Disease Profiles Flashcards

(461 cards)

1
Q

Psoriasis

A

Chronic inflammatory dermatosis

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2
Q

Psoriasis: Sex incidence

A

Equal

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3
Q

Psoriasis: Peaks in incidence

A

20s and 50s

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4
Q

Psoriasis: Risk Co-morbidities (6)

A

Psoriatic Arthritis
Metabolic Syndrome
Crohn’s Disease
Cancer
Depression
Uveitis

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5
Q

Psoriasis: Increased risk of what?

A

Cardiovascular MI

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6
Q

Psoriasis: Drugs that precipitate Psoriasis (4)

A

Beta blockers
Lithium
Anti-malarial drugs
Swift withdrawal of topical or systemic steroids

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7
Q

Psoriasis: What cytokines are involved? (3)

A

TNF-Alpha
IL-17
IL-23

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8
Q

Psoriasis: Types (4)

A

Psoriasis vulgaris
Guttate
Palmoplantar Pustular
Erythrodermic pustular

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9
Q

Psoriasis: Koebner phenomenon

A

New lesions arise at the sites of trauma

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10
Q

Psoriasis: Pathological changes

A

Epidermal hyperplasia

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11
Q

Psoriasis: Epidermal Hyperplasia

A

Increased epidermal turnover

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12
Q

Psoriasis: What is the name for the chronic plaques?

A

Psoriasis vulgaris

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13
Q

Psoriasis: Pathology - Initiating event

A

Keratinocytes under stress release factors that stimulate plasmacytoid dendritic cells to produce IFN-alpha, IL-1beta, IL-6 and TNF

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14
Q

Psoriasis: Pathology - Chemical signals activate what cells?

A

Dendritic cells

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15
Q

Psoriasis: Pathology - What happens to dendritic cells?

A

Migrate to the lymph nodes and present to and activate TH1 and TH17 cells

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16
Q

Psoriasis: Pathology - What reaction occurs in the dermis?

A

T cells stimulate an inflammatory cascade involving anti-microbial peptide release and neutrophil attracting chemokines

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17
Q

Psoriasis: Pathology - What enables the formation of munro micro abscesses?

A

Complement attracting neutrophils to the keratin layer

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18
Q

Psoriasis: Pathology - Complement attracting neutrophils in the keratin layer enable the formation of what?

A

Munro micro abscesses

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19
Q

Psoriasis: Pathology - What CD cells are involved?

A

CD8+

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20
Q

Psoriasis: Pathology - Function of dermal fibroblasts

A

Release keratinocytes and epidermal growth factors

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21
Q

Psoriasis: Pathology - What occurs to keratinocytes?

A

Proliferation

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22
Q

Psoriasis: Typical clinical presentation

A

Symmetrically distributed red scaly plaques with well defined edges

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23
Q

Psoriasis: Location of plaques

A

Extensors - Elbow and Knee
Scalp
Sacrum
Hands, Feet and Nails
Trunk

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24
Q

Psoriasis: How does it present on dark skin?

