Skin and Systemic disease Dan Flashcards
T/F
Hepatitis B infection can be associated with urticaria, Gianotti-Crosti syndrome and polyarteritis nodosa
T
40% of type II cryoglobulinaemia is HCV associated
F
70-90%
Hepatitis B is associated w Necrolytic acral erythema
F
Hep C
Necrolytic acral erythema appears as well-circumscribed, dusky erythematous plaques with adherent scale
T
The connection between lichen planus and hepatitis C infection may actually be due to geographical variation
T
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
F
melanin
Strongest risk factors for expression of PCT are HCV infection and homozygosity for C282Y (haemochromatosis) mutation
T
Jaundice is first seen as a yellowish hue of the sclerae and soft palate
T
Carotenaemia also discolours the sclerae
F
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
T
Acrodermatitis enteropathica is AD
F
AR
Cullens and Grey-Turners signs are the most common skin changes associated with pancreatitis
F
Jaundice and panniculitis are
Pancreatic panniculitis can be caused by acute or chronic pancreatitis, pancreatic carcinoma, pancreatic pseudocyst, traumatic pancreatitis
T
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
F
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
F
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
T
Classically MEN type I but also MEN type II
Skin changes persist following removal of glucogonoma
F
Clinical features of Necrolytic migratory erythema include an itchy, burning rash on flexural sites including lower abdomen, groin, buttocks and thighs
T
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
F
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
T
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
T
Fabry’s disease can be treated using synthetic alpha-galactosidase
T
Fabrys get renal disease and other systemic manifestations due to deposition of neutral glycosphingolipids in vascular endothelium and tissue
T
Exostoses arising from the posterior aspect of the iliac bones (“iliac horns”) are considered pathognomonic of Nail Patella syndrome
T
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
T
All patients who present with cutaneous vasculitis should have a urine dipstick and serum creatinine and eGFR checked
T
Kyrle’s disease is a reactive perforating collagenosis associated w renal failure
T
also associated w diabetes
Very itchy
Pruritus in renal failure is often associated w hyperuricaemia
T
Nephrogenic systemic fibrosis is associated w gadolinium containing radiocontrast dyes used in patients with chronic renal failure
T
Patients with liver transplants appear to have a higher incidence of cutaneous malignancy than renal transplant patients
F
– vice versa. Due to higher immunosuppression required + inc longevity post transplant
Post-renal transplant, patients BCC risk increases up to 250 fold
F
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
T
90% of children with Kawasaki disease develop coronary artery aneurysms
F
25%
The cardiac muscle is classically the only organ not affected in systemic sclerosis
F
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
T
Loeffler’s syndrome describes pulmonary infiltrates and peripheral eosinophilia and is often due to parasitic infection
T
Relapsing polychondritis is due to autoantibodies against type III collagen
F
type II
Dermatomyositis is usually related to anti-La antibodies
F
anti-Jo1
Yellow nail syndrome occurs without associated systemic features
F
pleural effusion, bronchiectasis, chronic lung infections, empyema, lymphoedema, sinus infections
Sickle cell anaemia and thalassaemia can be causes of recalcitrant lower leg ulcers
T
Aquagenic pruritus appears in polycythaemia rubra vera with RBC counts above 200g/L
F
aquagenic pruritus can predate the diagnosis of PCV
- follow up any aquagenic pruritus pts and reinvestigate regularly
Erythromelalgia is associated with sickle cell anaemia
F
with PCV
Eosinophilia myalgia syndrome is an idiopathic fibrotic condition
F
associated with ingestion of tryptophan
Wells’ syndrome may clinically mimic bacterial cellulitis, but has a dense eosinophilic infiltrate
T
Hypereosinophilic syndrome has eosinophilis in excess of 1500/uL w no apparent cause for greater than 6/12
T
Can have endomyocardial fibrosis, eosinophilic vasculitis, thrombosis, cutaneous lesions
Late onset CAH is the most common AR disorder in humans
T
which skin diseases are associated w leukaemia?
Sweet’s syndrome Neutrophilic dermatosis of dorsal hands Pyoderma gangrenosum Subcorneal pustular dermatosis EED Neutrophilic eccrine hidradenitis Vasculitis Eosinophilic pustular folliculitis erythroderma JXG w NF-1 - juvenile chronic myelomonocytic leukaemia Leukaemia cutis Relapsing polychondritis - MDS
Leukaemia cutis is more common in chronic leukaemia than acute
F
more common in acute [AML]
Although can occur in any chronic and may herald a blast transformation
Coumarin necrosis is due to a temporary reduction in levels of vitamin K dependent natural anticoagulants causing a temporary state of thrombophilia
T
Warfarin necrosis is more common in Males
F
females
Which Genodermatoses are associated w haematological malignancy?
