Peau et oncologie cutanée Flashcards
What are the main functions of the skin (4)
- Protection (UV, mechanical, chemical, thermal)
- Immunologic (barrier to microorganisms)
- Metabolic (vitamin D synthesis)
- Thermoregulation
What is the embryological origin of the epidermis
Ectoderm
What is the embryological origin of the dermis
Mesoderm
What is the embryological origin of melanocytes
Neural crest
What is the embryological origin of Langerhans cells
Mesenchymal origin
How long does it take for keratinocyte differentiation to occur
28-45 days from basal layer to cornified layer
What 4 cells are found in the epidermis
- Keratinocytes
- Melanocytes
- Langerhans cells
- Merkel cells
What 3 cells are found in the dermis
- Fibroblasts
- Macrophages
- Mast cells
aussi occasionnellement: lymphocytes
What appendage cells are found in the epidermis
None
What appendage cells are found in the dermis
- Hair follicles
- Sebacious glands
- Eccrine and Appocrine sweat glands
- Meissner’s corpuscules
- Pacinian corpuscules
- Ruffini endings
- Bulb of Krause
What is the function of eccrine glands
Thermoregulation
What is the function of apporcine glands
Sweat
What is the function of Langerhans cells
Immunity (antigen response)
Cell for:
Light Touch
Dynamic 2-point discrimination
Meissner’s corposcule
Cell for:
Vibration
Deep pressure
Pacinian corpuscules
Cell for:
Sustained pressure
Hot temperature
ADD
Cell for:
Cold temperature
Bulb of Kraus
What are the 5 layers of the epidermis
Mneumonic: ‘’ Come Lets Get Sun Burned’’
Stratum corneum
Stratum lucidum
Stratum granulosum
Stratum spinosum
Stratum basale
What are the 2 layers of the dermis
- papillary layer
- reticular layer
What type of collagen is primarily found in the papillary and reticular layers of the dermis
Papillary: type 3
Reticular: type 1
What is the primary type of collagen found in bones
Type 1
What is the primary type of collagen found in tendons
Type 1
What is the primary type of collagen found in hyalin cartillage
Type 2
What is the primary type of collagen found in arteries
Type 3
What is the ratio of type 1:3 collagen in the adult skin
4:1
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
What are common areas for hypertrophic scarring
Areas of high tension: knees, ankles
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
Common area for keloids
Ches, earlobs, upper arms
What is the ratio of type 1:3 collagen in feotal wounds
1:3
What is the precursor of tropoelastin
Elastin
What cells confer stretch and elastic recoil to the skin
Elastin
What are the types of receptors found in the skin
- mechanoreceptors
- thermoreceptors
- chemoreceptors
- nociceptors
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
Actinic changes (photoaging) is clinically evidenced by?
Rhytids
Increased skin laxity
Pigmentary mottling
What is the pathophysiology of Ehlers-Danlos Syndrome
abnormal collagen cross linking
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
What are findings of Cutis Laxa (Elastolysis)
- hypoelastic skin
- premature aging
- NORMAL wound healing
- ventral hernias
- cardiopulmonary issues
- GI issues
What are findings of Pseudoxanthoma Elasticum
- cobblestone yellowish plaques
- calcification and degeneration of elastic fibers
- NORMAL wound healing
- occular problems
- cardiac anomalies
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
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
Mechanism of action of silicone for scars
Hydration and occlusion leading to decreased fibroblast activity
Scar warming leading to increased collagenase activity
Injectable projects for scar treatment, + 1 side effect for each
Corticosteroid (depigmentation)
5-Fluorouracil (ulceration)
Bleomycin (skin and pulmonary fibrosis)
Fat grafting
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%
What is the radiation protocol for keloid scars
Three fractions starting post-op day 2 with a total dose of 15-30 Gy
Mechanism of allograft
Gets vascularized and is then rejected day 10
Phases of skin graft take
1) Imbibition 24-48h
2) Inosculation 48-72h
3) Revascularisation 4-6 days
Common causes fo skin graft failure
Shear
Fluid collection: seroma/hematoma
Infection
