disease profiles Flashcards
autoimmune skin condition which antibodies are produced against hemi desmosomes proteins that are involved in the maintenance of the dermo epidermal junction
bullous pemphigoid
what does bullous pemphigoid result in
interuption of the dermo epidermal junction and the formation of sub epidermal blisters
presentation of bullous pemphigoid
elderly, well dermarcated plaques may present 1 year prior to blisters, large tense itchy blisters on skin or erythemous base typically trunk or proximal limbs
nikolsky sign what in bullous pemphigoid
negative
what is nikolski sign
skin finding in which the top layers of the skin slip away from the lower layers when rubbed
how to diagnose bullous pemphigoid
biopsy - sub epidermal blisters and inflammatoru infiltrates within blister and immunodluorescence - linerar IgG + complement along the basement membrane
management of bullous pemphigoid (local disease)
high potency topical steroids
management of bullous pemphigoid (systemic disease
oral steroids +/- tetracycline +/- antihostamine
why is an antihistamine used in bullous pemphigoid ?
for sedating and antu pruritic properties
why is tetracycline used in bullous pemphigoid
steroid sparing agents
how to treat bullous pemphigoid if there is no response ti antihistamine or tetracycline
immunosuppression
autoimmune condition in which there is antibodies produced against desmoglein 3
pemphigus vulgaris
what is desmoglein 3
one of the desmosome proteins involved in cell-cell adhesion
what does pemphigus vulgaris result in
intra epidermal blisters and acantholysis
what is acantholysis
seperatiion of indiduals keratinocytes
presentation of pemphigus vulgaris
middle aged, multiple painful, flaccid, fragile blisters and erosions of the skin and mucous membranes
pemphigous vulgaris nikolsky sign
positive
diagnosis of pemphigous vulgaris
biopsy intra epidermal blister with accumulation of inflammatory cells within the dermis and immunofluorescence - chicken wire depositation of IgG within the epidermis
management of local pemphigous vulgaris
topical steroids and topical anaesthetics
management of systemic pemphigous vulgaris
high dose steroids and immuno suppresion + /- rituximab
what immunosuppressant is used in the management of pemphigois vulgaris
mycophenolate, azathioprine, dapsone or cyclophosphamide
autoimmune skin condition caused by auto antibodies against TTG caues sub epidermal blisters in skin
dermatitis herpetiformis
what is TTG
the antibody implacated in ceoliac disease
presentation of dermititis herpetiformis
small intenesly itchy blisters on an erythemous swollen base
diagnosis of dermititis herpetiformis
blood anti TTG, biopsy of sub epidermal blitsers with papillary micro abseceses, immunofluorescence - granular deposits of IgA within papillae of epidermis
management of dermititis herpetiformis
gluten free diet +/- dapson
rare complication of dermititis herpitiformis
increased risk of small bowel lymphoma
incidence of psoriasis in men and women
equal
incidence of psoriasis age wise
peaks in 20s and 50s
precipitating factors of psoriasis
stress, trauma, alcohol, smoking , strep throat, drugs
drugs putting people at risk of psoriasis
B blockers, lithium, anti malarial drugs, swift withdrawal of topical or systemic steroids
underlying pathological processes of psoriasis
increased epidermal proliferation, dilation and proliferation of dermal blood vessels, inflammation and accumulation of immune cells especially T cells in teh dermis and epidermis
what does increased epidermal proliferation result in
hyperkeratosis and para keratosis
what is parakeratosis
retention of nuclei in corneocytes due to increased proliferation redcing time cells are are allowed for migration and differentation
histological changes in psoriasis
hyperkeratotis stratum corneum with parakeratosis, absence of granual layer, thicking of prickle cells layer, munroabscesses, large dilated papillary blood vessels
what are munro absesses
neutrophil filled abscesses with stratum corneum
most common type of psoriasis
chronic plaque
appeaace of chronic plaque psoriaces
often symetrical and scale may be silvery
rash on chronic plaque psoriasis
midly itchy, palpable, scaly, erythematois plaques
what is auspitz sign
removing scale causes pin point bleeding
