Shorts Flashcards
Lupus Pernio
Purple-red or violaceaous plaques and nodules - hard and indurated
Nose cheeks ears lips
Systemic manifestasions of sarcoidosis
Cutaneous - lupus pernio and erythema nodosum
Ocular - anterior uveitism glaucoma cataracts optic neuritis, conjunctival nodules
Parotid gland swelling
Interstitial lung disease
Restrictive cardiomyopathy abd pulmonary HTN
Hepatosplenomegaly and portal HTN
Lymphadenopathy
VII palsy and mononeuritis multiplex
Arthropathy
Sarcoidosis pathophysiology
Infiltrative - non caseating epithelioid granulmomas
Sarcoidosis - lungs
BHL, ILD, Bronchiectasis, restrictive lung defect, concurrent obstructive lung defect
Sarcoidosis - Abdomen
Elevated ALT / ALP, hepatic granulomas, cholestasis, hepatosplenomegaly
Sarcoidosis - heart
Restrictive cardiomyopathy, cardiac granuloma, pulmonary hypertension
Sarcoid - nervous system
CN palsies (VII most common)
Aseptic meningitis, ataxia, hypothalamic/pituitary involement
Mononeuritis multiplex
Psychosis
Sarcoid - lymphadenopathy
1/3 pts lymphadenopathy, R paratracheal LN most common, then cervical LN
Sarcoid - metabolic
Hypercalcaemia hypercalcuira
Sarcoid - MSK
Arthropathy
Sarcoid - investigations
FBC, U&E, ESR, LFTs, Ca, ACE, CXR, ECG, Echo, Slit lamp (opthal), BAL / tissue biopsy
Sarcoid - treatment
Topical - steroids
Systemic - oral steroids, methotrexate, hydroxychloroquine, azathioprine, thalidomide, allopurinol
Causes of raised serum ACE
Sarcoid Berylliosis Lymphoma TB Asbestosis
Lupus vulgaris
Enlarging re-brown papule coalesce to form slow growing plaque with thick hyperkeratotic margins and central atrophy
Cutaneous TB
Pretibial myxodema
Thyroid dermopathy
Bilateral asymmetrical firm non pitting plaques or nodules with pink/purple or brown appearance
Anterolateral aspect lower legs, tender +/- pruritic
Prominent hair folliceswith shiny skin orange peel appearane
Graves disease - determine thyroid status ?surgery ?radio-I
5% graves pts
Acanthosis nigricans
Obesity Insulin resistance / DM Cushings Acromegaly Hypo/per thyroidism PCOS Malignancy - AC GIT
Cushing’s
Moon face Centripedal obesity Buffalo hump Purpura Striae and thin skin Easy bruising Proximal myopathy
Acromegaly
Facies - prominent supraorbital ridge, prognathism, bitemporal hemianopia, carpal tunnel, thick skin
Ehlers-Danlos sydrome
Skin - thin, hyperelastic, fragile, impaired wound healing with cigarette paper scars, fish mouth wounds over sites of minor injury
Hyperextensible joints - apposition of thumb to forearm, hyperextension of elbow and knee >10%, flat foot
Eyes - myopia, blue sclerae, retinal haemorrhage / detatchment
CV - mitral prolapse, regurgitations, aneurysms
GI - GI blood loss
MSK - OA, kyphoscoliosis
Ehlers Danlos - GI
GIB
Diverticulae
Megacolon +/- rupture
Ehlers Danlos - CV
MV prolapse
MR/AR
Arterial aneurysms
Aortic dissection
DDx blue sclerae
Ehler Danlos
Marfans syndrome
Osteogenesis imperfecta
Joint hypermobility
Ehler Danlos Marfans Turners Noonans Fragile X Downs
Peutz - Jeghers
Brown-black pigmented macules on the peri-oral areas
Look into mouth as well!
