conditions Flashcards
What is Dermatitis Herpetiformis
chronic immunobullous condition associated with coeliac disease
Dermatitis Herpetiform epidemiology
- Young adults (15-40year olds)
- Caucasians
- More common in males
Dermatitis Herpetiform
Aetiology
- Autoimmune disease in pxs with gluten intolerance
- Risk factors: coeliac disease, family hx of autoimmune
Dermatitis Herpetiform
Pathophysiology
- Intolerance to gliadine part of gluten causes autoimmune reaction in patient
- IgA molecules are released into circulation before migrating to skin and creating the appearance of this disease
Dermatitis Herpetiform
Histophysiology
- Papillary dermal micro abscesses
- Subepidermal blisters
- Neutrophil + eosinophil infiltrates in the dermal papillae
Where is gluten found
- barley
- rye
- wheat
Dermatitis Herpetifrom
presentation
Small clusters of vesicles +/or papules:
- Present on knee + elbow extensor surfaces, scalp + buttocks
- Symmetrical distribution
- Extreme pruritus
GI Symptoms of coeliac disease
- Fatigue
- Steatorrhea
- Diarrhoea
- Bloating
- Weight loss
- Pale stools
Dermatitis Herpetiform
Diagnosis
- Skin biopsy (direct immunofluorescence shows granular IgA deposits in dermal papillae)
- tTG IgA antibody testing
- Duodenal biopsy (if coeliac disease suspected)
Dermatitis Herpetiform Managment
- GF diet
- Topical steroids (symptomatic relief)
- Dapsone (for rash + pruritis)
Type of cancer associated with dermatitis herpetiform
Small bowel lymphoma
PEMPHIGUS VULGARIS
Definition
Autoimmune condition characterised by intraepithelial blistering of skin + mucous membranes
PEMPHIGUS VULGARIS
epidemiology
- 50-60yr olds
- Ahkenazi Jews + Indians
- Ppl affected by other autoimmune conditions
PEMPHIGUS VULGARIS
Aetiology
- Idiopathic aetiology, occurs spontaneously
- Genetic influence
- Speicifc triggers (Drugs, malignancy, infection, Trauma)
PEMPHIGUS VULGARIS
Pathophysiology
- Circulating IgG molecules attack (the protein desmoglein 3) found within desmosomes holding keratinocytes together at the bottom of the epidermis
- Separating keratinocytes from eachother
- Fluid-filled blister appears
PEMPHIGUS VULGARIS
Histopathology
- Intra-epidermal blistering (basal layer stays stuck to dermis)
- Supra basal clefts + blisters containing acantholytic cells
PEMPHIGUS VULGARIS
Diagnosis
- Positive Nikolsky Sign
- Skin biopsy
- Acantholytic cells present
- Immunofluorescnes shows intracellular deposits of IgG on surface of keratinocytes throughout epidermis (chicken wire pattern)
PEMPHIGUS VULGARIS
Management
- Local (topical steroids + topical anaesthetics)
- Systemic (prednisolone)
- May need other specialties invovled (eg. opthamology)
PEMPHIGUS VULGARIS
prognosis
- relapsing + remitting course
- Stop treatment during inactive disease periods + restart during flare ups
BULLUS PEMPHIGOID
Definition
A rare, subepidermal blistering disease of autoimmune aetiology.
BULLUS PEMPHIGOID
EPIDEMEOLOGY
- Elderly (rare <50)
- Common in elderly w neurological conditions (like parkinsons, stroke and dementia)
BULLUS PEMPHIGOID
aetiology
- HLA associations (Suggesting genetic element)
- Idiopathic + spontaneous cause
- Possible triggers (medication, Injury, Skin infection)
BULLUS PEMPHIGOID
Pathophysiology
- IgG (IgE + Tcells also involved) attacks BP180 + BP230 proteins in BM of epidermis causing an acute inflam response (incl. neutrophil recruitment, complement activaation + release of proteolytic enzymes)
- This causes destruction of hemidesmosomes + later formation of subepidermal blisters
BULLUS PEMPHIGOID
histopathology
- Eosinophils prominent
- Subepidermal cleft w. obvious inflam infiltrates (eg. neutrophils + T cells)
BULLUS PEMPHIGOID
presentation
- Pruritus and erythema - (may precede blistering onset by months)
- Large, tense bullae.
- few or many
- located anywhere (often flexures)
- may burst and become crusted erosions - Sparing of mucosal surfaces.