A

More white with silver scale

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25
Psoriasis: Scratching can lead to what?
Lichenification
26
Psoriasis: Auspitz Sign
Removal of surface scale reveals tiny dilated capillaries in elongated dermal papillae that bleed
27
Psoriasis: Nail Disease Changes seen (3)
Nailbed pitting Onycholysis Subungual Hyperkeratosis
28
Psoriasis: Nail bed pitting
Superficial depressions in the nailbed
29
Psoriasis: Onycholysis
Separation of the nail plate from the nail bed
30
Psoriasis: Subungual hyperkeratosis
Thickening of the nail bed
31
Psoriasis: Most common type
Psoriasis Vulgaris
32
Psoriasis: Psoriasis Vulgaris
Symmetrical plaques on the extensor surfaces of the limbs, scalp and lower back
33
Psoriasis: Flexural Psoriasis
Smooth erythematous plaques without scale in flexures and skin folds that are colonised by candida yeast
34
Psoriasis: Guttate psoriasis
Mutliple small tear-drop shaped erythematous plaques on the trunk after a streptococcal infection in young adults
35
Psoriasis: Pustular psoriasis
Multiple petechiae and pustules on the palms and soles
36
Psoriasis: Generalised Erythrodermic Psoriasis
Rare and serious form characterised by erythroderma and systemic illness
37
Psoriasis: Unstable plaque psoriasis
Rapid extension of existing or new plaques induced by infection, stress, drugs or drug withdrawal
38
Psoriasis: Sebopsoriasis
Overlap of seborrheic dermatitis and psoriasis affecting the scalp, face, ears, chest due to colonisation by malassezia
39
Psoriasis: Palmoplantar Psoriasis
Psoriasis of the palms and soles with keratoderma and fissuring
40
Psoriasis: What investigation can aid diagnosis?
Biopsy
41
Psoriasis: Histology - Epidermis appearance
Thickened epidermis
42
Psoriasis: Histology - Impact on the keratin layer
Increased keratin content
43
Psoriasis: Histology - Impact on keratinocytes
Parakeratosis - retention of nuclei in keratinocytes due to rapid and abnormal differentiation of keratinocytes
44
Psoriasis: Histology - What occurs in the upper epidermis?
Accumulation of neutrophils to form micro-abscesses
45
Psoriasis: Histology - What occurs in the dermis?
Elongated Rete pegs - project into the dermis
46
Psoriasis: Management - Examples of Vitamin D Analogues (2)
Caclipotriol Calcitriol
47
Psoriasis: Management - Vitamin D Analogues - What is used for localised plaques?
Caclipotriol
48
Psoriasis: Management - Vitamin D Analogues - What is used for flexures?
Calcitriol
49
Psoriasis: Management - First line management
Potential corticosteroid + topical vitamin D + Emollient
50
Psoriasis: Management - Treatment for scalp psoriasis
Greasy ointments to soften scale Steroids Vitamin D analogues
51
Psoriasis: Management - Treatment for Psoriasis of the Axilla
Topical steroids for the face, flexures and groin Calcineurin Inhibitors
52
Atopic Dermatitis
Itchy skin lesions
53
Atopic Dermatitis: Most common in what population?
Children
54
Atopic Dermatitis: Genetic predisposition
Mutations within the fillagrin gene impairs the skin barrier function
55
Atopic Dermatitis: Immunopathology - Initiating factor
Langerhans cells in the epidermis process the antigen
56
Atopic Dermatitis: Immunopathology - Processed antigen is presented to what by Langerhans cells?
T Helper Cells
57
Atopic Dermatitis: Immunopathology - Sensitised T Helper Cells have what action?
Migrate into lymphatics and then to regional nodes where antigen presentation is amplified
58
Atopic Dermatitis: Immunopathology - What is the final stage that causes the dermatitis?
Antigen challenges cause T cell proliferation that migrate and infiltrate the skin
59
Atopic Dermatitis: Acute - Presentatio of lesiosn
Papulovesicular erythematous lesions with oedema Scaling and crusting may be present
60
Atopic Dermatitis: Acute -Dyshidriotic eczema
Spongiosis coalesces into vesicles or bullae
61
Atopic Dermatitis: Acute - What histological feature is present in the upper dermis?
Inflammatory infiltrate
62
Atopic Dermatitis: Acute - Spongiosis description
Fluid accumulates around the keratinocytes
63
Atopic Dermatitis: Acute - What can develop from spongiosis?
Blister or vesicle development
64
Atopic Dermatitis: What does crust indicate?
Staphylococcus aureus
65
Atopic Dermatitis: What does eczema herpeticum indicate?
Herpes simplex virus infection
66
Atopic Dermatitis: Clinical presentation of Eczema Herpeticum
Monomorphic punched out lesions
67
Atopic Dermatitis: Presentation of Adult Eczema
Generalised dryness and itching with a primary manifestation of hand eczema
68
Atopic Dermatitis: Presentation of Childhood Eczema
Predominantly flexural eczema
69
Atopic Dermatitis: Presentation of Infantile Eczema
Eczema primarily involving the face, scalp and extensor surfaces of the limbs
70
Atopic Dermatitis: Contact Allergic Dermatitis - What type of reaction is this?
Type IV Hypersensitivity reaction
71
Atopic Dermatitis: Contact Allergic Dermatitis - Example of triggers (4)
Nickel Chemicals Topical therapies Plants
72
Atopic Dermatitis: Contact Allergic Dermatitis - Routes of exposure (3)
Direct skin contact Airborne contact Injection
73
Atopic Dermatitis: Contact Allergic Dermatitis - Sensitisation Phase Description
Generation of memory T cells following exposure to an antigen via Langerhan cells in the epidermis
74
Atopic Dermatitis: Contact Allergic Dermatitis - Allergic Phase Description
Activated of sensitised Th cells in response to an antigen, causing activation of cell-mediated cytotoxicity and release of inflammatory cytokines
75
Atopic Dermatitis: Contact Allergic Dermatitis - Investigation
Patch Testing
76
Atopic Dermatitis: Contact Allergic Dermatitis - Description of Patch Testing
Allergens prepared on a FinnChamber which are applied on the back and removed after 48 hours - the results are read between 48-96 hours
77
Atopic Dermatitis: Contact Irritant Dermatitis
Non-specific physical irritation due to e.g. soap, cleaning products and nappy rash
78
Atopic Dermatitis: Atopic Eczema - 3 causative factors
Reduced skin barrier function Environment Immunological changes
