Skin and Systemic disease Dan Flashcards
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Hepatitis B infection can be associated with urticaria, Gianotti-Crosti syndrome and polyarteritis nodosa
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40% of type II cryoglobulinaemia is HCV associated
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70-90%
Hepatitis B is associated w Necrolytic acral erythema
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Hep C
Necrolytic acral erythema appears as well-circumscribed, dusky erythematous plaques with adherent scale
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The connection between lichen planus and hepatitis C infection may actually be due to geographical variation
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What are the cutaneous features of chronic liver disease?
Acral erythema Spider naevi + telangiectasia Dilated abdominal/chest veins - caput medusa Nail – clubbing, pallor, Muehrcke’s lines Bruising, purpura Jaundice Thin skin, striae Excoriations Features of malnutrition Associated lesions - xanthomas - PCT - Vasculitis - LP
Pigmentation in haemochromatosis is due to haemosiderin deposition in the skin
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melanin
Strongest risk factors for expression of PCT are HCV infection and homozygosity for C282Y (haemochromatosis) mutation
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Jaundice is first seen as a yellowish hue of the sclerae and soft palate
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Carotenaemia also discolours the sclerae
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Acquired zinc deficiency in adults may be characterised by an reticulate eczema, erosions in the perianal and genital areas, cheilitis, hair loss and Beau’s lines
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Acrodermatitis enteropathica is AD
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AR
Cullens and Grey-Turners signs are the most common skin changes associated with pancreatitis
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Jaundice and panniculitis are
Pancreatic panniculitis can be caused by acute or chronic pancreatitis, pancreatic carcinoma, pancreatic pseudocyst, traumatic pancreatitis
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any cause of pancreatitis or pancreatic insult.
1/3 are caused by pancreatic carcinoma, so must always be aware
Trousseau’s sign describes a Sister Mary Joseph nodule when it occurs in conjunction with pancreatic carcinoma
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10% of cases of sister mary joseph nodule is a metastasis due to pancreatic carcinoma
Trousseau’s sign describes multiple, superficial migratory thrombophlebitis and is classically associated with Lung cancer (most often) and pancreatic ca (25%)
Necrolytic migratory erythema occurs in association w malignant adrenal carcinoma
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hyperglucagonaemia – glucagonoma – alpha-cell tumour in tail of pancreas
Other causes include
- pancreatic insufficiency
- intestinal malaborsoprtion or protein loss
- liver cirrhosis
- aberrant glucoagon secreting tumours – bronchial or nasopharyngeal
Glucagonomas occur in MEN syndrome
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Classically MEN type I but also MEN type II
Skin changes persist following removal of glucogonoma
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Clinical features of Necrolytic migratory erythema include an itchy, burning rash on flexural sites including lower abdomen, groin, buttocks and thighs
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Wht are systemic features of glucagonoma?
Weight loss Anaemia Diabetes Diarrhoea Weakness Venous thrombosis Psychiatric disturbances Zinc deficiency
Marked spongiosis and hyperkeratosis are typically seen in necrolytic migratory erythema
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mild spongiosis and dyskeratosis
superficial perivascular inflammation
Clefts and necrotic keratinocytes and cellular debris
Clinical features of Necrolytic migratory erythema include an itchy, burning rash on flexural sites including lower abdomen, groin, buttocks and thighs
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fragile bullae and vesicles, irregular centrifugal annular lesions which can crust
has fluctuating course of cyclical pattern
angular cheilitis and glossitis
Glucagonoma can be treated with somatostatin analogues
Octreotide – improves rash and symptoms by altering glucagon metabolism
Lantreotide can last for 2 weeks
Fabry’s disease is due to alpha-galactosidase A deficiency
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Fabry’s disease can be treated using synthetic alpha-galactosidase
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Fabrys get renal disease and other systemic manifestations due to deposition of neutral glycosphingolipids in vascular endothelium and tissue
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Exostoses arising from the posterior aspect of the iliac bones (“iliac horns”) are considered pathognomonic of Nail Patella syndrome
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occur in about 80%
considered pathognomonic
Which genodermatoses associated w renal carcinoma?
Birt-Hogge-Dube syndrome
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC, Reed syndrome)
Cowden’s disease
von Hippel-Lindau syndrome
Muir-Torre (renal pelvis transitional epithelium - all others are RCC)
Cutaneous features of systemic amyloidosis include waxy, purpuric lesions as well as hepatomegaly, oedema and carpal tunnel
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All patients who present with cutaneous vasculitis should have a urine dipstick and serum creatinine and eGFR checked
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Kyrle’s disease is a reactive perforating collagenosis associated w renal failure
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also associated w diabetes
Very itchy
Pruritus in renal failure is often associated w hyperuricaemia
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Nephrogenic systemic fibrosis is associated w gadolinium containing radiocontrast dyes used in patients with chronic renal failure
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Patients with liver transplants appear to have a higher incidence of cutaneous malignancy than renal transplant patients
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– vice versa. Due to higher immunosuppression required + inc longevity post transplant
Post-renal transplant, patients BCC risk increases up to 250 fold
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BCC risk by inc 10x
SCC risk by inc 65–250x
Uncontrolled expression of HPV infection may be a co-factor in the increased risk of SCC in immunosuppressed transplant patients
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90% of children with Kawasaki disease develop coronary artery aneurysms
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25%
The cardiac muscle is classically the only organ not affected in systemic sclerosis
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involved in 80%
What are the Stigmata of subacute bacterial endocarditis?
Septic emboli or immune complex cause skin findings
purpuric, pustular or erythematous
non-specific small vessel vasculitis
spinter haemorrhages
Roth sponts – haemorrhages in conjunctivae and retina
Oselrs nodes – small, tender, red papules on distal phalanx
Janeway lesions – faint, red, macular lesions on thenar and hypothenar eminences
What are Features of Carney complex?
Skin – naevi + blue naevi, lentigines, ephelides, myxoid neurofibromas
Pigmented adrenocortical nodules – adrenal hyperplasia and is therefore a rare cause of ACTH independent Cushing’s syndrome
Precocious puberty in females
Atrial myxomas
Also
- GH-secreting pituitary tumours
- Sertoli cell and leydig cell testicular tumours
- Thyroid cysts
- Ovarian cysts and tumours
Systemic sclerosis may be associated w fibrosing alveolitis and interstitial fibrosis
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Loeffler’s syndrome describes pulmonary infiltrates and peripheral eosinophilia and is often due to parasitic infection
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Relapsing polychondritis is due to autoantibodies against type III collagen
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type II
Dermatomyositis is usually related to anti-La antibodies
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anti-Jo1
Yellow nail syndrome occurs without associated systemic features
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pleural effusion, bronchiectasis, chronic lung infections, empyema, lymphoedema, sinus infections
Sickle cell anaemia and thalassaemia can be causes of recalcitrant lower leg ulcers
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Aquagenic pruritus appears in polycythaemia rubra vera with RBC counts above 200g/L
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aquagenic pruritus can predate the diagnosis of PCV
- follow up any aquagenic pruritus pts and reinvestigate regularly
Erythromelalgia is associated with sickle cell anaemia
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with PCV
Eosinophilia myalgia syndrome is an idiopathic fibrotic condition
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associated with ingestion of tryptophan
Wells’ syndrome may clinically mimic bacterial cellulitis, but has a dense eosinophilic infiltrate
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