Multiple hamartomas GI tracts (polyps)
Pallor/koilonychia, abdominal pain, obstruction/intussuseption
AD condition - germline mutation Chr 19
Malignancies associated - pancreas, GIT, breast. Cancer predisposing condition!
Genetic councessling / endoscopic surveillance
Necrobiosis lipoidica diabeticorum
Wll circumscribed coalescing oval plaques anterior surface of lower legs
Shiny with wazy atrophic centres and red/brown margins with telangectasia
Diabetes
Pemphigus
Fragile superficial blisters with clear fluid
Mucous membranes - tender
Drug Hx
Associated with myaesthenia gravis
Heal with scarring
IgG agains desmogleins
Penicillamine / benpen / cephalosporin / ACEi / NSAIDs
Pemphigoid
Deeper blisters, unlikely to rupture
More elderly
No mucosal involement
Preilation for flexural areas
Dermatitis herpetiformis
Erythematous vesicles/papules - intense pruritus
Extensor surfaces
Coeliac disease
DDx bullous skin eruption
Burns/ contact dermatitis
Impetigo / chicken pox
Treating dermatitis herpetiformis
Gluten free diet
Dapsone
Clubbing - respiratory
Bronchogenic Ca Mesothelioma ILD Bronchiectasis CF Empyema TN Asbestosis
Clubbing - CV
CCHD
Subacute endocarditis
Atrial myxoma
Clubbing - GI
CLD IBD GI lymphoma Coeliac and tropical sprue Whipples Disease
DDx unilateral clubbing
idiopathic
Bronchial AV fistula
Axillary / SC / ulnar aneurysm
Brachial plexus injury
Tuberous sclerosis (physical examination)
Salmon coloured papules and nodules onface in butterfly distribution esp nasolabial folds
Periungal fibromas - smooth firm nodular lesions adjacent to nails (more common in toes)
Shagreen Patch - ireegular uneven thickened plaques in cobble stone appearance lumboscaral
Ash leaf macules - areas of hypopigmentation
Cafe-au-laif spots
Tuberous sclerosis - genetics
AD, 2 loci, Chr 9 and 16, 65% spont mutation
Tuberous sclerosis - pathophysiology
Mutation in hamartin or tuberin (both tumour supressing)
Chr 9 and 16
Tuberous sclerosis - organ involements
Eye - retinal hamartoma, retinal hypopigmentation, angiofibromas of eyelid
Heart - cardiac rhabdomyoma
Brain - tubes, mental retardation, seizures
Lungs - cysts, PTX
Kidneys - hamartoma / angiomyolipoma, renal cysts
Mouth - gingival hamartoma, dental pits, high arched palate
Bone - cysts, sclerotic lesions
Abdomen - hamartoma, polyposis
Vascular - aneurysm aorta / carotid / renal / intracranial
Alopecia
Patches of hair loss, well circumscribed with a distinctive border (nail involement 40%)
Autoimmune condition, associations with vitiligo, hashimotos, graves, addisons, hypoparathyroid, DM, pernicious anaemia, MG, SLE, IPF, PBC
Treating alopecia accreta
Non pharmacological (cosmetic camouflage) Steroids, anthralin, minoxidil
Diagnostic criteria for SLE
4 of 11 from: Malar rash Discoid rash Photosensitivity Oral ulcer Arthritis Serositis Renal involement Neurological involement Haematological involement Immunological involement ANA +ve
Causes of drug induced lupus
Isoniazid Methyldopa Phenytoin Carbamazepine Sulphonamides COCP Tetracycline
Shorts station: SLE
Skin: Rash - sun exposed photosenstive incl malar rash with sparing nasolabial folds. Macular / papular or maculopapular. Scaling.