79
Atopic Dermatitis: Atopic Eczema - What is fillagrin?
A filament aggregating protein
80
Atopic Dermatitis: Atopic Eczema - Function of fillagrin protein?
Reduces AMP in the skin
81
Atopic Dermatitis: Atopic Eczema - What cells are involved?
TH2 cells Dendritic Cells Keratinocytes Macrophages Mast Cells
82
Atopic Dermatitis: Atopic Eczema - Typical Presentation
Ill-defined erythema with scaling with dry skin
83
Atopic Dermatitis: Atopic Eczema - Location
Flexors
84
Atopic Dermatitis: Atopic Eczema - Associated with what other diseases? (3)
Asthma Allergic Rhinitis Food Allergy
85
Atopic Dermatitis: Atopic Eczema - What is the presentation of this in black individuals?
Nodular pruigo - well-defined lichenification with itchy nodules
86
Atopic Dermatitis: Atopic Eczema - Diagnostic Criteria is 3 or more of what? (5)
Visible flexural rash - cheeks and extensors in infants History of flexural rash - cheeks and extensors in infants Personal history of atopy - of first degree relative if under 4 Generally dry skin Onset before the age of 2
87
Atopic Dermatitis: Drug Related Eczema - what type of reactions are these?
Type I or IV hypersensitivity reactions
88
Atopic Dermatitis: Photo-induced Eczema - Cause
Reaction to UV light Secondary to photosensitising drugs
89
Atopic Dermatitis: Photo-induced Eczema - Presentation
Well defined eczema e.g. cuff of collar
90
Atopic Dermatitis: Lichen Simplex
Chronic skin condition as a result of repetitive scratching
91
Atopic Dermatitis: Stasis Dermatitis
Eczema induced by physical trauma to the skin due to venous insufficiency - increases the hydrostatic pressure of the blood
92
Atopic Dermatitis: Stasis Dermatitis - where does this affect?
Lower legs
93
Atopic Dermatitis: Discoid Eczema
Eczema that occurs in circular or oval patches - often due to infection
94
Atopic Dermatitis: Seborrheic Eczema - Alternate name
Cradle cap
95
Atopic Dermatitis: Seborrheic Eczema - What areas are affected?
Nose Eyebrows Ears Scalp
96
Atopic Dermatitis: Dyshidriotic Eczema
Sudden acute flare up of eczema in which spongiotic vesicles join together
97
Atopic Dermatitis: Dyshidriotic Eczema - Presentation
Tiny blisters form on the hands, side of the fingers and feet that are severely itchy
98
Atopic Dermatitis: General Management
Remove triggers or irritants Emollients
99
Atopic Dermatitis: Management of Mild Eczema
Topical steroid
100
Atopic Dermatitis: Management of Moderate Eczema
Moderate topical steroid - Bethamethasone valerate or Clobetasone butyrate Use mild steroid in face area
101
Atopic Dermatitis: Management of Severe Eczema
Potent topical steroid - Bethamethasone valerate Reduced potency on sensitive areas
102
Atopic Dermatitis: Dupixent Mechanism of Action
Blocks Type II IL-4 and IL-13 receptors
103
Atopic Dermatitis: Lebrikzumab Mechanism of Action
Blocks IL-13
104
Atopic Dermatitis: Tralokinumab Mechanism of Action
Blocks IL-13
105
Atopic Dermatitis: Pascolinumab Mechanism of Action
Blocks IL-4 (ineffective as IL-13 also needs to be blocked)
106
Haploinsufficiency
Only one copy of the gene is working causing a reduced protein production
107
Tuberous Sclerosis
Autosomal dominant condition of benign tumours in organ systems of the body
108
Tuberous Sclerosis: What genetic pattern is observed?
Autosomal dominant
109
Tuberous Sclerosis: What chromosomes are affected? (2)
9q34 16p13.3
110
Tuberous Sclerosis: What genes are involved?
TSC1 and TSC2
111
Tuberous Sclerosis: What do TSC1 and 2 code for?
Tubers and Hamartin - tumour regulating genes
112
Tuberous Sclerosis: Penetrance
High
113
Tuberous Sclerosis: How may this present in infants?
Infantile seizures
114
Tuberous Sclerosis: Earliest cutaneous sign
Ash-leaf Macule - depigmented macules can be observed by Woods Lamp
115
Tuberous Sclerosis: Presentation on nails (2)
Periungual Fibromata Longitudinal ridging
116
Tuberous Sclerosis: Presentation on the skin (2)
Shagreen patch - leather texture Facial angiofibroma
117
Tuberous Sclerosis: Presentation in teeth
Enamel pitting
118
Tuberous Sclerosis: What may occur to the falx cerebri?
Cortical tubers or calcification - may induce seizures
119
Tuberous Sclerosis: Impact on the organs
Angiomyolipomas - Heart, Lungs and Kidneys
120
Tuberous Sclerosis: What may be seen on X-ray?
Bone Cysts
121
Epidermolysis Bullosa
Group of inherited disorders with blister formation in response to mechanical trauma
122
Epidermolysis Bullosa: How many genes are involved?
10
123
Epidermolysis Bullosa: What type of condition is this?
Autoimmune
124
Epidermolysis Bullosa: Examples of genes involved? (4)
Keratin 5 and 14 Laminin Integrins Collagen 17
125
Epidermolysis Bullosa: 3 Types
Simplex Junctional Dystrophic
126
Epidermolysis Bullosa: Keratin - Type I Keratins
K9-K20
127
Epidermolysis Bullosa: Keratin - Type II Keratins
K1-K8
128
Epidermolysis Bullosa: Simplex - What layer is affected?
Epidermal layer affected
129
Epidermolysis Bullosa: Junctional - What layer is affected?
Dermoepithelial layer affected
130
Epidermolysis Bullosa: Dystrophic - What layer is affected?
Upper dermis affected
131
Epidermolysis Bullosa: Clinical presentation
Skin fragility Blistering at birth with skin loss
132
Epidermolysis Bullosa: Investigation
Skin Biopsy
133
Neurofibromatosis
Genetic condition that causes tumours along the nervous system
134
Neurofibromatosis: Aetiology
Mutations in the NF1 gene
135
Neurofibromatosis: What signs are shown in the skin? (2)
Cafe au lait macules - more than 5 Axillary or inguinal freckles
136
Neurofibromatosis: What tumour presents?
Neurofibromas
137
Neurofibromatosis: What signs are observed in the eyes? (2)
Optic glioma >2 Lisch Nodules - present on the iris
138
Necrotising Fasciitis
Rapidly progressive infection resulting in extensive necrosis of the superficial fascia and overlying subcutaneous fat that can develop into a life-threatening condition
139
Necrotising Fasciitis: Type I causative organisms
Mixed anaerobes Coli forms
140
Necrotising Fasciitis: When does Type I typically occur?
Post-abdominal surgery
141
Necrotising Fasciitis: Type II Causative Organisms