Inadequate perfusion from recipient site
Explain primary contracture of skin graft
immediate contracture
Results of retained elastin in dermis
FTSG
Explain secondary contracture of skin grafts
Contraction in healed grafts
Result of myofibroblast activity
STSG
Adv and disadv of STSG
Adv:
Higher survival
Reharvest
Greater availability of donor
Disadv:
Secondary contracture
Poor color/texture match
Hair growth not possible
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
Time to repeithialize donors of STSG
14-21 days
What is the most common form of skin cancer
Basal cell carcinoma
What is the most common malignancy of the eyelid
BCC
What is the predominant location of basal cel carcinomas
80% of BCCs are in the head and neck
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)
What are the genetic mutations associated with BCC
- PTCH (patch gene codes for SHH pathway)
- p53 gene
- p-450 and glutathione (in truncal BCC)
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
What is the inheritance pattern of Gorlin Syndrome (Nevoid basal cell syndrome)
Autosomal dominant
9q22.3-q31
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
What is the inheritance pattern of Xeroderma Pigmentosum
Autosomal recessive
List 6 features of xeroderma pigmentosum
Dentition anormale
arthralgies
retard cognitif
télangiectasies
photosensibilité
multiple epithelial malignancies
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
What is the risk of malignant transformation of Nevus sebaceus of Jadassohn
10-15% risk of malignant transformation to BCC
Nevus sebaceus of Jadassohn predisposes to which type of malignancy
BCC
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
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%
What % of BCCs will recur within 5 years
30-50%
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
What is the overall metastasis risk of basal cell carcinoma
<0.1%
What are the main sites of metastasis for BCCs
- lymph nodes
- lungs
- bones
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
Where do basal cell carcinomas originate from
Pluripotent epithelial cells of the epidermis and hair follicles at the dermoepithelial junction
What is the most common histologic type of BCC
Nodular (50-60%)
What is the most aggressive form of BCC
Mopheaform (high incidence of +ve margins after excision)
What is the goal of treatment in management of cutaneous malignancies
Cure tumor with preservation of function and cosmesis
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
List 3 surgical treatment options for BCC
- Curretage and electrodesiccation (for low risk)
- Mohs surgery
- Surgical excision with margins (Gold standard)
% des BCC qui sont histologiquement mixte
40%
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)
List 2 adjuvant therapies for management of BCC
- Radiation
- Vismodegib (SHH pathway inhibitor) - last resort, surtout si métastase
List adverse events associated with Vismodegib
- Muscle spasms
- Alopecia
- Loss of taste
- Weight loss
- Fatigue
What is the cure rate of BCC using Mohs
99% cure rate
What are the recommended margins for BCC
4mm (small, face and other low risk)
**10mm high risk (large, trunk and extremities)
What is the cure rate of BCC treated with C&E
Overall 74%
96-100% for tumors <2mm
What are the contra-indications to cryosurgery in BCC management
- tumors deeper than 3mm
- cold intolerance
- morpheaform type
- recurrent BCC
- cosmetically sensitive area
What should you warm your patients about when using cryosurgery to treat BCCs
- prolonged edema (4-6 weeks)
- permanant pigment loss
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
What are the limitations of punch biopsies
Cannot assess lesion dept beyond the punch
Why are shave biopsies not recommended in the context of melanomas
Cannot assess lesion dept at all
In which layer of the skin is melanoma in situ confined to
Basal Layer of Epidermis
What is the margin of choice for a melanoma in situ of the face
5mm
Name 3 considerations in the long term management of Gorlins
- Frequent physicals exam
- Nicotinamide (B3)
- Sun protection
Pour quelle pathologie cutanée la radiotx est-elle contre-indiquée et pourquoi?
