (before school age)
150
nommer 5 arguments contre l'excision d'un CMN
* Anesthésie générale délétère avant 1 an * Diminue pas le risque de mélanome (n'est pas restreint à la lésion) * Risque de déclencher transfo maligne * Impossible à exciser complètement * Peut pâlir avec les années
151
Name 4 risk factors for malignant lentigo
- Fitspatrick 1-2 - Sun exposure - Advanced age - Familial hx
152
Name 2 ways to have better margins during ressection for malignant lentigo
- Woods lamp - dermoscopie
153
Why are frozen sections not adequate in the context of melanoma
car détruit l'architecture cellulaire
154
Name 3 conditions associated with congenital melanocytic nevis
- neurocutaneous melanosis - malignant melanoma - neurofibromatosis (GCMN) - spina bifida (GCMN) - myelomeningocele (GCMN)
155
List 3 surgical treatment options for congenital melanocytic nevi
- Serial excision - Tissue expansion - Skin graft
156
Name a long term sequellae of congenital melanocytic nevi
- Hypertrophic scarring - Malignant transformation
157
Mutations in melanoma
BRAF, cKIT, NRAS
158
Syndromes qui augmentent le risqué de mélanome?
FAMMM/BK Mole Albinisme CMN, neuromélanose XP BRCA PTEN
159
Resection margins for melanoma
In situ: 0.5-1cm B <1mm: 1cm B 1-2mm: 1-2cm B 2-4 mm: 2cm B > 4mm: 2cm
160
Elements to look for in melanoma biopsy, which are the 3 most important prognostic factors
Breslow (+) Ulceration status (+) Dermal mitotic rate (+) Deep and peripheral margin status Microsatellitosis Desmoplasia Lymphovascular/perineural invasion
161
Melanoma workup
Pour Stade I-II, aucun labo ou imagerie nécessaire Pour Stade III+ (N>1+), labo de base, hépatique, LDH, CXR, CT poumon, CT abdo-pelvien, PET
162
5 DDx of mélanonychie
* Hematome sous-unguéal * Onychomycose * Mélanome in situ * Mélanome invasif * Hyperplasie mélanocytaire
163
Resection for subungeal melanoma
Amputation proximal to distal interphalangeal joint ## Footnote except for in situ, possibilité d'être plus conservateur
164
Indication for SLNB in melanoma
<0.8mm with ulceration OR >0.8 with/without ulceration ## Footnote dès stade IB (aka T1b ou T2a)
165
Indication for Therapeutic lymph node dissection melanoma
* ganglion cliniquement positif * SLNB + ET patient incapable d'adhérés aux suivis recommendés * SLNB + ET fardeau tumoral entrainant une prédiction élevé d'avoir d'autres gg + ## Footnote NCCN 2024: il n'est plus indiqué de faire une dissection axillaire simplement pour un SNLB positif
166
Thérapies adjuvantes pour le mélanome
Immunothérapie : Nivolumab Pembrolizumab XR Interféron (?) Chimiothérapie ## Footnote si BRAF +: Dabrafenib/trametinib
167
Melanoma follow-up
**in situ**: H/P q1an **Stade IA -IIA** H/P q6-12 mois x 5 ans puis q 1 an **Stade IIB-IV** H/P q3-6 mois x 2 ans puis q3012 mois x 3 ans puis q1 an Pour screen une récurrence ou métastase: Considérer imagerie q3-6 mois x 2 ans puis q 6-12 mois x 3 ans puis seulement pour sx spécifiques ## Footnote imagerie à considérer pour investiguer sx spécifique
168
Qu’est-ce que le Syndrome LEOPARD
Lentigines EKG anomalies Ocular hypertelorism Pulmonary stenosis Abnormal genitals Retarded growth Deaf
169
List 5 DDx for bullous lesions
- bullous pemphygoid - cicatricial pemphygoid - Epidermolysis bullosa - S/TEN - erythema multiform - graft vs host disease - Staphyloccocal scaled skin syndrome
170
Name 4 types of Epidermolysis bullosa
- simplex - junctional - dysmorphic - Kindler syndrome
171
Name 3 findings associated with dystrophic type Epidermolysis bullosa
- pseudosyndactylie - ungeal dystrophy - alopecia - dental caries - corneal ulcers - renal insufficiency - 50% risk of SCC by age 30
172
What is the risk of SCC in patients with dystrophic epidermolysis bullosa
50% by age 30
173
Describe the management components of dystrophic epidermolysis bullosa