Group A streptococcus infection
142
Necrotising Fasciitis: Risk Factors (4)
Immunosuppressed Obesity PWID Peripheral arterial disease
143
Necrotising Fasciitis: Systemic Symptoms (3)
Fever Chills Altered mental state
144
Necrotising Fasciitis: Skin manifestation (2)
Manifests as suspected cellulitis - diffuse erythema that doesn't respond to antibiotic therapy Purple skin discolourisation
145
Necrotising Fasciitis: Investigation
Microbiology - blood culture with gram staining and cultures from deep tissue
146
Necrotising Fasciitis: Management
Surgical debridement and antibiotics
147
Acne Vulgaris
Inflammatory condition of the pilosebaceous unit
148
Acne Vulgaris: Typical age in Females
14-17
149
Acne Vulgaris: Associated with what disorders? (2)
Endocrine disorders - PCOS and Hyperandrogenism
150
Acne Vulgaris: Typical age in Males
16-19
151
Acne Vulgaris: What are the potential triggering events for this? (2)
Increased androgens at puberty Patients have increased androgen sensitivity of the sebaceous glands
152
Acne Vulgaris: What event induces plugging of the pilosebaceous units?
Hypercornification causes keratin plugging of the pilosebaceous units
153
Acne Vulgaris: What produces comodones?
Keratin and sebum build up
154
Acne Vulgaris: What can increase sebum production?
Androgens
155
Acne Vulgaris: What can rupture cause?
Acute inflammation and foreign body granulomas to produce inflammatory lesions - papules, pustules, cysts and nodules
156
Acne Vulgaris: Distribution
Sebaceous gland sites - Face, Upper back and Anterior chest
157
Acne Vulgaris: What is a comodone?
White and blackheads
158
Acne Vulgaris: What is the difference between white heads and black heads?
Black heads have oxidised pus
159
Acne Vulgaris: Complications of Chronic Acne (2)
Atrophic scares - ice pick scars or hypertrophic keloid scars Skin hyperpigmentation
160
Acne Vulgaris: Mild grading
Scattered papules and pustules with comedones
161
Acne Vulgaris: Moderate grading
Numerous papules, pustules and mild atrophic scarring
162
Acne Vulgaris: Severe grading
Cysts, nodules and significant scarring
163
Acne Vulgaris: Aim of treatment
Control and prevent scarring
164
Acne Vulgaris: Topical treatment options (3)
Benzoyl peroxide Topical vitamin A derivatives - Retinoids Topical antibiotics
165
Acne Vulgaris: Action of Benzoyl Peroxide (2)
Keratolytic Anti-bacterial
166
Acne Vulgaris: Action of Vitamin A Derivatives
Drying effect
167
Acne Vulgaris: Action of Vitamin A Derivatives
Drying effect
168
Acne Vulgaris: Systemic Treatment Options (2)
Antibiotics - for at least 6 months Isotretinoin
169
Acne Vulgaris: Effect of Isotretinoin
Effects sebaceous gland activity
170
Acne Vulgaris: Initial management
Oral Antibiotic or Topical Retinoid
171
Acne Vulgaris: Second Line Management
Oral Isotretinoin - must be hospital only prescribing
172
Acne Vulgaris: Effect of oral isotretinoin
Usually causes an initial flare up for 2-3 weeks then steadily improves at 16 weeks
173
Acne Vulgaris: Contraindication of Isotretinoin
Pregnancy - causes congenital defects
174
Acne Vulgaris: What can antibiotics be combined with to reduced antimicrobial resistance? (3)
Benzoyl peroxide Retinoids Zinc
175
Acne Vulgaris: Examples of Retinoids (3)
Adapalene Isotretinoin Tretinoin
176
Acne Vulgaris: If there is a poor response to topical treatment what is administered to those under 12?
Erythromycin or Clarithromycin
177
Acne Vulgaris: If there is a poor response to topical treatment what is administered to those over 12?
Lymecycline, Doxycycline or Erythromycin
178
Rosaecea
Condition in which the facial blood vessels dilate to produce a flushed appearance of the cheeks and nose
179
Rosaecea: Peak Age
30-60 years
180
Rosaecea: Sex epidemiology
More common in females
181
Rosaecea: Presentation of rash
Vascular ectasia Patchy inflammation with plasma cells Pustules and Papules Erythema
182
Rosaecea: Triggers
Alcohol Heat Spicy food Stress Sunlight
183
Rosaecea: Location
Nose Chin Cheeks Forehead Spares the naso-labial folds
184
Rosaecea: Impact on the nose
Rhinophyma - enlarged unshapely nose due to thickening of skin
185
Rosaecea: Impact on the eyes
Conjunctivitis
186
Rosaecea: First line treatment
Topical Metronidazole
187
Rosaecea: Action of Ivermectin
Reduce the demodex mite
188
Rosaecea: Second Line Management
Topical Metronidazole + Oral Tetracycline
189
Rosaecea: Second Line Management for severe cases
Low dose Isotretinoin
190
Rosaecea: Management of Telangiectasia
Vascular laser
191
Lichen Planus
Rash impacting the skin causing swelling and irritation
192
Lichen Planus: Age epidemiology
Mainly impacts middle aged individuals
193
Lichen Planus: Immunopathology
T-cell mediated inflammation to an unknown protein in the skin and mucosal keratinocytes
194
Lichen Planus: Characterised by damage to what?
The basal epidermis
195
Lichen Planus: Associated with what virus?
Hepatitis C
196
Lichen Planus: Diagnostic Test
Biopsy - useful to differentiate SCC and Lichen Planus
197
Lichen Planus: Histological features (5)
Irregular sawtooth acanthosis Hypergranulosis Orthohyperkeratosis Band-like upper dermal infiltrate of lymphocytes Basal damage with formation of Cytoid bodies
198
Lichen Planus: Duration
12-18 months
199
Lichen Planus: Nail presentation
Nail ridges
200
Lichen Planus: Location
Volar wrists and forearms Shins Ankles
201
Lichen Planus: Wickhams Striae
Fine lace-like pattern on the surface of papules and buccal mucosa
202
Lichen Planus: Presentation
Itchy Violaceous pink or purple flat-topped shiny papules
203
Lichen Planus: Management
Topical or Oral Steroids with Emollients
204
Lichen Planus: Must check for what?
Drug precipitant - can cause lichenoid drug eruption
205
Bullous Disorder
Autoimmune diseases where damage to adhesion mechanisms in the skin result in blistering
206
Bullous Pemphigoid
Sub-epidermal blister caused by an autoimmune reaction
207
Bullous Pemphigoid: Typical age
>60 years
208
Bullous Pemphigoid: Main pathophysiological mechanism