syndrome de Gorlin
Radiotx met plus à risque de cancer de peau
What is a sebaceous nevus
Naevus of Jadassohn
Hamartomatous sebaceous glands proliferation
Most commonly on scalp
Present at birth
What is the expected evolution of neavus sebaceous during childhood
Growth proportional to child development until adolescence. Turns into a veracious lesion in adolescence
Name 4 different diagnosis to neavus sebaceous
Epidermal naevus
Sebaceous naevus
Linear psoriasis
Sebaceous carcinoma
Cutis aplasia
What is the most frequent malignant transformation of sebaceous naevus
BCC
Name 2 structures that can lead to malignant transformation
- Apocrine glands
- Eccrine glands
- Hair follicles
Name 4 risk factors for Merkel carcinoma
UV radiation
Immunosupression
Advanged age
Merkel cell polyomavirus infection
Describe Merkel carcinoma lesions characteristics
Painless
Firm
Shinny
Flesh colored or erythematous nodule
Rapidly growing
What is the most appropriate treatment option for Merkel carcinoma
1-2cm
Down to fascia
Sentinel lymph node biopsy
What adjuvant therapy should be used in the management of Merkels carcinoma
Radiation therapy
(proven to reduce recurrence rates)
Name 2 imaging modalities that you would use to stage Merkel carcinoma
PET-Scan
TAP-Scan
+/- MRI
Where do SCC arise from
Cellules malpighienne de la membrane basale de l’épiderme
Types of SCC
Verrucous
Ulcerative
Marjolins ulcer
Subungeal
Lifetitme risk of malignant degeneration of burn scar
Marjolain Ulcer: 2%
Name 8 risk factors for squamous cell carcinoma
Fitzpatrick Type 1-2
Sun exposure
HPV
HSV
Immunosuppression
Prior radiation
Chronic wound
Advanced age
Name characteristics of low risk (LR), high risk (HR) and very high risk (VHR) SCC
NCCN 2024
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
Leukoplakie: patche blanchâtre a/n muqueuse orale
Queyrat: Bowen génital ou oral
Bowen: SCC in situ
What is Bowens disease of penis glans, vulva or oral mucosa, what is its % of malignant transformation
Erythroplasia of Queyrat
30%
Name 2 premalignant lesions associated with SCC of the lip
Leucoplakia (15% malignant transformation)
Actinic Cheilitis
Areas of metastasis of SCC
Lungs
Liver
Brain
Skin
Bone
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
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)
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)
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
2 Indications for SLNB SCC
Recurrent
OR
Multiple HR features
Management d’un SCC sous-unguéal
Amputation à l’IPD
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
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
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
4 Lésions mélanocytaires pré-malignes
Névus dysplasique (10%)
Mélanome in situ
Lentigo malin (Hutchison freckle) (5%)
Névus mélanocytaire congenital (1-10%)
Atypical mole syndrome criteria et risque de transfo maligne
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
10% de transfo maligne
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
Where are melanocytes normally located
Basalis layer of epidermis
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
Where are melanocytes located in congenital melanocytic neavus
Deep reticular dermis (can invade appendages, nerves and vessels)
Name 2 important prognostic findings on pathology for melanoma other than tumor depth
Ulceration
Mitotic rate
Microscopic satelitosis
Melanoma growth patterns/types
Superficial spreading melanoma
Nodular
Lentigo maligna
Acral-lentginous melanoma
Desmoplastic melanoma
Amelanotic melanoma
Noncutaneous melanoma
Ocular melanoma
Precursor of lentigo maligna
Hutchinson freckle
Characteristic of acral melanoma
melanonychia
Usually flat with irregular border
Long radial growth
Describe T1-T4 for melanoma
nombre de mitose n’est plus important
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 ?
janis 2022
Name 2 characteristics of neurocutaneous melanosis
CNS involvement
>2 small or 1 large
qu’est-ce que la neuromélanose cutanée
invasion mélanocytaire des méninges et du parenchyme cérébral associé à un CMN
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
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)
Name 2 reasons why we usually proceed with surgical excision of neavus sebaceous
Aesthetic consideration (cause of alopecia)
Risk of malignant transformation
Name 3 possible tumoral transformations of sebaceous neavus
Sebaceous carcinoma
SCC
BCC (most common)
Microcystic adnexal carcinoma
Name 3 reconstruction options for a posterior scalp defect that cannot be closed primarily
2nd intension
Skin graft
Tissue expansion
Local rotation flap
What is the significant of a melanoma recurrent a few cm away from the primary lesion?