- avoid mechanical trauma - infection prevention - non adhenrant dressings - debride bullaes - avoid chronic topical antibiotics - frequent physical exams - multidisciplinary approach
174
What are features of erythema multiforme
- precipitated by HSV - target lesions - non painful - usually on palmes and soles
175
What are the 2 types of erythema multiforme
Minor: no mucosal or systemic involvement Major: mucosal and systemic involvement
176
What other consultant should you ask for in patients with erythema multiforme
ophtalmology
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What is the management of erythema multiforme
- Minor - supportive treatment - More severe - antiviral +/- immunosupression
178
What is the pathophysiology of Staphylococcal Scalded Skin Syndrome
- Seperation of desmosomes found int he granulosum layer of the epidermis (desmoglein-1) - Hematogenous dissemination of exfoliative toxins
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What are clinical findings of SSSS
- **SPARING OF MUCOUS MEMBRANES** - malaise - fever - Nikolsky + - irritability - pain - affects whole body (head to toes)
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What is the expected evolution of SSSS
Resolution within 1-2 weeks
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What is the management of SSSS
IV antibiotics (b-lactamase + clindamycin)
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What is the mortality rate associated with SSSS
4% children 60% adults
183
Melanoma with Breslow 11 on thigh. What are your margins?
2cm
184
What are two important technical points of a biopsy for melanoma
- orientation of specimen - excision down to fascia
185
What is your management of a melanoma lesion (list 4)
- WLE with 2cm margins - Sentinel lymph node biopsy - Linear reconstruction or STSG - Dr. B to confirm
186
What are your margins for a 1.5cm SCC lesion of the cheek
6mm
187
What are 3 important things to check on physical exam in the context of SCC
- Evaluation of lesion characteristics - Adherence of the lesion to deep plains - Regional lymph node palpation - Cranial nerve exam - Laxity of surrounding for reconstruction
188
Draw a limberg
Add picture in brainscape
189
What are 3 things on path report that are rick factors of recurrence for SCC
- Perineural invasion - Clark 4 or more - Poor differentiation - Lymphatic or vascular involvement - Desmoplastic histology
190
What is the name of a SCC that arises from a chronic wound?
Marjolin ulcer
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What are the surgical margins for marjolin ulcers
2cm
192
You are in OR and frozen sections are not available. What do you do with the deficit?
STSG
193
Name 2 functional complications of cervico-facial flap with reconstruct a cheek deficit and 2 ways to mitigate this
Ectropion - anchor down to periosteum - canthopexy - Frost sutures - Tenzel modification
194
Classification of CMN ?
195
Pathologies associated with GCMN?
- NF - Spina bifida - Myelomeningocele
196
Most frequent localisation of CMN?
Trunk > Extremities > H&N
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What is a late CMN?
Absent at birth, but appears < 2 years of age
198
What it neurocutaneous melanosis?
Melanocytic proliferation within the brain parenchyma + GCMN (or > 2 smaller melanocytic nevi) Risks factors: - >20 satellite nevi - CMN in a midline location over trunk or calvaria
199
Sx of neurocutaneous melanosis?
Can be Asx (50%) Usually symptomatic by 2 years of age Increased ICP (lethargy, headache, vomiting, photophobia) and result in hydrocephalus, seizures, developmental delay , cranial nerve palsies, bladder and bowel dysfunction
200
Surgical treatments for CMN?
Serial excision (< 3 excisions) Tissue Expansion STSG/FTSG FLAP
201
What is a Nevus of Ota?
blue-brown unilateral periocular macula (areas follow V1-V2) At birth or around puberty Asians and Black especially
202
What is a Nevus of Ito?