Anti-hemidesmosome antibodies react with major or minor antigens of the hemidesmosomes anchoring basal cells to the basement membrane
209
Bullous Pemphigoid: What type of hypersensitivity reaction is this?
Type II
210
Bullous Pemphigoid: What type of antibodies are anti-hemidesmosome antibodies?
IgG
211
Bullous Pemphigoid: Antigen-Antibody complex results in what?
Complement activation and tissue damage
212
Bullous Pemphigoid: Complement activation and tissue damage causes what to occur?
Interruption of the dermo-epidermal junction and formation of the sub-epidermal blister
213
Bullous Pemphigoid: Diagnostic Test
Biopsy - With Immunofluorescence and Histology
214
Bullous Pemphigoid: Immunological findings
Linear IgG and complement deposited around the basement membrane
215
Bullous Pemphigoid: Histological findings
Subepidermal blisters and inflammatory infiltrates (mainly eosinophils) within the blister
216
Bullous Pemphigoid: What forms the roof of the blister?
Epidermis
217
Bullous Pemphigoid: What happens to older lesions on histology?
Shows re-epithelialisation of the floor to mimic pemphigus vulgarus
218
Bullous Pemphigoid: Distribution
Localised to one area or widespread on the trunk and proximal limbs
219
Bullous Pemphigoid: Morphology of bullae
Large tense bullae on the erythematous base
220
Bullous Pemphigoid: What happens when blisters burst?
Leaves erosions
221
Bullous Pemphigoid: Is it scarring?
No
222
Bullous Pemphigoid: Early in disease presentation
Urticated itchy plaques
223
Bullous Pemphigoid: Nilkolsky sign
Negative
224
Nilkolsky Sign
Top layers of the skin slip away from the lower layers when rubbed
225
Bullous Pemphigoid: Diagnosis
Skin Biopsy with Immunofluorescence studies
226
Bullous Pemphigoid: Treatment for local disease
High potency topical steroids
227
Bullous Pemphigoid: Treatment for systemic disease
Oral steroids +/- Tetracycline Antibiotics +/- antihistamines
228
Bullous Pemphigoid: What if there is no response to treatment?
Administer immunosuppression - Azathioprine or Methotrexate
229
Pemphigus Vulgaris
Autoimmune bullous disease
230
Most common subtype of the pemphigus group?
Pemphigus vulgaris
231
Pemphigus Vulgaris: Sex incidence
Equal
232
Pemphigus Vulgaris: Age presentation
Middle age - 40-60
233
Pemphigus Vulgaris: What type of reaction is this?
Type II Hypersensitivity
234
Pemphigus Vulgaris: Mechanism of pathophysiology
IgG antibodies against desmoglein 3 - this is responsible for maintaining the desmosomal attachments
235
Pemphigus Vulgaris: What occurs once antigen-antibody complexes are generated?
Complement activation and protease release causes disruption to desmosomes to cause acantholysis
236
Pemphigus Vulgaris: Clinical presentation
Fluid-filled blisters that rupture to form shallow erosions
237
Pemphigus Vulgaris: What process is involved?
Pacantholysis
238
Pemphigus Vulgaris: Pacantholysis definition
Lysis of intercellular adhesion sites
239
Pemphigus Vulgaris: Has no involvement with what cells?
Basal cells
240
Pemphigus Vulgaris: There is a loss in the integrity of what?
Epidermal cell adhesion
241
Pemphigus Vulgaris: Distribution
Scalp Axilla Face Groin
242
Pemphigus Vulgaris: Splits through what?
Intra-epidermal space
243
Pemphigus Vulgaris: Nikolsky sign
Positive
244
Pemphigus Vulgaris: Mucosal involvement effects what areas?
Eyes Genitals
245
Pemphigus Vulgaris: Diagnosis tool
Skin biopsy with immunofluorescence
246
Pemphigus Vulgaris: Hallmark on Biopsy
Chicken wire deposition of IgG within the epidermis
247
Pemphigus Vulgaris: Management of local disease
Topical steroids and anaesthetics
248
Pemphigus Vulgaris: Management of systemic disease
High dose oral steroids with immunosuppression +/- Rituximab
249
Pemphigus Vulgaris: How long does remission take with treatment?
3-6 months
250
Dermatitis Herpetiformis
Autoimmune bullous disorder associated with coeliac disease
251
Dermatitis Herpetiformis: Strong association with what disease?
Coeliac Disease
252
Dermatitis Herpetiformis: 90% of patients have what disorder?
Gluten sensitive enteropathy
253
Dermatitis Herpetiformis: Associated with what gene?
HLA-DQ2 haplotype
254
Dermatitis Herpetiformis: Peak Incidence
15-40 years
255
Dermatitis Herpetiformis: Pathophysiology
IgA antibodies target the gliadin component of gluten but cross-reacts with connective tissue matrix proteins - tissue transglutaminase
256
Dermatitis Herpetiformis: Where do the immune complexes form?
In the dermal papillae
257
Dermatitis Herpetiformis: What does immune complex formation do?
Activate complement and generates neutrophil chemotaxins to induce sub-epidermal blisters
258
Dermatitis Herpetiformis: Clinical presentation
Intensely itchy symmetrical lesions on an erythematous and swollen base followed by blisters
259
Dermatitis Herpetiformis: Locations
Elbows Knees Buttocks Scalp Shoulders
260
Dermatitis Herpetiformis: Diagnostic blood tests
Anti-TTG
261
Dermatitis Herpetiformis: Biopsy findings
Immunofluorescence showing granular IgA deposts in the dermal papillae Histology shows sub-epidermal blisters with papillary micro-abscesses
262
Dermatitis Herpetiformis: Management
Gluten free diet
263
Dermatitis Herpetiformis: Complications
Small bowel lymphoma
264
Dermatitis Herpetiformis: Hallmark
Papillary dermal microabscesses
265
Skin Cancer: Risk Factors
Sun exposure Skin type Immunosuppression - Chronic inflammatory diseases or organ transplants Environmental carcinogens
266
Skin Cancer: Genetic Susceptibility examples (4)
Xeroderma Pigmentosum Albinism Naevoid Basal Cell Carcinoma Epidermolysis Bullosa
267
Skin Cancer: What is Xeroderma Pigmentosum?
Defect in XPA-G nucleotide excision repair gene to cause skin cancer on a UV exposed site with neurological degeneration
268
Skin Cancer: What is Epidermolysis Bullosa?
Deficiency in Type VII collagen to cause skin fragility and blistering leading to chronic wounding with inflammation and scarring
269
Skin Cancer: Examples of encironmental carcinogens (5)
Ionising radiation Arsenic Coal tar Trauma Chronic wounding
270
Skin Cancer: Examples of Immunosuppression that increase the risk (4)