auspitz sign in chronic plaque psoriasis
positive
where does chronic plaque psoriasis preferentially develop
on extensor aspect on knees, elbows, sacrum and scalp
where is scap psoriasis most commonly seen
posterior aspect of the scalp
what is koebner phenomenon
plaques of psoriasis develop at site of trauma 2-6 weeks after a trauma
what types of trauma can induce koebner phenomenon
physical, sunlight or caused by another skin condition
what age group is guttate psoriasis usually seen in
15-25 year olds
when is the onset of guttae psoriasis
7-10 days after infection
most common infection caising guttae psoriasis
strep throat
what is the appearance of guttae psoriasis
well demarcated, scaly, erythematous plaques that are pear drop in shape and develop on the trunk
where is fexural psoriasis seen (age group)
elderly patients
where does flexural psoriasis develop
in groin, axilla or under breasts
appearance of flexural psoriasis
erythematous, glazed, well demarcated plaques with out scale
how can erythrodermic psoriasis develop
de novo or in patients who have deterioating psoriasis
what can the onset of erythrodermic psoriasis be precipitated by
removal of potent steroids
what can erythrodermic psoriasis lead to
complete failure of skin
appearance of erythrodermic psoriasis
well demarcated plaques with absent scale with confluent full body erythema
what is generalised pustular psoriasis associated with
pain, fever and malaise
appearance of pustular psoriasis
sterile pustules within plaques of psoriasis and widespread erythema
what is palmo plater pustulosis
a distinct condition that is related to psoriasis
who does palmo plantar pustulosis effect (age and gender)
typically women over 50
what does palmo plantar pustulosis have a strong association with
smoking
what is the appearnace of palmo plantar pustulosis
multiple sterile yellow pustules that develop into brown macules then develop scale
nail disease associated with psoriasis
pitting, onchyoysis, subungual hyperkeratosis, sydtrophy, oil drop lesion
what is a risk of those with severe psoriasis
cardiovascular provlems
use of emollients in patients with psoriasis
used liberally by all pateints - great ointments may need to be used on scalp
what is generally considered the first line topical therapy in psoriasis
vit D analogues
examples of vit D analogues
calciptriol/calcitriol
how is dithranol used in psoriasis
in short term regimes for stable chronic plaque disease
why is dithranol only used in short term regimes for stable chronic plaque disease
as it burns and stains normal skin
where is coal tar used in psoriasis
only in inpatient and smelly and messy
what is used for flexural disease and palmer plantar disease
steroids
when is used to break down hyperkeratotis skin
salicylate
when is photodynamic therapy used in psoriasis
in severe widespread disease and as a first line for guttae psoriasis
what UV can be used in photodynamic therapy for psoriasis
UVB or PUVA
side effects of photo dynamic therapy
sunburn, conjunctivitis, excerbatoion of HSV
when in systemic therapy used in psoriasis
in severe or non responsive disease
options for systemic therapy in psoriasis
methrotrexate, ciclosporin terinoids, biologics (infliximab)
what is synomonous with eczema
dermatitis
what is dermatitis
an umbrella term used to describe a group of inflammatory skin conditions that share similar clinical and histological presentations
shared histological finidngs of dermatitis
spongiosis, ancanthosis, hyperkeratosis, dilation of blood vessels, eosinophilic infiltration
shared general clinical features of dermatitis
itchy, ill defined, erythematous rash +/- scale, sxcoriations, papules, vesicles, ooze and crust
chronic features of dermatitis
scale, skin thickening pigment changes and lichenification
what is atopic dermatitis
endogenous dermatitis associated in defective barrier function of the skin
what is the genetic contribution to atopic dermatitis
fillagrin protein mutations
what is fillagrin protein
a. protein involved in maintaining the waterproof protective nature of the keratin layer
what does mutation in the fillagrin gene lead to
predisposition to all atopic disease
prevelenec of atopic dermatitis in urban area and high socio economic class
higher
immunological influences of atopic dermatitis
overactive T cells
risk factors for atopic dermatitis