BULLUS PEMPHIGOID
diagnosis
- Negative Nikolsky sign
- Skin biopsy => Subepidermal blistering (full thickness epidermis as blister roof)
- Antibody testing
- Indirect IMF for circulating BP180/BP230 antibodies
- Direct IMF shows antibodies along basement membrane
BULLUS PEMPHIGOID
Management
- Oral Prednisolone (MAIN TREATMENT)
- Antibiotic therapy (Tetracyclines)
- Topical steroids (potent or v potent)
- Antihistamines (for pruritus)
BULLUS PEMPHIGOID
Why are tetracycline antibiotics used
They have anti-inflam effect + are steroid sparing agents
What is negative Nikolsky sign
Rub skin, top layer will not shear off
Hair types
- Lanugo
- Vellus
- Terminal
What is scarring alopecia
follicles lost
What is non-scarring alopecia
Hair follicles still present (Can regrow)
Examples of non-scarring localised hair loss
- Alopecia areata
- Androgenetic (pattern alopecia)
- Trichotillomania
- Traction Alopecia (due to hair stlying)
- Tinea capitis (Scalp fungal infection)
Examples of localised scarring alopecia
- Burns/Trauma
-Chronic discoid lupus (CDLE) - Lichen Planus
- Frontal fibrosing alopecia
Causes of generalised hair loss
- Telogen effluvium (eg. sever illness, stress, childbirth)
- Endocrine (eg. thyroid disease)
- Drugs
- Dietary deficiency (eg. iron, zinc, vit D)
- Diffuse alopecia areata
- Malnutrition
- Androgenetic alopecia
Is generalised hair loss normally scarring or non-scarring
Non-scarring
Investigations for hair loss
- for fungal exam:
- Skin scraping
- Hair plucking
- Woods lamp examination (some fungal species flourescent) - general look at hair folicles
- Dermoscopy
- Scalp biopsy +/- Immunofluorescence
- Blood tests (FBC, TFTs, Iron/Zinc levels, Hormone profile)
What is this
alopecia areata
- circular areas of hair loss
- preservation of hair follicles
- Broken hair, tapering hair
Process of alopecia areata
- Autoimmune
- normal recovers itself (takes months to years to recover)
- recurrent episodes/extensive involvement = less likely to regrow
What is it called when alopecia areata affects:
- whole scalp
- Whole body
- Alopecia totalis
- Alopecia universalis
Pharmacological treatment for alopecia areata
- Topical steroids
- IL steroids
- Wig provision
what is this?
Trichotillomania
- self-induced hair loss (hair pulling)
- Asymmetrical
- Associated with psychological issues
What is this
Tinea capitis
fungal infection of scalp
Tinea Capitis investigations
skin scraping
hair plucking
woods lamp
Tinea capitis treatment
oral anti-fungal agent
Tinea capitis epidemeology
pre-pubertal kids
areas of poverty/overcrowded living
What is kerion
Dramatic tinea capitis - big immune response to fungal infection
Kerion identifiers
- Abscess formation
- More inflamed
- Localised scarring hair loss
What is this
Chronic Discoid Lupus Erythematosus
DCLE Diagnosis
clinical + biopsy with DIF
DCLE treatment
- Potent topical steroids
- hydroxychloroquine
always - photoprotection
Androgenetic/Pattern hair loss stages in men + women
- Localised problem
Males: Generally starts at resection at temples then vertex of scalp then it joins together)
Women: front of hair margin preserved, general thinning across scalp)
Androgenetic hair loss investigation
hormone levels
iron
zinc
Androgenetic hair loss treatment
- Minoxidil (regaine) (vasodilator, stops when not in use)
- Anti-androgens (common for females with PCOS)
- If extensive, wig
Hair excess broad categories
- Hirsutism
- Hypertrichosis
Hirsuitism
- Definition
- Excess hair growth in females in male distribution due to ^ androgen levels or ^ end organ response to normal androgen levels
Causes of hirsutism
- Familial/constitutional - associated w seborrhoea, acne, androgenetic alopecia
- Hormonal - eg.PCOS/Androgen secreting tumour
Hirsutism investigation
- History + exam
- Ask about other signs of androgen excess (eg. irregular menstrual cycle, acne, difficulty conceiving) - Hormone profile
- Ovarian US if indicated
What is hypertrichosis
Excessive hair growth in a non-adrogenic distribution
Hypertrichosis causes
- Local
- General
- Naevi (moles), occult spina bifida (indicated by faun tail sign), Chronic scarring/inflammation
- Malnutrition, Anorexia, Prophyria, occult malignancy, drugs (eg. minoxidil, phenytoin, cyclosporin)
What causes Beau’s line
caused by transient arrest in nail growth which occurs during acute stress/illness
What condition may they have
Psoriasis
- Nail pitting
- Dystrophy
- Subungual hyperkeratosis
- Onikolysis
- Oil drop sign
Normal nail label
What causes acute paronychia
Staph aureus
Strep pyogenes
What is this
Acute paronychia
Acute paronychia treatment
- drain it
- oral Antibiotics
What causes chronic paronychia
where is it commonly seen
- Candida / can be mould-related
- people who commonly deal with water
Chronic paronychia
topical or oral antifungals
protect skin from being wet all the time
What is this
fungal nail infection
- onicholysis
- subungual hyperkeratosis
Investigations for fungal nail infection
Nail clipping
Treatment for fungal nail infection
- Topical anti-fungal nail varnish
- Oral antifungal agent used for 3-6months if extensive disease (eg.Terbinafine)
What is this
Subungual haematoma
- normally due to trauma
Subungual haematoma treatment
- Let it grow out over time
- hole can be bore in nail to relieve pressure which is source of pain
what is this
malignant melanoma
NB - Hutchinson’s sign (spreading pigmentation)
What is this
myxoid cyst
- at proximal nail fold
- accumulation of synovial fluid
- gutter effect in nail due to (pressures growing nail)