Organ transplant patients Haematological malignancies Inflammatory conditions receiving immunosuppressive therapy e.g. IBS, Crohns or RA HIV
271
Skin Cancer: 2 Main types
Keratinocyte skin cancer Melanoma
272
Skin Cancer: Examples of Keratinocyte Skin Cancer (4)
Basal cell carcinoma Squamous Cell Carcinoma Actinic Keratoses Bowen's Disease
273
Skin Cancer: Melanoma is in what layer?
The basal layer of the epidermis
274
Skin Cancer: What layer do basal cell carcinomas arise from?
Basal layer of the epidermis
275
Skin Cancer: What layer do the squamous cell carcinomas arise from?
Suprabasal layers
276
Skin Cancer: Most common skin cancers?
Keratinocyte
277
Skin Cancer: What % of skin cancers are BCCs?
75%
278
Skin Cancer: Basal Cell Carcinoma - Age of incidence
30-40
279
Skin Cancer: Basal Cell Carcinoma - Pathophysiology
Epidermal keratinocyte dNA is damaged by solar UV radiation to cause mutations in tumour suppressor genes, loss of apoptotic function and mutations in proto-oncogenes Clonal selection of non-apoptosing, mutated cells Solar UV suppresses normal cell-mediated immune response against tumour cells
280
Skin Cancer: Basal Cell Carcinoma - Description of growth
Slow with local destruction
281
Skin Cancer: Basal Cell Carcinoma - 4 types
Nodular Superficial Infiltrative Pigmented
282
Skin Cancer: Basal Cell Carcinoma - Description of Nodular BCC
Slow growing, shiny pearly nodule with superficial telangiectasia
283
Skin Cancer: Basal Cell Carcinoma - Location of nodular BCC
Face
284
Skin Cancer: Basal Cell Carcinoma - Name if ulcerated nodular BCC
Rodent ulcer
285
Skin Cancer: Basal Cell Carcinoma - Description of Superficial BCC
Erythematous well-demarcated scaly plaques with a slightly raised whipcord margin
286
Skin Cancer: Basal Cell Carcinoma - Superficial BCC size
Larger than 20mm at presentation
287
Skin Cancer: Basal Cell Carcinoma - Growth description for superficial BCC
Slow growth over months or years
288
Skin Cancer: Basal Cell Carcinoma - Description of Infiltrative BCC
Thickened yellowish plaques with a poorly defined margin
289
Skin Cancer: Basal Cell Carcinoma - Description of spread of infiltrative BCC
May infiltrate tissues widely and along nerves
290
Skin Cancer: Basal Cell Carcinoma - Description of Pigmented BCC
Brown, blue or greyish lesion of nodular or superficial histology
291
Skin Cancer: Basal Cell Carcinoma - Pigmented BCC more common in what patients?
Those with dark skin
292
Skin Cancer: Basal Cell Carcinoma - Pigmented BCC may resemble what?
Malignant melanoma
293
Skin Cancer: Basal Cell Carcinoma - Investigation
An incisional biopsy - must be sent to histology before treatment
294
Skin Cancer: Basal Cell Carcinoma - Treatment of choice
Wide excision with histology to ensure complete removal of the tumour with adequate margins
295
Skin Cancer: Basal Cell Carcinoma - Treatment for infiltrative BCC and tumours of the nasal creases
Mohs Microscopic Surgery
296
Skin Cancer: Basal Cell Carcinoma - Treatment options for non-surgical treatment
Cryotherapy Photodynamic therapy Topical Imiquimod
297
Skin Cancer: Basal Cell Carcinoma - Targeted treatment for advanced cancers or those unable to tolerate surgery
Vismodegib
298
Skin Cancer: Basal Cell Carcinoma - MOA of Vismodegib
Hedgehog inhibitor that binds to SMO
299
Skin Cancer: Squamous Cell Carcinoma
Malignant tumour that arises from supra-basal keratinocytes
300
Skin Cancer: Squamous Cell Carcinoma - Occasionally arises from sites of what conditions? (3)
Chronic leg ulcers Site of burns Chronic lupus vulgaris
301
Skin Cancer: Squamous Cell Carcinoma - Genetic Factors that increase the risk (3)
Fair skin type Xeroderma pigmentosum Oculocutaneous albinism
302
Skin Cancer: Squamous Cell Carcinoma - Most common skin cancer in what populations?
Immunosuppressed patients
303
Skin Cancer: Squamous Cell Carcinoma - Associated with what first line risk factor?
Lifetime cumulative UV exposure
304
Skin Cancer: Squamous Cell Carcinoma - Pattern of growth and spread
Locally invasive with low risk of metastasis
305
Skin Cancer: Squamous Cell Carcinoma - Description of presentation
Warty or hyperkeratotic crusted lump or ulcer
306
Skin Cancer: Squamous Cell Carcinoma - Arises on what skin type?
Sun-damaged
307
Skin Cancer: Squamous Cell Carcinoma - Which age range at risk?
Elderly
308
Skin Cancer: Squamous Cell Carcinoma - 90% of presentations are where?
Head Neck Hands Forearms
309
Skin Cancer: Squamous Cell Carcinoma - Growth pattern
Fast growth - may be painful or bleed
310
Skin Cancer: Squamous Cell Carcinoma - Precursor lesions
Actinic keratoses Bowen's Disease
311
Skin Cancer: Squamous Cell Carcinoma - What metastases are most common?
Loco-regional spread with nodal metastases
312
Skin Cancer: Squamous Cell Carcinoma - Management
Complete surgical excision with biopsy
313
Skin Cancer: Melanoma
Proliferation of atypical melanocytes with potential for dermal invasion and widespread metastases
314
Skin Cancer: Melanoma - Age of incidence
Increases with age
315
Skin Cancer: Melanoma - Associated with what main risk factor?
Intermittent intense sun exposure or sunburn in childhood
316
Skin Cancer: Melanoma - Risk factors (4)
Fair skin Multiple melanocytic naevi Family history of melanoma Immunosuppression
317
Skin Cancer: Melanoma - 60% have what associated genetic mutation?
Activating BRAF mutation
318
Skin Cancer: Melanoma - Acral melanomas are associated with what mutation?
c-kit
319
Skin Cancer: Melanoma - Rare genetic conditions that are associated? (2)
Xeroderma pigmentosum Oculocutaneous albinism
320
Skin Cancer: Melanoma - 4 types
Superficial spreading Acral lentiginous malignant melanoma Lentigo maligna melanoma Nodular malignant melanoma
321
Skin Cancer: Melanoma - Superficial spreading description
Large flat irregularly pigmented lesion
322
Skin Cancer: Melanoma - Superficial spreading most common sites
Trunk Limbs
323
Skin Cancer: Melanoma - Superficial spreading description of growth
Grows laterally before vertical invasion develops
324
Skin Cancer: Melanoma - Acral letiginous malignant melanoma presentation