age <5, family/personal history of atopy
what does the atopic march describe
clinical progession of patients with atopy with eczema in infancy, asthma at 2 years old and hay fever at 7
presentation of atopic dermatitis
usually between 6 months and 5 years, associated with dry skin, will follow relapsing and remitting course
rash distribution of atopic dermatitis in infants
face, scalp, extensor surfaces and flexor surfaces and napkin area spares
rash distribution of atopic dermatitis in children and adults
flexor surfaces, especially wrist, cubital fossa, popliteal fossa and ankles
triggers of atopic dermatitis
stress, non compliance with treatment, allergens
diagnostic criteria of atopic dermatitis
itch + 3 or more of visible flexure rash, history of flexure rash, personal history of atopy, dry skin in past year, onset before age of 2
associated bacterial infection with atopic dermatitis
staph A and produces yellow weeping crust over the eczema
viral infections associated with atopic dermatitis
eczema herpeticum - infection with herpes simplex - emergency
presentation of eczema herpeticum
monomorphic punched out lesions
management of eczema herpeticum
IV aciclovir
management of atopic dermatitis
everyone - emollients
for itch - anti histamines
flare - topical steroids
mild - mild topical steoirds
moderate - moderate topical steroids
severe - potent topical steroids +/- UV light therapy +/- systemic immunosuppressio
example of mild topical steroids
hydrocortisones, eumovate
examples of moderate topical steroids
betnovate +/- tacrolimus
examples of potent tpoical steroids
Dermovate
what to use in patienst relying on continual us of sterois
tacrolimus
what vit D analogue to use for plaques in psoriasis
calcipqotriol
what vit D analogue to use of flexures in psoriasis
calcitriol
what is seborrheic dermatitis considered
endogenous dermatitis
what is seborrheic dermatitis caused by
commensal yeast on skin
what is the development of seborrheoic dermatitis associated with
immunosuppression due to the likes of HIV or drugs such as ciclosporin
presentation of seborrhoeic dermatitis rash
itchy rash, erythematous and scaly
distribution of seborrhoeic dermatitis on newborns
cradle cap, flexural surfaces abd napkin area
distribution of seborrhoeic dermatitis on adults
forhead, nasolabial folds, behind ear and anterior chest
management of seborrhoeic dermatitis in infants
- emoillients +/- topical steroids
management of seborrhoeic dermatitis in adults
emollients + topical steroids + antifungal
example of anti fungal
ketocanozole
what is pompolyx
a type of eczema that effects the hands and soles of the feet
what is the rash on pompholyx present as
erythematous rash with intensely itchy vesicles that burst to produce superficial erosions
what is azteototic
a type of eczema seen in elderly patients
what is the rash associated with azteototic like
dry skin with polyhedral fissures creating a crazy paving pattern seen on the lower limbs
treatment of azteototic
emollients
what is venous eczema
a type of eczema presenting in elderly pateints that develops due to venous insuffieicency of lower limbs
what is the managemnet for venous eczema
compression to treat venous insuffiency
what does discoid develop as a result of
chronic itch
what is discoid associated with
atopic eczema and other conditions that cause itch sich as renal failure
what is the rash of discoid like
often widespread, disc shaped, intensely itchy lesions
what is irritant contact dermatitis
a non immune form of dermatitis
what is irritant contact dermatitis caused by
repeated exposure to substances that abrade irrtate and aggrevate skin
what occupations commonly cause irrtant contact dermatitis
hairdressers, cleanerd and hospital workers
what is the mechanism of contact allergic dermatitis
is a type 4 T cell mediated hypersensitivity reaction in response to an antigen that they have had exposure to
when do rash a skin changes occur in contact allergic dermatitis
48-96 hours after exposure to the antigen
whatputs people at risk of contact allergic dermatitis
an increased risk in those with chronic skin conditions such as leg ulcers due to exposure of topical therapyies
how to diagnose contact allergic dermatitis
patch testing
management of contact allergic dermatitis