Same as Nevus of Ota but areas innervated by posterior supraclavicular and lateral cutaneous brachial nerves
203
List 5 features of LEOPARD syndrome
ADD
204
Name 4 syndromes/conditions that increase the risk of melanoma
Atypical mole syndrome Neuromelanosis Albinism Xeroderma pigmentosum
205
What are the 3 most important prognosis factors for melanoma
Breslow thickness Ulceration Mitotic rate
206
Name 4 tumors associated with Gorlins
- BCC - Medulloblasoma - Meningioma - Fibrosarcoma - Rhabdosarcoma
207
Describe classic appearance of BCC
Well defined borders, flesh-colored, pearly nodule, ovelying telangiesttasia
208
List 4 agressive subtypes f BCC
Micronodular Basosquamous Sclerosing/Morpheaforme Infiltratif
209
Name 4 genetic syndromes with higher risk of developping SCC
ADD
210
Name 4 genetic syndromes with higher risk of developping BCC
Épidermolyse buleuse XP Albinisme Anémie de Fanconi Werner syndrome
211
Name 4 genetic syndromes with higher risk of developing melanoma
FAMMM Li-Fraumeni Xeroderma Pigmentosum Cowden Syndrome Albinism Neuromelanosis
212
Risk factors for hydradenitis suppurativa?
- Obesity - Afro American - Family Hx - Woman - Tobacco
213
Which type of gland is involved in HS ?
- Apocrine glands
214
What is the classification of HS? (with their associated tx?)
Hurley Classification : I: Simple, single of multiple abcesses tx: Deroofing, topical clindamycin x 12 weeks, intralesional kenalog, consider monthly Nd-YAG or CO2 laser II:lesions widely separated, single or multiple recurrent abcesses tx: Deroofing, Oral clinda + rifampin x 10 weeks consider monthly Nd-YAG III: Diffuse involvement of affected region, multiple interconnected tracts and abscesses tx: wide surgical excision
215
Non surgical tx of HS?
- Diet - Weight loss - Hygiene - Smoking cessation - ATB - Anti-TNF, Anti- IL1 - Laser, steroid injection - XRT
216
Gold standard tx of HS?
WLE 1-2 cm margin
217
What is the reccurence rate of HS after WLE?
30%
218
# Criteria for neurofibromatosis grade 1 What are the criteria for neurofibromatosis type 1
Must have 2 or more of the following features * 6 or more cafe au lait macules (over 5mm in diameter prepubertal) (over 15 mm in diameter postpubertal) * Two or more neurrofibromas orr one plexiform neurrofibroma * Frreckling int he axillary or inguinal regions * Optic glioma * Two or more Lisch nodules (iris hamartomas) * Distinctive dosseous lesion: sphenoid dysplasia, thining of a long bone cortex, pseudoarthrosis Firrst degree relative with NF1
219
Gene implicated in NF type 1 + inheritance pattern
Damage to chromosone 17q11.2 Autosomal dominant
220
Other name for NF type 1
Von Recklinghausen disease
221
Where is located (at the cellular level) a neurofibroma
In peripheral nerve seath
222
Risk of malignant peripheral sheath tumor in neurofibromatosis
10%
223
Criteria for NF type 2
Vestibular schwannomas (acoustic neuromas) in both ears OR Vestibular schwannoma in one ear and one or several first-degree relatives with NF2
224
Do NF type 2 also have skin ttumors
Yes but much less extensive than NF type 1
225
Dermatofibrosarcome protuberans (DFSP), clincial aspect of lesion
nodulaire, couleur peau, violacé, kéloide
226
DFSP localisation
Tronc > extrémités > H&N (50-40-10%)
227
Risk factor (4) for DFSP
Burn Tattoo Radiation therapy Surgical incision
228
DFSP tx + margins
Marge 3-4cm, incluant le fascia superficiel vs Mohs
229
5 year survival rate des mélanome selon le stade
ADD