Pigmented lesions on the palm, sole or under the nail
325
Skin Cancer: Melanoma - Lentigo maligna melanoma presentation
Invasive tumour that develops with pre-existing lentigo maligna
326
Skin Cancer: Melanoma - Lentigo maligna melanoma location
Sun-damaged face, neck or scalp
327
Skin Cancer: Melanoma - Nodular malignant melanoma location
Trunk
328
Skin Cancer: Melanoma - Nodular malignant melanoma presentation
Rapidly growing pigmented nodule that bleeds or ulcerates
329
Skin Cancer: Melanoma - RGP
Radial Growth Phase
330
Skin Cancer: Melanoma - VGP
Vertical growth phase
331
Skin Cancer: Melanoma - RGP description
Melanomas grow as macules either entirely in situ or with dermal microinvasion
332
Skin Cancer: Melanoma - VGP description
Melanoma cells invade. thedermis forming an expasnile mass with mitosis
333
Skin Cancer: Melanoma - Only what type of melanomas can metastasies?
Those in VGP
334
Skin Cancer: Melanoma - Why are nodular melanomas considered more aggressive?
They do not have an RGP
335
Skin Cancer: Melanoma - Why are melanomas more likely to metastasises than keratinocyte skin cancers?
As melanocytes are motile cells
336
Skin Cancer: Melanoma - Sites
Common on sun-exposed sites - Scalp, Face, Neck, Arm, Trunk and Leg
337
Skin Cancer: Melanoma - What appearance would suggest a melanoma?
Change in shape Irregular pigmentation Bleeding Satellite nodules Ulceration New adult pigmented lesion
338
Skin Cancer: Melanoma - First Line Management
Narrow complete excision
339
Skin Cancer: Melanoma - What is used to determine prognosis?
Breslow Criteria
340
Skin Cancer: Melanoma - Breslow Criteria
Determines the prognosis based on tumour depth
341
Skin Cancer: Melanoma - <1mm Breslow Criteria
5 year survival of 95-100%
342
Skin Cancer: Melanoma - >4mm Breslow Criteria
5 year survival of 50%
343
Skin Cancer: Melanoma - Metastasis Bresow Criteria
5 year survival of 5%
344
Skin Cancer: Melanoma - Where is the Breslow Criteria measured from?
Granular layer of the epidermis down to the deepest point of invasion
345
Skin Cancer: Melanoma - Treatment for advanced disease with c-kit mutation?
Imatinib
346
Skin Cancer: Melanoma - Treatment for advanced disease with BRAF mutations?
Debrafenib or Vemurafenib
347
Skin Cancer: Melanoma - How to reduce resistance to BRAF inhibitors?
Add a MEK inhibitor
348
Melanoyctic Lesions: Melanocytes are derived from what?
The neural crest
349
Melanoyctic Lesions: Early in embryogenesis the melanoblasts migrate from the neural crest to where?
Skin Uveal Tract Leptomeniges
350
Melanoyctic Lesions: Where are melanocytes located?
Basal layer
351
Melanoyctic Lesions: Involves what gene?
Melanocortin 1 Receptor Gene
352
Melanoyctic Lesions: Melanocortin 1 Receptor Gene encodes what?
MC1R protein
353
Melanoyctic Lesions: MC1R determines what?
Balance of pigment in the skin and hair
354
Melanoyctic Lesions: Eumelanin has what impact?
Causes any hair colour other than red
355
Melanoyctic Lesions: Phaeomelanin has what impact?
Causes red hair
356
Melanoyctic Lesions: MC1R has what action?
Converts Phaeomelanin into Eumelanin
357
Melanoyctic Lesions: One defective MC1R copy has what impact?
Freckling
358
Melanoyctic Lesions: Two defective MC1R copy has what impact?
Red hair and freckling
359
Biological word for freckles
Ephilides
360
Melanoyctic Lesions: Ephilides - What are these?
Patchy increases in melanin production (Freckles)
361
Melanoyctic Lesions: Ephilides - Occurs after what?
UV exposure
362
Melanoyctic Lesions: Ephilides - Reflects an increased what?
Density of melanocytes
363
Melanoyctic Lesions: Actinic lentigines
Age or liver spots related to UV exposure on the face, forearms and dorsal hands
364
Melanoyctic Lesions: Actinic lentigines - Refects increased what>
Melanin and basal melanocytes
365
Melanoyctic Lesions: Actinic lentigines - Impact on the epidermis
Elongated rete ridges
366
Melanoyctic Lesions: Naevi - Types (4)
Usual Dysplastic Spitz Blue
367
Melanoyctic Lesions: Naevi - First stage of development
Junctional naevus
368
Melanoyctic Lesions: Naevi - Second stage of development
Compound Naevus
369
Melanoyctic Lesions: Naevi - Thirs datge of development
Intradermal Naevus
370
Melanoyctic Lesions: Naevi - Junctional Naevus
Melanocytes proliferate to form clusters of cells at the dermoepidermal junction - nests of melanocytes attach to the epidermis
371
Melanoyctic Lesions: Naevi - Compound Naevus
Junctional clusters with groups of cells in the dermis
372
Melanoyctic Lesions: Naevi - Intradermal Naevus
All junctional activity is ceased and the naevus becomes entirely dermal
373
Melanoyctic Lesions: Congenital Melanocytic Naevi - Small classification
<2cm
374
Melanoyctic Lesions: Congenital Melanocytic Naevi - Medium classification
2-20 cm
375
Melanoyctic Lesions: Congenital Melanocytic Naevi - Large classification
>20cm
376
Melanoyctic Lesions: Usual Type Acquired Naevi - Pathophysiology
Melanocyte:Keratinocyte Ration breaks down at cutaneous sites to allow the formation of a simple naevi
377
Melanoyctic Lesions: Usual Type Acquired Naevi -Why do immunosuppressed leukaemia patients have more naevi?
As naevus induction is immunoregulated
378
Melanoyctic Lesions: Dysplastic Naevi - Presentation
Asymmetrical and >6cm in diameter with variegated pigment
379
Melanoyctic Lesions: Dysplastic Naevi -Two types
Sporadic Familial
380
Melanoyctic Lesions: Dysplastic Naevi - Inheritance of familial type
Autosomal inheritance
381
Melanoyctic Lesions: Dysplastic Naevi - Familial has a high penetrance of what gene?
CDKN2A
382
Melanoyctic Lesions: Dysplastic Naevi - Features
Architectural and cellular atypia
383
Melanoyctic Lesions: Dysplastic Naevi - Why does architectural and cellular atypia occur?
Host reaction to fibrosis and inflammation
384
Melanoyctic Lesions: Halo Naevi - Presentation
Peripheral halo of depigmentation
385
Melanoyctic Lesions: Blue Naevi - Depth
Entirely dermal
386
Melanoyctic Lesions: Halo Naevi - Consist of what?
Pigment-rich dendritic spindle cells
387
Melanoyctic Lesions: Spitz Naevi - Consist of what cells?