antigen avoidance, regular emollients, topical steroids during flares
what is acne
an inflammatory condition of the pilosebaceous unit
what are the pathological changes seen in acne
duct occlusion, increased sebum production, bacterial colonisation, duct rupture
whatis duct occlusion due to
hyper cornification
what does hypercornification produce
comedones
what are black heads
open comedones
what are white heads
closed comedones
what can worsen occlusion in acne
cosmetics and pre menstral oedema
what is sebum
oil used to lubricate the skin in an androgen sensitive manner
how can sebum production be increased
due to increased androgen production, increased availability of androgens and increased sensitivity of androgen receptors
what is the bacteria that causes bacterial colonisation in acne
propionobacterium which is a normal bacteria natirally found in the skin
what aids bacterial colonistaion
sebum
what causes the duct of the hair follicle to rupture in acne
presence of propianobacterium acne which prodces inflammation of the dermis and formation of lesions such as papules, pustules, cysts and nodules
risk factors for acne
age between 12-25, family history, greasy skin, endocrine disorders
aggregating factors of acne
stress, sweating, pre menstural period
distribution fo acne
face, chest and upper back
examples of non inflammatory lesions in acne
black heads and white heads
examples of inflammatory lesions in acne
papules, pustules, cysts, nodules on erythematous base
complications of chronic acne
scars and skin hyper pigmenation
describe acne scars
ice pick scars of hypertrophic keloid scars
mild acne classification
scattered comedones, papules and pustules
moderate acne classification
numberous papules, pustules and mild atrophic scarring
severe classification of acne
numerous papules, pustules, mild atrophic scarring, cysts, nodules and significant scarring
how to treat mild acne
topical only - retinoid, benzyl peroxide +/- antibiotic
what antibiotic to use in mild acne
erythromycine or clindamycin
how to treat moderate acne
topical therapies + oral antibiotic (erythromycin or oxytetracycline, contraceptive pill can be used in women
how to treat severe acne
topical therapies + isotretinoin
what gender is acne rosacea more common in
women
what age range does acne rosacea spike in
30 and 40s
presenation of acne rosacea
facial flushing, rash - erythema with papules and pustules on nose chin, cheeks and forehead wuth sparing of nasolabial folds
complications of acne rosacea
talangectasia, rhinopyma
what is facial flushing in acne rosacea triggered by
alcohol, hot drinks, stress, spicy food
first line management of acne rosacea
topical metronidazole
second line for acen rosacea
topical therapies + doxycycline
third line for acne rosacea
isotrenanoin
how to treat talangectasia and rhinopyma
laser therapy
what is talangectasia
“spider veins”, dilated or broken bood vessels lacyer near surface or mucous membranes
what is pityraisis rosea
a spontaneous inflammatory skin condition
what may bring on pityraisus rosea
virus
what age group pityraisis rosea seen in
teenages and young adukts
presentation of pityraisus rosea
sudeen onset, hearald patch, subsequent rash
what is hearald patch
oval shaped erythematous scaly patch usually on trunk or neck that is larger than other lesion
what is the rash like in pityraisis rosea
smaller oval shaped scaly lesions on trunk and extremities that spread along skin cleavages which gives a christmas tree appearance
what is the management of pityraisis rosea
self limiting - emmolients and anti histamines
distribution of lichen planus
flexor surfaces of wrist/foreaerm, ankles, legs and oral mucosa - can develop at site of trauma
describe rash associated with lichen planus
intensely itchy rash purple, flat topped papules or plaques that are rhomboid or poly angular in shage +/- white lacy markings
describe oral lesions associated with lichen planus
lacy white lesions on inside of cheek
what is lichen planus associated with
Hep C
management of lichen planus
topical steroid + anti histamine
what is tuberose sclerosis
a multi system autosomal dominant genetic condition
what is the most common genetic skin condition
tuberose sclerosis
what are the skin manifestations of tuberose sclerosis
ash leaf macules, perigungal fibroma, sebaceus adenoma, shagreen patches
