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4.5 Cutaneous manifestations
by Dr.
128
Medical
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04/05/2012

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Term
What systemic diseases should you suspect in someone with generalized pruritis without a rash?
Definition
lymphoma (hodgkin's), endocrinopathy (diabetes, thyroid disease), renal insufficiency/ESRD, hepatobiliary disease
Term
violacious ulcer with an undermined border and a boggy base=
Definition
pyoderma gangrenosum
Term
How does pyoderma gangrenosum heal?
Definition
with cribiform scarring
Term
What are the systemic diseases associated with pyoderma gangrenosum?
Definition
hematologic disease (leukemia, monoclonal gammopathy, myelofibrosis), inflammatory bowel disease (esp Crohn's), arthritis, chronic active hepatitis, connective tissue disease
Term
Describe the appearance of aphthous ulcers?
Definition
shallow mucosal ulcerations
Term
What are the systemic diseases associated with aphthous ulcers?
Definition
SLE, Crohn disease, Behcet disease
Term
Which HLA type is associated with Behcet disease?
Definition
HLA B51
Term
Describe the cutaneous manifestations of Behcet's disease.
Definition
oral and genital aphthae, cutaneous pustular vasculitis
Term
Besides cutaneous manifestations what are the other symptoms of Behcet's disease?
Definition
uveitis, arthritis, meningoencephalitis, vasculitis (large vessel)
Term
Where is the typical location of venous leg ulcers?
Definition
medial malleolus
Term
What are teh signs of venous stasis associated with venous ulcers?
Definition
edema, coppery color, +/- eczematous changes
Term
What causes venous ulcers?
Definition
venous hypertension due to underlying valvular incompetence, fluid overload, or venous thrombosis (hypercoagulable states)
Term
Where are arterial ulcers typically located?
Definition
punched out ulcers over bony prominences (lateral foot, toes)
Term
What are teh features specific to arterial ulcers?
Definition
shiny atrophic skin and decreased pulses, change in limb color with elevation; very painful (worsens with elevation)
Term
What symptom is associated with arterial leg ulcers?
Definition
claudication
Term
What causes arterial leg ulcers?
Definition
atherosclerosis (PVD)
Term
What's another name for neuropathic ulcers?
Definition
malum perforans
Term
What are neuropathic ulcers located usually?
Definition
weight-bearing surfaces (great toe, heel, metatarsal heads)
Term
What causes neuropathic leg ulcers?
Definition
diabetes, leprosy, tertiary leus (tabes dorsalis)
Term
What is the pathology of vitiligo?
Definition
absence of melanocytes
Term
What is depigmentation associated with?
Definition
autoimmune disease (DM, thyroiditis, pernicious anemia, Addison's disease)
Term
What is the ddx for hypomelanotic macules?
Definition
"ash-leaf" macules of tuberous sclerosis versus decreased sensation in leprosy
Term
Name the three common symptoms of tuberous sclerosis?
Definition
MR, epilepsy and adenoma sebaceum
Term
What is the first sign (like at birth) of tuberous sclerosis?
Definition
ash-leaf macule
Term
Patients with tuberous sclerosis can have tumors of the...
Definition
CNS, kidneys, and heart
Term
What is the ddx for diffuse hyperpigmentation?
Definition
addison's disease, hemochromatosis ("bronze diabetes"), heavy metal ingestion
Term
Where do patients with addison's disease get hyperpigmented?
Definition
palmar creases, nails, gums
Term
What is the characteristic triad of hemochromatosis?
Definition
hyperpigmentation, cirrhosis, diabetes mellitus
Term
What types of organs are affected in NF type I?
Definition
skin, eye, CNS, osseous lesions
Term
What is Crowe's sign?
Definition
axillary freckling= pathognomonic for NF 1
Term
What are the CNS problems associated with NF type 1?
Definition
optic nerve glioma and other tumors 9usually benign), intellectual impairment in 40%, epilepsy
Term
What are the distinctive osseous lesions of NF type 1?