Chediak higashi Wiskott Aldrich NF-1 + JXGs Dyskeratosis congenita Fanconi’s anaemia Ataxia telangiectasia Werner's, Bloom's and Rothmund-Thomson
Erythematous plaque or urticated lesion with expands with central clearing occur in erythema chronicum migrans
T
After the initial lesion, crops of vesicles can develop at sites distant to erythema chronicum migrans site
F – lesions resembling EAC or EM can develop. Smaller than initial lesion
Histologically erythema chronicum migrans shows superficial and deep perivascular lymphocytic infiltrate around vessels and appendages w occasionally plasma cells
T
erythema marginatum rehumaticum clinically consists of rings or segments of rings, pale or dull red in colour which can fade in a few hours and occur in recurrent crops in different sites
T
Erythema gyratum repens is always associated with malignancy
F
80% of the time
Has also been associated with CREST syndrome, tuberculosis, drug hypersensitivity
Erythema gyratum repens is most commonly associated w lung cancer
T – 33%
then bowel, oesophagus, urogenital, breast, pancreas, haematological
Erythema gyratum repens is thought to be immunologically mediated
T – supported by deposition of C3 or IgG in sublamina densa
Erythema gyratum repens clinically resembles the grain of wood
T
DDx for Erythema gyratum repens
TEN LL T = Tinea imbricata E = Erythrokeratoderma variabilis N = Necrolytic migratory erythema Lupus Lapiere's Subacute annular variant of psoriasis
Annular erythema of infancy is really just EAC in children
T
In annular erythema of infancy and infective source can be sought and treated, often with a good response of cutaneous lesions
T
Annular erythema associated with extractable nuclear antigens is seen in Lupus, Sjogren’s disease, Neonatal lupus
T
and is associated with anti-SSA and anti-SSB antibodies
Common associations with erythema annulare centrifugum are fungal infections, other viral infections, drugs, lymphoma
T
Histologically EAC shows perivascular ‘sleeve-like’ lymphohistiocytic infiltrate
T
‘coat sleeving’
Erythema annulare centrifigum appears as a small, pink, infiltrated papule, which gradually enlarges as a ring
T
and spreads leaving a trailing scale
What is the treatment of Treatment for EAC?
Discovery and elimination of cause if possible Antihistamines Topical corticosteroids Vitamin D Narrow-band UVB ? systemic immunosuppressants
Facial features of acromegaly include prognathism, frontal bossing, oily skin, large and furrowed tongue, triangular ears
T Also skin shows; Acanthosis nigricans Addisonian like pigmentation due to increased MSH Cutis verticis gyrata (secondary)
Pituitary insufficiency may manifest as hyperpigmentation due to uncontrolled secretion of MSH
F
Decreased MSH – hypopigmentation, but mucous membranes retain normal colour
Also inc sunburning and lack of tanning
Pituitary Cushing’s disease causes hyperpigmentation as secretion of pro-opiomelanocortin causes overproduction of ACTH and MSH
T
What are screening tests for Cushing’s disease
8 am cortisol
24-hour urinary cortisol repeated 2x or
1mg overnight dexamethasone suppression tests
- values 4 x normal
Nelson’s syndrome is eponymous for empty sella syndrome
F
Nelson’s syndrome is the rapid growth of pituitary adenomas following adrenalectomy
Secondary adrenal insufficiency due to hypothalamic or pituitary insufficiency lacks pigmentary changes
T
Describe features of Addisonian pigmentation
Slow onset
Light exposed areas – face, dorsa of hands
Accentuation in genital, perineum, axillae, areolae, any areas subject to friction (eg elbows, knees, under clothing)
Scars
Tongue, mucous membranes
Darker hair and longitudinal melanonychia
Eruptive lentigines and darkening existing pigmented lesions
Addisonian pigmentation can be seen in primary adrenal insufficiency, Nelson’s syndrome, tumour casuing ectopic ACTH secretion, Cushings disease
T
In congenital adrenal hyperplasia and late onset adrenal hyperplasia 17-hydroxyprogesterone, cannot be converted to pogesterone
F
cannot be converted 11-deoxycortisol
Congenital adrenal hyperplasia is associated with endogenous steroid induced acne
F acne in CAH is due to excessive androgens Other symptoms include Premature pubic hair Hirsuitism Acne Temporal baldness Infertility Accelerated growth spurt and diminished final height in males and females
What are Nail changes seen in hyperthyroidism?