what are ash leaf macules
oval shaped areas of hyper pigmentation
what is perigingal fibroma
fibroma of the nail bed
sebaceous adenoma
angiofibroma of the sebaceous glands on the face
what are shagreen patches
sir, skin coloured nodules
what is neurofibromatosis type 1
a multisystem autosomal dominant genetic condition
what are the skin manifestations of neurofirbomatosis type 1
neurofibromas, axillary or inguinal freckles, cafe au lait macules
how many cafe au lait macules do you need to diagnose
over 6
what are cafe au lait macules
light briown macules
what are the systemic manifestations of type 1 neurofibromatosis
optic glioma, lisch nodules on iris
histology of lichen planus
saw tooth
what are salmon patches
non vascular birth marks usually found on face or nuchal area
why are salmon patches thought to develop
persistant fetal circulation
appearance of salmon patches
central erythemous macule
what happens to salmon patches over time
vast majority regress
what are port wine stains
capillary malformations that are present at birth and are permanent
where are portwine stains usually found
in head and neck region
appearance of portwine stains
red macule usually unilateral
what happens to portwine stains overtime
darken and thicken
how do port wine stains grow
proportionally with the face and dont extend from the original site
how to describe the locations of portwine stains on the face
in relation to the distribution of the trigeminal nerve
associated conditions with portwine stains
sturge- weber syndrome, klippel- trenaunay
what is associated with struge weber syndrome
V1 portwine stain, seizures, learning disability, hemi paresis, glaicoma, vascular malformation is found in the cerebral cortex on the ipsilateral side to the port wine stain
what are infantile haemangioma also called
strawberry neavi
what are infantile haemangiomas
benign vascular tumours that usually appear in the first moneth of life in the head and neck region
who are infantile haemangiomas most common in
woman and premature babies
appearance of infantile haemangiomas
superficial red plaque
what happens to infantile haemangiomas
they mostly undergo complete or partial resolution
presentation of erythema multiforme
target lesions
severe erythema multiforme
steven johnstone synfrome or toxic epidermal necrolysis
what is erythema multiforme major associated with
mucosal involvement
what are the causes of erythema multiforme
herpes, mycoplasma, hep b, ebv, cmv, thiazide diuretics, anti malarials, sulphonylureas, penicillins, allopurinol
management of erythema multiforme
supportive care and treat underlying cause
systemic disease associated with diabetes
necrobiosis lipoidica
presentation of necrobiosis lipoidica
firm waxy, red brown or yellpw plaques on anterior shin associated with increased risk of ulceration
what is associated with diabetes, addisons and malignancy
acanthosis nigricans
what does acantosis nigricans look like
thickened brown hyperpigmented velvety skin in flexural areas
what is associated with graves
pretibial myxoedema
what does pretibial myxoedema look like
purple brow, indurated waxy plaques on anterior shins
what is erythema nodosum associated with
IBD, TB, sarcoids, OCP use
presentation of erythema nodosum
painfl, erythematous nodules on shins
what is pyoderma gangrenosum associated with
IBD and rheumatoid
presentation of pyoderma gangernosum
pustules and blisters that break down to form necrotic ulcer with purplish margins
what is kaposi sarcoma associated with
HIV
what does kaposi sarcomas look like
brown/purple nodules
what is the difference between SJS and TENS
the extent of skin invlovement
extent of skin involvement in SJS
<10%
extent of skin invlovement in TENS
> 30%
presentatin of SJS/TENS
widespread erythema and blistering of the skin and mucousal surfaces - the blisters merge and undergo necrosis and large sheets of skin are lost
causes of SJS and TENS
penicillins, NSAIDs, anti-convulsants
management of TENS and SJS
cessation of causative drug, supportive care, emollients, Iv Ig
what is the most common type of porphyria
PCT - cutanea tarda
what is porphyria
a group of diseases caused by errors in haem and accumulation of toxic precursors
what is the pathophysiology of PCT
caused by deficiency in the enzyme uroporphyrinogen decarboxylate.