Definition
sphenoid wing dysplasia, pseudoarthrosis, kyphoscoliosis
Term
What is the FALCON mneuomnic for?
Definition
NF type 1: family history, axillary freckling, lisch nodules, cafe au lait macules, optic nerve glioma, osseous lesions, neurofibromas
Term
What are teh cutaneous manifestations of Peutz Jeghers?
Definition
lentigines on lips nose, buccal mucosa, hands and feet
Term
What is Peutz-Jeghers syndrome?
Definition
hamartomatous polyps of the GI tract (especially small bowel) with an increased risk of malignancy (GI and extraintestinal)
Term
What is Leopard syndrome?
Definition
lentigines, EKG conduction defects, ocular hypertelorism, pulmonary stenosis, abnormal genitalia, retarded growth, deafness
Term
Where is acanthosis nigricans found?
Definition
intertriginal areas
Term
What are the associations with acanthosis nigricans?
Definition
idiopathic (in obesity), familial, endocrinopathy (insulin resistance), malignancy (usually occurs simultaneously with tumor)
Term
What kinds of malignanceis are associated with acanthosis nigricans?
Definition
GI (stomach most commonly), breast, ovarian, prostate lung
Term
What are the underlying diseases associated with tripe palms form of acanthosis nigricans?
Definition
underlying malignancy (lung, GI, uterine)
Term
What causes melasma?
Definition
hyper-estrogen states (pregnancy)
Term
What causes the skin to have a blue-grey color?
Definition
drugs= amiodarone, tetracycline; argyria (chronic silver ingestion), chrysiasis (gold)
Term
What causes the skin to look purple?
Definition
phenothiazines
Term
What causes skin to look orange?
Definition
carotenemia
Term
What are teh dermatologic stigmata of cirrhosis?
Definition
jaundice, spider angiomata, palmar erythema, terry's nails, caput medusae, gynecomastia
Term
What are Terry's nails?
Definition
opaque white proximal nail with distal erythema
Term
How do you tell the difference between carotenemia and jaundice?
Definition
patients with carotenemia have a normal, white sclera
Term
What causes carotenemia?
Definition
sweet potatos, squash, carrots
Term
What is the appearance of erythema nodosum?
Definition
transient tender nodules on shins
Term
What causes erythema nodosum?
Definition
strep/sarcoid, paracoccidiodes, lymphoma/leukemia, OCPs, TB, Crohn's/IBD, histoplasmosis, yersinia
Term
What causes gouty tophi?
Definition
deposits of monosodium urate crystals in synovial and nonarticular tissues
Term
Where are gouty tophi most likely to occur?
Definition
elbows, knees, ears, hands
Term
Where do rheumatoid nodules occur?
Definition
often over sites of pressure
Term
What kind of xanthoma often occurs in normolipemic patients?
Definition
xanthelasma
Term
What type of xanthoma is associated with a paraproteinemia?
Definition
diffuse plane xanthoma
Term
What are the types of planar xanthomas?
Definition
xanthelasma and diffuse plane xanthoma
Term
What causes eruptive xanthomas?
Definition
very high triglyceride levels
Term
Describe the apperance of eruptive xanthomata?
Definition
yellow-brown papules on extensor extremities, shoulders, and buttocks
Term
"Plum-colored" nodules are often a sign of...
Definition
lymphoma or metastasis
Term
What is paget's disease?
Definition
can be a mammary intraductal carcinoma or an exrammary lesion (50% with underlying adenocarcinoma)
Term
What are the MC sites for internal malignancy in a patient with extramammary paget's disease?
Definition
bladder and rectum
Term
Unilateral nipple eczema is suspicious for _____.
Definition
Paget's disease
Term
In the first step of managing a patient with mycosis fungoides, it is important to...
Definition
evaluate for nodal involvment
Term
Zinc deficiency leads to what papulosquamous lesion?
Definition
acrodermatitis enteropathica
Term
What kind of lesion is associated with an underlying glucagonoma?