Metastasis in transit
Name 3 possible DDx for a cutaneous lesion on the finger with bony erosion?
Osteomyelitis
Marjolin ulcer
BCC
Osteosarcome
What are 2 cutaneous findings associated with Gorlins other than BCCs
- palmar or plantar pits
- facial milia
- epidermal cysts
Name 2 squeletal findings associated with Gorlins
- calcification of falx cerebri
- bifid ribs
- scoliosis
- syndactylie
- sprengel deformity (scapula)
What is the % risk of malignant transformation in congenital melanocytic nevi
small = 1 %
giant = 5-10%
Nommer 5 indications d’IRM pour les CMN
3 CMN ou plus
1 GCMN
CMN du midline
CMN au visage
Sx neuro
c’est indications correspondent aussi aux facteurs de risques de mélanose neurocutanée
Name 2 surgical considerations in the management of CMN
- Serial excisions (depending on size)
- Excision at young age (before school age)
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
Name 4 risk factors for malignant lentigo
- Fitspatrick 1-2
- Sun exposure
- Advanced age
- Familial hx
Name 2 ways to have better margins during ressection for malignant lentigo
- Woods lamp
- dermoscopie
Why are frozen sections not adequate in the context of melanoma
car détruit l’architecture cellulaire
Name 3 conditions associated with congenital melanocytic nevis
- neurocutaneous melanosis
- malignant melanoma
- neurofibromatosis (GCMN)
- spina bifida (GCMN)
- myelomeningocele (GCMN)
List 3 surgical treatment options for congenital melanocytic nevi
- Serial excision
- Tissue expansion
- Skin graft
Name a long term sequellae of congenital melanocytic nevi
- Hypertrophic scarring
- Malignant transformation
Mutations in melanoma
BRAF, cKIT, NRAS
Syndromes qui augmentent le risqué de mélanome?
FAMMM/BK Mole
Albinisme
CMN, neuromélanose
XP
BRCA
PTEN
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
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
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
5 DDx of mélanonychie
- Hematome sous-unguéal
- Onychomycose
- Mélanome in situ
- Mélanome invasif
- Hyperplasie mélanocytaire
Resection for subungeal melanoma
Amputation proximal to distal interphalangeal joint
except for in situ, possibilité d’être plus conservateur
Indication for SLNB in melanoma
<0.8mm with ulceration
OR
>0.8 with/without ulceration
dès stade IB (aka T1b ou T2a)
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 +
NCCN 2024: il n’est plus indiqué de faire une dissection axillaire simplement pour un SNLB positif
Thérapies adjuvantes pour le mélanome
Immunothérapie : Nivolumab
Pembrolizumab
XR
Interféron (?)
Chimiothérapie
si BRAF +: Dabrafenib/trametinib
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
imagerie à considérer pour investiguer sx spécifique
Qu’est-ce que le Syndrome LEOPARD
Lentigines
EKG anomalies
Ocular hypertelorism
Pulmonary stenosis
Abnormal genitals
Retarded growth
Deaf
List 5 DDx for bullous lesions
- bullous pemphygoid
- cicatricial pemphygoid
- Epidermolysis bullosa
- S/TEN
- erythema multiform
- graft vs host disease
- Staphyloccocal scaled skin syndrome
Name 4 types of Epidermolysis bullosa
- simplex
- junctional
- dysmorphic
- Kindler syndrome
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
What is the risk of SCC in patients with dystrophic epidermolysis bullosa
50% by age 30
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
What are features of erythema multiforme
- precipitated by HSV
- target lesions
- non painful
- usually on palmes and soles
What are the 2 types of erythema multiforme
Minor: no mucosal or systemic involvement
Major: mucosal and systemic involvement
What other consultant should you ask for in patients with erythema multiforme
ophtalmology
What is the management of erythema multiforme
- Minor - supportive treatment
- More severe - antiviral +/- immunosupression
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
What are clinical findings of SSSS
- SPARING OF MUCOUS MEMBRANES
- malaise
- fever
- Nikolsky +
- irritability
- pain
- affects whole body (head to toes)
What is the expected evolution of SSSS
Resolution within 1-2 weeks
What is the management of SSSS
IV antibiotics (b-lactamase + clindamycin)
What is the mortality rate associated with SSSS
4% children
60% adults
Melanoma with Breslow 11 on thigh. What are your margins?