what can cause PCT
genetic, alcohol, iron and oestrogen replacement
clinical features of PCT
photo sensitivity of dorsum of hands, blisters and bullae that easily rupture and cause erosions, hyperpigmentation and hypertrichosis - especially at the top of the cheek
diagnosis of PCT
by testeting elevated urine porphyrins or through skin biopsy
management of PCT
avoid sunlight and triggers, phlebotomy to reduce iron levels and antimalarials such as chloroquine
what are seborrhoeic keratoses also known as
basal cell papillomas
what are seborrhoeic keratoses
benign proliferations of epidermal keratinocytes
presenation of seborrhoeic keratoses
usually elderly patients, on head forearms, back of hands and trunk, well demarcated raised usually brown coloured lesion with well defined edges warty granular surface and stuck on appearance, no pain or itch
management of seborrhoeic keratoses
non surgical - cryotherapy, curettage
what are actinic lentinges also known as
sun or liver spots
where do actinic lentinges develop
in sun exposed sites
pathophysiology of actinic letinges
melanocyte proliferation as a protective mechanism against sun exposure
appearance of actinic lentinges
flat brown macule
what are melanocytic neavi
moles
how can moles develop
can be congenital or acquired
what is a mongolian blue spot
a large blue coloured congenital neavi that is found on the back and buttocks
what is spitz
fleshy pink papule seen in children
appearance of halo
surrounded by an area of depigmentation
what is dermatofibroma
benign tumour of fibroblasts
location of dermatofibroma
anywhere but usually on the lower limb
development of dermatofibroma
associated with trauma such as an insect bite
appearance of a dermatofibroma
firm, hyperpigmented or pink papule +/- associated pain and itch
what is pyogenic granuloma
capillary haemangioma most that develops at the site of trauma especially on fingers
appearance of pyogenic granuloma
well defined papule, red papule associated with bleeding
management of pyogenic granuloma
surgical excision
what is the most common type of skin cancer
basal cell
what is the least dangerous type of skin cancer
basal cell
does basal cell carcinoma metastasize
rarely, but it can be locally destructive
who usually presents with basal cell carcinoma
middle ages patients on sun exposed areas
risk factors for basal cell carcinoma
fair skin, UV light exposure, intermittent sun damage during childhood
presentation of basal cell carcinoma
slow growing
3 types of basal cell carcinoma
nodular, superficial and infiltrative
characteristics of nodular basal cell carcinoma
raised lesion that has a pearly shine, rolled edges and a picket fence border, may also be associated with central depression or ulceration and talangectasia
diagnosis of basal cell carcinoma
clinical - biopsy
management of basal cell carcinoma
conservative, surgical or topical imquimod or 5 - fluro uracil
what are actinic keratosis
pre malignant lesions associated with partial thickness intra epidermal proliferation of atypical keratinocytes
where are actinic keratosis found
sun exposed areas, especially on the head
presentation of actinic keratosis
slow growing macules or plaques that have associated erythema, scale and a crumbly yellow crust
diagnosis of actinic keratosis
clinical +/- biopsy
what is bowens disease
pre malignant lesions associated with full thickness dysplasia within the epidermis
what is SCC that has not broken through the dermis
bowens disease
location of bowens disease lesions
on legs - especially the shins of older woman
presentation of bowens disease
usually a solitary lesion, slow growing, red scaly plaques
what is the second most common skin cancer
squamous cell
what does squamous cell carcinoma have the potential to do
metastasize
who and where does squamous cell carcinoma present
in middle aged patients on sun exposed sites
risk factors for squamous cell carcinoma
HPV, fair skin, cumulative sun damage over the years, immunosuppresson especially in post transplant patients
presentation of squamous cell carcinoma
growth of lesion over months, firm erythematois plaque on sun exposed site, associated scale, crust, bleeding, ulceration, itch and tenderness
diagnosis of squamous cell carcinoma
biopsy
management of squamous cell carcinoma
screen for mets, surgical
risk factors for melanoma
fair skin, sun bed use, immunosuppression, family or personal history, history of intermittent sun damage in childhood
growth phases of melanoma
radial and vertical
radial growth phase of melanoma
melanoma grows horizontally within the epidermis - catch at this stage as there is no risk of metastasis
vertical growth phase of melanoma
occurs after the radial growth and is when the lesion will become elevated. once it invades the dermis there is a risk of metastasis