Thin, onycholysis (Plummer’s nails), fast growing
Plummer’s = onycholysis + koilonychias; begins on 4th finger
Diamond’s triad which is associated w Graves disease refers to what?
pretibial myxoedema, thyroid acropachy, exophthalmos
Pretibial myxoedema develops in patients with hypothyroidism
F
hyperthyroidism
Histologically pretibial myxoedema may be seen as a thickened dermis with increased collagen
F
thickened dermis, w extensive deposits of acid mucoplysaccharides acausing separation of collagen fibres
Thyroid acropachy refers to the eye changes seen in Graves disease
F
clubbing of fingers and toes in associated w soft tissue swelling of hands and feet w/ periosteal bone formation
Cutaneous findings of hypothyroidism include pale, cold scaly skin w asteatotic eczema and anhidrosis
T
Loss of the medial third of the eyebrow – Hertog’s sign, is common in hypothyroid patients
F
loss of lateral third
The main dermatological clinical signs seen in parathyroid disease are related to calciphylaxis and ossification
T
or angiofibromas if MEN1
Paget’s disease of the breast is thought to be due to direct epidermal extension of an underlying ductal squamous cell carcinoma
F
adenocarcinoma
25% of EMPD have underlying cancer
T
Extramammary paget’s disease can be from direct extension from carcinoma of underlying apocrine glands or from a distant tumour
T
Most common source of cutaneous metastases are upper aerodigestive tract
F
melanoma and breast ca
Sister Mary Joseph’s nodule is most commonly related to bowel tumours
T
Cutaneous metastases usually have a poor prognosis and indicate disseminated disease
T
What neoplasias are associated w Gorlins syndrome?
BCCs Rhabdomyosarcoma Medulloblastoma (in 3% of Gorlins. Also 3% of medulloblastoma pts have Gorlin’s) Astrocytoma Meningioma Fibrosarcomas Uterine fibroids, desmoids and sarcomas Ovarian fibroma Cardiac fibroma
CDKN2A mutation is associated with melanoma and renal cell carcinoma
F Not RCC. Melanoma Breast Thyroid GIT SCCs Larynx Pancreas Ocular
Cowden’s syndrome is not associated w increased risk of cutaneous malignancy
F
Inc melanoma risk
also thyroid, breast, uterine, GU
Muir Torre syndrome is associated with increased risk of thyroid carcinoma
F
Uterine cancer is the cancer most strongly associated with Acanthosis nigricans
F
gastric adenocarcinoma
Tripe palms alone (esp with clubbing) are indicative of bronchial carcinoma
T
Sign of Lesar-Trelat (eruptive Seborrheic keratosis) is highly indicative of internal malignancy
F only weak association May also occur in HIV infection, acromegaly and in resolving phase of erythrodermic dermatoses - MEAL M = Malig E = Erythroderma A = AIDs L = Lymphoproliferative 20%
Acrokeratosis Neoplastica effects the hands and feet only
F
all peripheries inc tip of nose helices of ears, hands and feet and may then become generalised
Bazex syndrome (AN) can occur without underlying malignancy
F
this is a required criterion for diagnosis
- most common in SCC or upper respiratory tract or GIT and w metastases to cervical lymph nodes
Multicentric reticulohistiocytosis is associated with solid tumour
T
25% of cases
Papules around nail folds – coral bead sign, symmetrical polyarthritis
Which haematological disorder is erythromelalgia linked with?
Polycythaemia rubra vera
Essential thrombocytaemia
Actinic cheilitis is a cutaneous manifestation of Crohn’s disease
F
Granulomatous cheilitis is
What are skin features associated with IBD?