Definition
necrolytic migratory erythema
Term
What papulosquamous lesion suggests an underlying lung cancer?
Definition
erythema gyratum repens
Term
Some immunodeficiency syndromes can manifest as what kind of papulosquamous lesions?
Definition
atopic dermatitis
Term
What causes an underlying zinc deficiency?
Definition
inherited or aquired (consider in alcoholics)
Term
What is the morphology of acrodermatitis enteropathica?
Definition
periorificial and acral dermatitis (starts as flaccid bullae and then crusts)
Term
What lesions look like acrodermatitis enteropathica?
Definition
seb derm, intertrigo, candidiasis, psoriasis
Term
What percent of the time is a glucaconoma metastatic at time of dx?
Definition
50%
Term
What are the other signs associated with necrolytic migratory erythema to suggest glucagonoma?
Definition
diabetes, weight loss, anemia, hypoaminoacidemia, diarrhea
Term
What type of skin lesion has a wood grain appearance with rapid migration?
Definition
erythema gyratum repens
Term
What causes erythema gyratum repens?
Definition
underlying bronchogenic carcinoma
Term
What causes exfoliative erythroderma?
Definition
psoriasis, paraneoplastic, lymphoma, leukemia, atopic dermatitis, allergic contact, idiopathic, ichthyosis, drugs, sezary syndrome, seb derm
Term
What is are purpura?
Definition
gneric term for visible hemorrhagic into the skin and mucous membranes
Term
What are intravascular causes of purpura?
Definition
thrombocytopenia (idiopathic thrombocytopenic purpura, leukemia, TTP, DIC), abnormal platelet function
Term
T/F You can palpate intravascular purpura.
Definition
false
Term
What type of purpura is due to poor blood vessel support?
Definition
extravascular purpura
Term
What causes extravascular purpura?
Definition
corticosteroid excess, vitamin C deficiency, multiple myeloma, Ehlers-Danlos
Term
What are the cutaneous manifestations of scurvy?
Definition
extravascular purpura, corkscrew hairs with perifollicular hemorrhage
Term
What kind of purpura is associated with amyloidosis/multiple myeloma?
Definition
extravascular ("pinch purpura"/"raccoon eyes")
Term
Why does scurvy result in collagen defects?
Definition
because ascorbic acid is a cofactor for lysyl hydroxylase
Term
What are the cutaneous manifestations of amyloidosis?
Definition
pinch purpura, waxy papules, macroglossia, deposition of amyloid in GI, heart, kidneys, underlying multiple myeloma
Term
What is the clasic palpable purpura?
Definition
leukocytoclastic vasculitis (LCV)
Term
What causes leukocytoclastic vasculitis?
Definition
bacteria (endocarditis, RMSF), viral (hep C and cyroglobulinemia, hep B), drugs (PCNs, thiazides, PTU), CTD, malignancy, idiopathic, Henoch-Schonlein purpura
Term
What is another name for osler-Weber-Rendu?
Definition
hereditary hemorrhagic telangiectasia
Term
What diseases are associated with telangiectasias?
Definition
hereditary hemorrhagic telangiectasia, ataxia telangiectasia, scleroderma, connective tissue disease
Term
What is ataxia telangiectaseia?
Definition
combined humoral and cell-mediated immune defects, increased risk of malignancy (lymphoproliferative), and telangiectasia usually appera after ataxia is evident (involves conjunctiva, auricles, neck, extremities)
Term
Describe the telangiectasias of slceroderma?
Definition
"mat-like"
Term
Nailfold telangiectasia is a sign of ....
Definition
connective tissue disease
Term
What is a common presentation for hereditary hemorrhagic telangiectasia?
Definition
epistaxis
Term
Where are the telangiectasias of hereditary hemorrhagic telangiectasia?
Definition
mucosal and palmar telangiectasia, AVMs in GI/pulmonary tree
Term
What do you call yellow waxy translucent tleangiectatic plauqes on the lower legs associated with diabetes?