Large spindle or epithelioid cells
388
Melanoyctic Lesions: Spitz Naevi - Age
<20 years
389
Melanoyctic Lesions: Spitz Naevi - Mimics what disease?
Melnaoma
390
Melanoyctic Lesions: Spitz Naevi - Colour
Pink
391
Melanoyctic Lesions: Spitz Naevi - Why is it pink?
Prominent vasculature
392
Melanoma: Sex incidence
More common in females
393
Melanoma: Age incidence
Middle age
394
Melanoma: Location
Scalp Face Neck Arm Trunk Leg
395
Melanoma: How does BRAF cause cell proliferation?
Upregulates MEK and ERK
396
Melanoma: What does a suffix b mean?
Tumour ulceration
397
Melanoma: How do satellite deposits appear?
Local dermal lymphatics
398
Melanoma: Management of melanoma with regional lymph node metastases?
Excised by radical lymphadenectomy
399
Melanoma: Where does haematogenous spread metastasise to?
Skin Soft tissue Heart Lungs GIT Liver Brain
400
Melanoma: Excision - In situ how much clearance?
Clear by circa of 5mm
401
Melanoma: Excision - Invasive but <1mm thick how much clearance?
1cm
402
Melanoma: Excision - Invasive but 1-2mm thick how much clearance?
2cm
403
Melanoma: Excision - If the sentinel node is positive what is done?
Regional lymphadenectomy
404
Examples of pre-cancerous dysplasia
Bowen's Disease Actinic Keratosis Viral lesions
405
How is superficial non-melanoma sun damage managed?
5% Imiquimod cream
406
Seborrhoeic Keratosis
Benign epidermal tumour
407
Seborrhoeic Keratosis: Pathophysiology
Benign proliferation of epidermal keratinocytes
408
Seborrhoeic Keratosis: Common in what ages?
Elderly
409
Seborrhoeic Keratosis: Leser-Trelat Sign
Eruptive appearance of lesions that indicates internal malignancy
410
Seborrhoeic Keratosis: Presentation
Has a stuck on appearance - Well-demarcated, raised brown coloured lesion
411
Seborrhoeic Keratosis: Location
Face Trunk
412
Seborrhoeic Keratosis: Surface description
Greasy hyperkeratotic surface - may have depressions or small cysts
413
Seborrhoeic Keratosis: Management
Most are self-limiting - may need cryotherapy or curettage if requested
414
Bowen's Disease: Location
Legs
415
Bowen's Disease: What is this?
Squamous cell carcinoma in-situ
416
Bowen's Disease: Common in what sex
Females
417
Bowen's Disease: Presentation
Scaly patch or plaque with an irregular border and no dermal invasion
418
Bowen's Disease: Presentation
Scaly patch or plaque with an irregular border and no dermal invasion
419
Acitinic Keratosis: Presentation
Atypical Bowenoid lesions with variable epidermal dysplasia
420
Acitinic Keratosis: Common precursor to what?
Invasive SCC
421
Acitinic Keratosis: Location
Scalp Face Hands
422
Acitinic Keratosis: Management
Topical Cytotoxic - 5-Flurouracil
423
Viral Lesions: Erythroplasia of Queryat-Penile Bowen's are associated with what virus?
HPV
424
Viral Lesions: HPV-16 is associated with what?
Penile dysplasia
425
Squamous Cell Carcinoma: Factors that increase risk? (5)
Thickness - >4mm Poor differentiation Lymphatic and vascular space invasion Perineural spread Sites of Poor Prognosis - Scalp, Ear or Nose
426
Xeroderma Pigmentosum: Aetiology
Defect in one nucleotide excision repair gene - XPA-G
427
Xeroderma Pigmentosum: Presentation
Skin cancer on UV-exposed site with neurological degeneration
428
Xeroderma Pigmentosum: Most individuals develop what by the age of 2?
Solar lentigins
429
Oculocutaneous Albinism
Rare inherited disorders characterised by a reduction or complete lack of melanin pigment in the skin, hair and eyes
430
Oculocutaneous Albinism: Inheritance pattern
Autosomal recessive
431
Oculocutaneous Albinism: Absence or defective what?
Tyrosinase
432
Oculocutaneous Albinism: Clinical presentation (5)
Sun sensitivity - increases the risk of skin cancer Photophobia Nystagmus - involuntary eye movement Amblyopia - Lazy eye Lack of pigment in the retina
433
Naevoid Basal Cell Carcinoma: Alternate name
Gorlin's Syndrome
434
Naevoid Basal Cell Carcinoma: Aetiology
Autosomal dominant familial cancer syndrome
435
Naevoid Basal Cell Carcinoma: Major features of clinical presentation (4)
Early onset with multiple BCC Palmar pits Jaw cysts Ectopic calcification falx
436
Naevoid Basal Cell Carcinoma: Minor features of Clinical presentation (3)
Skeletal abnormality Cardiac or Ovarian fibroma Medulloblastoma
437
Photoageing: Age normal ageing starts
35
438
Photoageing: Fat in normal ageing
Subcutaneous fat is lost
439
Photoageing: Why does the skin become dry in normal ageing?
Due to less hair follicles, sweat and sebaceous glands
440
Photoageing
Ageing of the skin in addition to normal ageing on sun exposed sites
441
Photoageing: Solar Elastosis
Skin coarse and yellow with a loss of elastic quality
442
Photoageing: What irregular pigmentation occurs?
Freckling Lentigo
443
Photoageing: Presentation
Easy bruising Telangiectasia Atrophy Roughness and Dryness Deep wrinkling
444
Ersipelas
Superficial form of cellulitis localised to the dermis and lymphatic system
445
Ersipelas: Causative organism
Streptococcus pyogenes
446
Ersipelas: Clinical presentation
Well demarcated erythematous plaque
447
Ersipelas: Location
Face
448
Ersipelas: Associated symptoms
Fever Systemic upset
449
Ersipelas: Management
Oral Flucloxacillin
450
Photosensitivity
Group of skin disorders that result from exposure to normal levels of UV light exposure
451
Photosensitivity: What classfication is used for skin types?
Fitzpatrick skin types
452
Photosensitivity: Type 1 Skin type
Very fair skin that always burns and never tans
453
Photosensitivity: Type 2 Skin Type
Fair that usually burns and sometimes tans
454
Photosensitivity: Type 3 Skin Type
Medium that sometimes burns and usually tans
455
Photosensitivity: Type 4 Skin Type
Olive that rarely burns and always tans
456
Photosensitivity: Type 5 Skin Type
Brown that never burns and always tans
457
Photosensitivity: Type 6 Skin Type
Black that never burns and always tans
458
Keratoacathoma
Rapidly growing epidermal tumours
459
Keratoacathoma: Presentation
Red papule with a central crater-like crusty keratinous plug
460
Keratoacathoma: Location
Sun-exposed sites
461
Keratoacathoma: Diameter
2-3cm