types of melanomas
superficial spreading, nodular, lentigo maligna, amelanotic
superficial spreading melanoma
most common relatively long radial growth phase
nodular melanoma
enters straight into vertical growth phase and has worse prognosis
lentigo maligna melanoma
usually on face of old women, within a lentigo
amelanotic melanoma
non pigmented lesions
how does melanoma occur
usually de novo but can develop from dysplastic neavi
how does a patient usually present with melanoma
usually after noticing a change in what they thought was a mole
assessment of melanoma
ABCDE
diagnosis of melanoma
clinical and biopsy
management of melanoma
surgical excision with 2mm margins, check breslows thickness, follow up for wide local excision
what is berslows thickness
the depth of tumour from basal layer of the epidermis
what does photosensitivity describe
a group of skin disorders that result from exposure to normal levels of UV light exposure
UVA
longer wave can penetrate glass
UBV
smaller wave length main contributing type to sunburn and skin cancer
factors affecting photosensitivity
intensity of UV light, genetics, drugs and skin type
always burns never tans
type 1
usually burns sometimes tans
type 2
sometimes burns usually tans
type 3
rarely burns always tans
type4
never burns always tans
type 5 and 6
what is xeroderma pigmentosum
autosomal recessive genetic disorders caused by. adefiecincy in the DNA repairs mechanisms in the skin
presentation of xeroderma pigmentosum
severe photosensitivity, accelerated photo aging, early formation of skin cancers
management of xeroderma pigmentosum
patients are required to undertake strict sun avoidance
what is porphyria
a group of cutaneous and systemic conditions associated with deficiency of enzymes involved in the synthesis of haem
where does acute intermittent porphyria usually present
females around the age of 30
presentation of acute intermittent porphyria
acute abdomen, mood disturbance, neurological, seizures
enzyme deficiency associated with acute intermittent porphyria
prophobilinogen deaminase
what accumulates in acute intermittent porphyria
porphobilinogen
what is the most common type of porphyria
prophyria cutanea tarda
who is PCT most commonly seen in
middle aged men
what is PCT commonly associated with
liver disease therefore often seen in those with haemochromatosis, hepatitis or alcohol misuse
enzyme deficiency in PCT
uroporphyrinogen decarboxylase
what accumulates in PCT
uroporphyrinogen
what js the rash like in PCT
blistering lesions on sun exposed sites that heal with scarring and are associated with hyper pigmentation
investigations of PCT
bloods - porphyrin studies, woods lamp - urine shines pink instead of blue
what is erthropoetic protopohphyria mode of inheritance
autosomal dominant
who is erthropoetic protopohphyria most commonly seen in
children
what is the rash associated with erthropoetic protopohphyria like
may be no rashbut there will be burning and itching on the skin with sun exposure
typical presentation of erthropoetic protopohphyria
child screaming when placed out in the sun
what is the enzyme deficient in erthropoetic protopohphyria
ferrochelatase
what accumulates in erthropoetic protopohphyria
protoporphyrin IX
common causative drugs of photosensitivity
NSAIDs, Diuretics, amiodarone, antibiotics - tetracyclines, ciprofloxacin
what is polymorphic light eruption
a photosensitive disorder whose mechanism is thought to be type4 hypersensitivity
who does polymorphic light eruption usually present in
women around the age of 30
presentation of polymorphic light eruption
usually after a few hours of sunlight exposure, erythema lesions of varying morphology but usually blisters and papules - severe itch
management of polymorphic light eruption
topical steroids and desensitizing using UVB prior to sunlight exposure
chronic actinic dermatitis is what type of reaction
type 4
who is chronic actinic dermatitis usually seen in
men over 50
presentation of chronic actinic dermatitis
develop on eczematous type rash on sun exposed sites, often associated with contact allergic dermatitis
management of chronic actinic dermatitis
sun protection and standard eczema treatments of emollients and topical sterids
what types of reaction is solar urticaria
type1
what. is the presentation of solar urticaria
immediate onset of erythema, urticaria and itchy rash upon exposure to sunlight - rash will resolve within a few hours
management of solar urticaria
sun avoidance and anti histamines
what are urticaria and angioedema
transient rashes caused by oedema caused by mast cell degranulation and release of inflammatory mediators
where is oedema when urticaria develops