Scarring alopecia. Palmar erythema, vasculitis lesions, purpura, urtricaria, livedo reticularis, telangectasia, alopecia, Raynauds, S/C nodules
Joint: Hands - symmetrical non erosive polyarthritis PIP, MCP, wrist, knee
Lung: ILD, effusion, pleurisy
CV: Pericarditis
Neuro: hemiparesis, CN lesion, MN multiplex, polyneuropathy
Renal: HTN, oedema, proteinuria/haematuria
Eyes: Sjorgen
Skin only = discoid lupus
Skin + = SLE
SLE + RA or SS or dermatomyositis = mixed CTD
Autoantibiodies in lupus
ANA (95%) Anti-DsDNA (60%, specific) Anti-Sm Ab (20%, specific) Anti Ro & La (ANA -ve cutaneous lupus) Antiphosphlipid 40%
Classification of lupus nephritis
I - Normal
II - Mesangeal GN. Degree of proteinuria, low C3. Steroids
III - Focal proliferative GN. Cyclophosphamide, azathiprine, MMF
IV - Diffuse proliferative
V - Membranous - steroids
VI - Sclerosing. Unlikely to respond to therapy
Managing SLE
Non pharm - avoid overexposure to sunlight, avoid high dose oestrogen COP, stop smoking and prevent CV disease
Steroids
NSAIDS
DMARDS - hydroxychloroquine
Cytotoxic - azathioprine, cyclophosphamide, MMF,
Livedo Reticularis - DDx
Physiological
Anti phosphlipid, SLE, cryoglubulinaemia, cold agglutinins, vasculitis, cholesterol or septic emboli
Erythema multiforme
Symmetrical distribution of target lesions (favouring palms and soles)
Note mucosal involement!!
Idiopathic 50%
TB, strep throat - group A, HSV, EBV, CMV, HIV, VZV, HAV, HBC, HCV
Enteritis - salmonella, campylobacter, yersinia
Sarcoid (lupus pernio, ILD), SLE, vasculitis, drugs
Drugs - penicillins, solphonamides, sulphonylureas, phenytoin, carbamazepine, NSAIDs, Aspirin, COCP
SJS
Variant of EM with mucosal membrane involement and severity
Vesibullous lesions with haemorrhage and necrosis
Conservative treatment - similar to burns
Thyroid examination - focussed
Examine from front - diffieculty breathing, goitre
Examine from side - exophthalmos, goitre, movement on swallowing and tongue protrusion
Examine from back - palpate swelling, ?movement on swallowing or tongue protrusion
Examine additional - temperature, pulse, thyroid acropatchy, myxoedema
Examining - acromegaly
Enlarged hands and feet. Sweatiness of palms.
Skin tags
Hyperpigmentation - acanthosis nigricans in 40% (neck and axillae)
Nails can be thick and hard
Prognathism
Macroglossia
Gynaecomastia
Other –> Bitemporal hemianopia, optic atrophy, carpal tunnel, HTN, cardiomegaly, diabetes, arthropathy, goitre, proximal myopathy
Acromegaly - causes and complications
Causes - pituitary adenoma (GH secreting). Rare: GHRH hypothalamic tumours / paraneoplastic carcinoid or SCLC
HTN, cardiomegaly, arthritis, diabetes, visual field defects, CN palsies
Acromegaly - investigation and management
GH excess - IGF (insulin like growth factor 1) in serum, OGTT (75mg glucose load, measure at 2h)
MRI to confirm
Transphenoidal resection can be curative
Medical - somatostatin analogues (like octreotide) inhibit GH release or dopamin agonists (bromocriptine, cabergoline) inhibit GH secretion
DDx painful thyroid
Thyroiditis - infectious, traumatic, radiation, subacute granulomatous
Goitre
MN diffuse - iodine deficiency, grave’s, hashimoto’s, de quervain’s
Solitary - adenoma, toxic adenoma, cysts, cancer, single palpable node in multinodular disease
DDx neck mass
Congenital - thyroglossal cyst (midline, moves on tongue protrusion)
Lymphadenopathy
Vascular - aneurysms, carotid body tumour
Salivary - salivary gland tumour, sialadenitis
Neurogenic - schwanomma, neurofibroma, nerve sheath tumours
Investigating thyroid tumours
TFTs to determine if functioning or non functioning
If TSH low - radioiodine uptake to deliniate the functioning nodule
If TSH normal/high - FNA + USS + cytology
Thyroid cancers
P apilary (most common, favourable prognosis, Rx or surgery) F ollicular (Rx or surgery M edullary (MEN 2a and b, calcitonin producing) A naplastic (bad)
Definition of myxoedema
Non pitting oedema
Causes of hyperthyroidism
Graves Toxic adenoma `Toxic Multinodular Goitre Iodine induced - amiodarone, contrast agents deQuervains (coxackie virus) Postpartum thyroiditis
Causes of hypothyroidism
AI (hashimotos) thyroiditis
Idiopathic
Previous Rx or thyroidectomy
Hypothalamic / pituitary disease
Autoantibodies in thyroid
Graves - thyrotropin receptor antibody (TRAb)
Hashimotos - anti TPO anti thyroglobulin
Pathophysiology of graves disease
AI disease - thyroid stimulating Ab which activate TSH receptors.