Definition
necrobiosis lipoidica
Term
What are the signs of sturge-weber?
Definition
port wine stain in V1 + ipsilateral leptomeningeal angiomatosis, may have ipsilateral glaucoma, "tram-track" calcifications along cerebral cortex
Term
What is Klippel-Trenaunay?
Definition
port wine stain + venous maliformation + hypertrophy of affectedlimb
Term
Urticaria is swelling of the ____ while angioedema is swelling of the ____.
Definition
dermis versus subcutis
Term
What's the difference between urticaria and urticarial lesions?
Definition
urticaria individual lesions lsat <2 hours; if they last more than 24 horus they are termed urticarial lesions
Term
What causes chronic urticaria?
Definition
idiopathic in 50% to 75% although some believe it is an autoimmune phenomenon (maybe due to infections, CTD, endocrinopathy, malignancy=lymphoma/leukemia)
Term
What causes angioedema without wheals?
Definition
hereditary= C1 esterase deficiency; or acquired= underlying lymphoma
Term
What causes erythema multiforme?
Definition
HSV is the MC cause!!! also, histoplasmosis
Term
Describe the lesions of erythema multiforme?
Definition
"multiform" target lesions, urticarial plaques, bullae
Term
What are the cutaneous stigmata of glucocorticoid excess?
Definition
atrophy, striae, purpura, telangiectasia, acne, lipodystrophy (moon facies, buffalo hump)
Term
What's the difference between acne due to steroids and normal acne vulgaris?
Definition
monomorphous erythematous papules and pustules without comedones
Term
What are the cutaneous signs of androgen excess?
Definition
thick coarse skin, acne, hirsutism, androgenic alopecia
Term
What is the name for hyperkeratosis on the palmar or plantar surface that is NOT due to friction?
Definition
keratoderma
Term
What is the name for the hyperkeratosis associated with menopause?
Definition
keratoderma climacterum
Term
hat is Howell-Evans?
Definition
syndrome of esophageal cancer with keratoderma of the hand
Term
Rieter's syndrome is associated with what HLA?
Definition
HLA-B27
Term
What is Reiter's syndrome?
Definition
urethritis, conjunctivitis, and arthritis with nongonnoccocal urethritis or dysentary and keratoderma
Term
What kind of poisoning causes keratosis?
Definition
arsenic
Term
What causes aquired ichthyosis?
Definition
lymphoma, HIV/AIDS, Sarcoidosis, Statins
Term
What causes onycholysis?
Definition
thyrotoxicosis, psoriasis
Term
What causes clubbing?
Definition
cardiopulmonary disease
Term
What causes mee's lines?
Definition
arsenic poisoning
Term
What causes Beau's lines?
Definition
acute illness
Term
What causes Muehrcke's nails?
Definition
hypoalbuminemia
Term
What causes half and half nails?
Definition
renal failure
Term
What causes splinter hemorrhages?
Definition
endocarditis
Term
Koilonychia is associated with...
Definition
iron deficiency anemia, polycythemia vera and plummer-vinson syndrome
Term
What do you call seperation of the nail plate from the nail bed?
Definition
onycholysis
Term
What are Mee's lines?
Definition
transverse white bands in the nail plate that move distally with nail growth
Term
What are Beau's lines?
Definition
transverse ridging associated temporarly with acute systemic stress
Term
What are Muehrcke's nails?
Definition
double white transverse bands int eh nail bed; associated with hypoalbuminemia as in nephrotic syndrome or cirrhosis; problem with nailbed so do not "grow out" with nail plate like Mee's lines
Term
Half and half nails are also known as...
Definition
lindsay's nails
Term
Describe the appearance of Lindsay's nails?
Definition
white proximal portion with a normal distal portion
Term
What causes splinter hemorrhages?
Definition
can be seen in endocarditis but more often just due to trauma
Term
What are the cutaneous manifestations of endocarditis?
Definition
osler's nodes, janeway lesions, splinter hemorrhages, LCV
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