within the dermis
presentation of urticaria
erythematous localised swellings of various shapes that can be itchy and stingy
how long does urticaria typically last for
less than 24 hours
where is oedema when angioedema develops
in the subcutaneous or sub mucousal tissues
presentation of angioedema
non pitting swelling
how long does angioedema last for
24-48 hours
acute angioedema / urticaria classification
recurrent daily episodes for less than 6 weeks
chronic angioedema/urticaria classification
recurrent daily episodes for longer than 6 weeks
what is the most common cause of acute urticaria
allergy
gold standard investigation for urticaria
skin prick testing
drugs that cause angioedema/urticaria
ACEi, NSAIDs, Codeine /morphine
physical causes of angioedema/urticaria
solar, cold, dermographism, cholinergic(shower/exercise), antoimmune (SLE)
management of angioedema and urticaria
antihistamines, allergen avoidance, UVB phototherapy
what does UVB phototherapy do
stabilises mast cells
what. isa primary varicella zoster infection
chicken pox
presentation of chicken pox
fever, malaise, intense itch, macules that develop into pustulesthen vesicles that crust over and recover
where does chicken pox usually start
face then trunk / limbs
management of chicken pox
supportive, vaccination
who is the chicken pox vaccine available for
non immune health workers, pregnant women, those in regular contact with immunosuppressed patients
what type of vaccine is the chicken pox vaccine
live attenuated
what is shingles also called
herpes zoster
how is shingles caused
reactivation of dormant chicken pox virus which is dormant in sensory nerve roots
what is the presentation of shingles
burning, tingling pain and itch along dermatological distribution which may precede rash - rash is erythematous macules, papules that develop into vesicles before crusting over and healing
specific locations of shingles
ophthalmic, ramsay hunt
shingles on the forehead and around the eyes means
reactivation of the virus in the trigeminal nerve
shingles on the forehead requires
urgent ophthalmology referral
what is ramsay hunt syndrome
reactivation of the virus within the geniculate nucleus of the facial nerve
what does ramsey hunt syndrome cause
rahs and pain in the auditory canal associated with bells palsy, deafness, vertigo, tennitus
management of shingles
oral aciclovir and analgesia
complications of shingles
post herpetic neuralgia - which is chrnic dermatomal pain following resolution of shingles
type 1 herpes simplex causes what
primarily oral lesions but also can cause genital lesion s
type 2 herpes simplex causes what
only genital lesions
presentation of herpes
grouped painful, itchy vesicles on an erythematous base that burst to form ulcers crust over and then heal without scarring - oral - gingiva stomatitis
hat can recurrent herpes present with
erythema multiforme
what is herpetic whitlow
inoculation of the virus in the finger to produce a solitary painful lesion
what is eczema herpeticum
a disseminated infection that presents with monomorphic punched out lesions
who gets eczema herpeticum
children with atopic eczema
management of herpes
analgesia, aciclovir, education
what is HPV
causitive organism of warts and cervical cancer
what are warts caused by
infection of keratinocytes by HPV
what are warts
raised papules with a firm roughened surface
what can warts be described as
papillomatous
what does HPV 1-4 cause
common warts
what does HPV 6/11 cause
genitcal warts
what does HPV 16/18 cause
cervical cancer
management of HPV - general
salicylic acid, cryotherapy imiquimod
management of genetal warts
podophyllin, imiquimod
causative organism of molluscum contagiousum
pox virus
presentation of molluscum contagiousum
self limiting viral infection seen in children with lesions developing on head neck and trunk - rash is itchy, solid pink papules witha umbilicated centre
who. ismolluscum contagiousum seen in
those with atopic eczema
what is erythema infectiosum called
slapped cheek disease
causative organism of erythema infectiosum
parvovirus B19
how to detect parvovirus B19
B19 IgM
presentation of erythema infectiosum
self limiting infection commonly seen in children, rash is initially bilateral erythema on cheeks but then turns into a maculopapular rash with lacy erythema on trunk and limbs
associated symptoms of erythema infectiosum
fever, poly arthritis
causative organism of Orf
parapox virus
where is parapox virus found
in sheep
presentation. of orf
in farmers, single firm fleshy nodules on the hands, self limiting
causative organism of hand foot and mouth
coxsackie virus
presentation. of hand foot and mouth
promdormal fever malaise, grey vesicles surrounded by erythema and ulcers - self limiting