2cm
What are two important technical points of a biopsy for melanoma
- orientation of specimen
- excision down to fascia
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
What are your margins for a 1.5cm SCC lesion of the cheek
6mm
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
Draw a limberg
Add picture in brainscape
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
What is the name of a SCC that arises from a chronic wound?
Marjolin ulcer
What are the surgical margins for marjolin ulcers
2cm
You are in OR and frozen sections are not available. What do you do with the deficit?
STSG
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
Classification of CMN ?
Pathologies associated with GCMN?
- NF
- Spina bifida
- Myelomeningocele
Most frequent localisation of CMN?
Trunk > Extremities > H&N
What is a late CMN?
Absent at birth, but appears < 2 years of age
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
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
Surgical treatments for CMN?
Serial excision (< 3 excisions)
Tissue Expansion
STSG/FTSG
FLAP
What is a Nevus of Ota?
blue-brown unilateral periocular macula (areas follow V1-V2)
At birth or around puberty
Asians and Black especially
What is a Nevus of Ito?
Same as Nevus of Ota but areas innervated by posterior supraclavicular and lateral cutaneous brachial nerves
List 5 features of LEOPARD syndrome
ADD
Name 4 syndromes/conditions that increase the risk of melanoma
Atypical mole syndrome
Neuromelanosis
Albinism
Xeroderma pigmentosum
What are the 3 most important prognosis factors for melanoma
Breslow thickness
Ulceration
Mitotic rate
Name 4 tumors associated with Gorlins
- BCC
- Medulloblasoma
- Meningioma
- Fibrosarcoma
- Rhabdosarcoma
Describe classic appearance of BCC
Well defined borders, flesh-colored, pearly nodule, ovelying telangiesttasia
List 4 agressive subtypes f BCC
Micronodular
Basosquamous
Sclerosing/Morpheaforme
Infiltratif
Name 4 genetic syndromes with higher risk of developping SCC
ADD
Name 4 genetic syndromes with higher risk of developping BCC
Épidermolyse buleuse
XP
Albinisme
Anémie de Fanconi
Werner syndrome
Name 4 genetic syndromes with higher risk of developing melanoma
FAMMM
Li-Fraumeni
Xeroderma Pigmentosum
Cowden Syndrome
Albinism
Neuromelanosis
Risk factors for hydradenitis suppurativa?
- Obesity
- Afro American
- Family Hx
- Woman
- Tobacco
Which type of gland is involved in HS ?
- Apocrine glands
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
Non surgical tx of HS?
- Diet
- Weight loss
- Hygiene
- Smoking cessation
- ATB
- Anti-TNF, Anti- IL1
- Laser, steroid injection
- XRT
Gold standard tx of HS?
WLE 1-2 cm margin
What is the reccurence rate of HS after WLE?
30%
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
Gene implicated in NF type 1 + inheritance pattern
Damage to chromosone 17q11.2
Autosomal dominant
Other name for NF type 1
Von Recklinghausen disease
Where is located (at the cellular level) a neurofibroma
In peripheral nerve seath
Risk of malignant peripheral sheath tumor in neurofibromatosis
10%
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
Do NF type 2 also have skin ttumors
Yes but much less extensive than NF type 1
Dermatofibrosarcome protuberans (DFSP), clincial aspect of lesion
nodulaire, couleur peau, violacé, kéloide
DFSP localisation
Tronc > extrémités > H&N (50-40-10%)
Risk factor (4) for DFSP
Burn
Tattoo
Radiation therapy
Surgical incision
DFSP tx + margins
Marge 3-4cm, incluant le fascia superficiel vs Mohs
5 year survival rate des mélanome selon le stade
ADD