HS EBA (rare) Erythema nodosum Aphthous ulceration Erythema multiforme Urticaria and angio-oedema Neutrophilic dermatosis – pyoderma gangrenous and sweets + Pyodermatitis-pyostomatitis vegetans Vasculitis and intravascular coagulation Sarcoid-type lesions at remote sites Granulomatous cheilitis Non specific skin changes secondary to malabsorption Anal and perianal conditions o Maceration o Erosion o Pruritis ani o Sin tags o Secondary infection o ‘metastatic’ granulomatous plaques o Perianal abscess o Fissure in ano o Fistula in ano Spreading ulceration of perineum and buttocks post colectomy Skin changes around stoma sites (PG or cur Crohns) Genital lesions o Men - Balanitis - Posthitis - Chronic penile lymphoedema and granulomatous lymphangiitis - Women - Vulval lesions
What are Bywater’s lesions?
Feature of RA
Nailfold thrombosis and infarction causing purpuric macules/papules on distal digits (esp. digital pulp) they are painless
Pyoderma gangrenosum is more common in patients with ulcerative colitis than those with Crohn’s disease
T
5% of all UC pts and parallels the GI disease
erythema nodosum is alos more often seen in UC than Crohns
Whats the pancreatic panniculitis and polyarthritis syndrome?
Pancreatitis
Arthritis (60%)
Nodular perforating panniculitis
Cancer of pancreas – more common cause than pancreatitis
Radiographic changes – osteolytic bone lesions from marrow necrosis (10%)
Eosinophilia – Schmidt’s triad is arthritis, nodules and eosinophilia
Amylase, lipase and triptase elevated
Serositis – pleuropericarditis often w/ fever
What is Jellinek’s sign?
hyperpigmentation of eyelid skin in hyperthyroidism
What is Pemberton’s sign?
red face on raising arms due to a large retrosternal goitre compressing the vena cava and obstructing venous return to the heart when the arms are raised
What is Maroni’s sign?
erythema of skin over the thyroid in hyperthyroidism
What is EMO syndrome?
Exophthalmos, pretibial Myxoedema, Osteoarthropathy; a rare complication of Graves disease
exopthalmos is rarely seen in pts with pretibial myxoedema
F
almost always seen
if these are present look for acropachy (Diamonds) and for osteoarthropathy (EMO)
Diabetic cheiroarthropathy does not respond to improved glycaemic control
F
gets better after period of tight control
Plummer-Vinson Syndrome is atriad of;
Dysphagia + iron deficiency + esophageal webs
T
middle aged women; rare
What are the mucocutaneous findings of Plummer-Vinson Syndrome?
Brittle nail koilonychias Angular chelitis Pale atrophic buccal mucosa Leukoplakia Glossitis
Hermansky Pudlak, Griscelli and Chediak Higashi syndromes are all at risk of haem malignancy
F
Only Chediak Higashi is
Other 2 are at risk of;
hemophagocytic lymphohistiocytosis - a type of cytokine storm involving lymphocytes and macrophages
Which derm conditions are associated with bowel polyps?
Peutz-Jeghers syndrome Gorlin's Gardner’s syndrome Cowden’s disease (multiple hamartoma and neoplasia syndrome) Bannayan-Riley-Ruvalcaba syndrome Cronkhite-Canada Syndrome (non-inherited) Birt-Hogg-Dube syndrome Neurofibromatosis
Which derm conditions are associated with bronchiolitis obliterans?
Paraneoplastic pemphigus
chronic GVHD
Sweets
Which genoderms get lipomas?
Cowdens Bunyan –Riley- Ruvalcaba syndrome Neurofibromatosis Gardner’s syndrome Proteus syndrome MEN 1 \+ specific lipomatosis syndromes
What dermatoses are associated with Hep B or C?
Small vessel vasculitis (B, C) Serum sickness-like reaction (B, C) Urticaria (B, C) Porphyria cutanea tarda (B, C) Pruritus (B, C) Erythema multiforme (B, C) Erythema nodosum (B>C) Polyarteritis nodosa (B [classic]>C) Gianotti–Crosti syndrome (B>C) Sarcoidosis (with interferon and/or ribavirin therapy; C>B) Cryoglobulinemic vasculitis (C>B) Necrolytic acral erythema (C) Lichen planus – particularly erosive oral disease (C) Livedo reticularis (C)
What are the derm associations of coeliac disease?
DH Psoriasis IgA pemphigus Alopecia Mouth ulcers Thrombocytopenic purpura Systemic associations with possible skin manifestations; SLE RA Sjogrens Sarcoidosis Diabetes AI thyroid disease Addisons Anaemia Autoimmune hepatitis, PBC, PSC MS and neuropathies