DDx proptosis
Graves
Cavernous sinus thrombosis
Retroorbital tumour
Managing graves disease
- Symptoms - B blocker, lubricant for eye, tape eyes at night
- Thionamides - carbimazole or PTU (aganulocytosis in 0.5%)
- Rx ablation - tissue destruction in 6-18 weeks. NOTE: may worsen exopthalmos and so can be avoided
- Surgery - thyroidectomy if large goitre or co existing nodule
AI in SS
Limited systemic - anti centromere Ab, ANA, RF
Diffuse systemic - Anti ScL 70 (topoisomerase 1); anti RNA polymerase 1, 2, and 3
Horner’s syndrome - on examination
Ptosis (partial, as upper eyelid innervated by III and Muller muscle - synpathetic), miosis, anhydrosis (dependant on site of lesion, none/face/arm/upper trunk.
Interruption of the sympathetic pathways starting at the hypothalamus and ending in the muller muscle/pupil/sweat glands
Post ganglionic lesions do not affect sweating
Horner’s syndrome - anatomy and DDx
1st order - hypothalamus –> brainstem –> cervical cord –> T1 nerve root ganglion. (demyelination, tumour, syringomyelia, infarct)
2nd order - T1 root ganglion –> cervical sympathetic chain –> superior cervical ganglion. (pancoast tumour, cervical rib, NF, CVC, anaeurysm/dissection, birth lower cervical plexus trauma)
3rd order - superior cervical ganglion –> Muller muscle, pupil and sweat glands. (herpes zoster, carotico-cavernous fistula, internal CA disseciton
Vitiligo
Well demarcated areas of depigmentation
Any area of skin and mucous membrane can be involved.
Associations with: Allopecia accreta, hashimotos, graves, addisons, hypoparathyroid, diabetes, IPF, PBC, pernicious anaemia, atrophic gastritis.
Management:
1. Arrest depigmentation - steroids (topical/systemic), topical tacrolimus, phototherapy
2. Cosmetic - sunscreen, camouflage, tattooing
SLE mnemonic
DOPAMINE RASH Discoid rash Oral ulcers Photosensitivity ANA Malar rash Immunological - dsDNA Neurological - seizures, psychosis Renal (lupus nephritis) Arthritis Serositis Haematological
Lupus nephritis classification
1 - normal 2 - mesangeal 3 - focal proliferative 4 - diffuse proliferative 5 - membranous 6 - sclerosing
Chorea vs hemibalismus vs dystonia
Chorea is a flowing movement
Hemibalismum is a flinging movement, usually 1/2 body, ususally post stroke
Dystonia is involuntary sustained muscle contracture
Torticollis - fixed or dynamic tilt, rotation, with flexion or extension of the head and/or neck
