Cutaneous Signs of Systemic Conditions Flashcards

1
Q

What systemic diseases often have skin symptoms?

A
  1. Endocrine
    (diabetes, thyroid, adrenal)
  2. Connective tissue
    (lupus, dermatomyositis)
  3. Inflammatory
    (sarcoidosis, IBD, dermatitis herpetiformis)
  4. Metabolic disease
    (porphyria cutanea tarda)
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2
Q

Describe diabetic dermopathy

A
  • common (30%) in long standing diabetes; marker for poor diabetic control
  • lower legs, possibly trauma related
  • atrophic, pink and hyperpigmented macules and plaques
  • no effective treatment
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3
Q

Describe acanthosis nigricans

A
  • common in type II diabetes mellitus
  • marker for insulin resistance
  • velvety hyperpigmented thickening of the skin (intertriginous and at flexures… not usually on extensors or face)
  • may or may not have skin tags
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4
Q

What are the three types of acanthosis nigricans?

A
  • AN 1 (familial rare genetic syndrome)
  • AN 2 (malignancy, especially gastric and lung)
  • AN 3 (related to obesity, insulin resistance, and endocrinopathy)
    • Type II DM
    • PCOS>acromegaly (too much GH)
    • Cushing’s
    • Hypothyroidism
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5
Q

What are some common cormorbidities of diabetes mellitus?

A
  • tinea (fungus)
  • candidiasis (yeast)
  • cellulitis
  • MRSA infections
  • neuropathic ulcers
  • peripheral arterial disease (ischemia) leading to various gangrene/infection
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6
Q

Describe the general skin findings of hyperthyroidism

A
  • fine, velvety, smooth skin
  • warm and moist (increased sweating)
  • hyperpigmentation (localized or generalized)
  • pruritus
  • fine, thin hair (mild, diffuse alopecia)
  • onycholysis of nails (lifting off nailbed)
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7
Q

Describe the general skin findings of hypothyroidism

A
  • dry, rough, coarse skin (cold and pale)
  • yellow discoloration from carotenemia (esp yellow palms)
  • thick scale on feet (keratodema)
  • dull, coarse, brittle, and slow growing hair
  • thin, brittle, and slow growing nails
  • generalized boggy and edematous skin with myxedema (in very profoundly low thyroid; rare!)
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8
Q

What is pretibial myxedema?

A
  • occurs in some with Grave’s disease (1-5%)
  • cutaneous infiltration of shin skin with mucin
  • “peau d’orange”, skin colored to brown red, firm
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9
Q

What is Addison’s disease?

A
  • primary adrenocortical insufficiency (cannot make cortisol)
  • autoimmune in 80% (can be post-TB, vascular, neoplastic, or genetic)
  • difficult to diagnose; often a delay in diagnosis
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10
Q

What are the skin manifestations of Addison’s?

A
  • hyperpigmentation (increased ACTH levels, due to low cortisol; melanocyte stimulating hormone/MSH-like effect)
    • diffuse, sun-exposed, sites of trauma/scars, axillae, perineum, nipples, palmar creases, nevi, mucous membranes, hair, and nails (striate)
  • loss of ambisexual hair in post-pubertal women
  • fibrosis and calcification of the ear cartilage (rare)
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11
Q

What is cushing’s disease?

A
  • overproduction of cortisol by adrenal glands
  • moon face
  • dorsicervical fat pad
  • truncal obesity and spindly limbs
  • dermatological symptoms:
    • striae distensae
    • easy bruisability/ fragile skin
    • slow wound healing
    • acne and hirsutism (male-pattern hair)
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12
Q

What is lupus erythematosus?

A
  • multisystem disorder that can prominently affect the skin (although it doesn’t always)
  • 80% of SLE (systemic lupus erythematosus) patients have skin problems
  • SOME lupus patients only have problems with their skin (called chronic cutaneous lupus)
    • cutaneous lupus has several subsets defined by appearance, timing, and pathology
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13
Q

What are the criteria for SLE?

A

**systemic lupus erythematosus;

  • Multisystem
    • arthritis/serositis
    • nephropathy
    • CNS, hematologic, immunologic involvement
    • abnormal ANA (antinuclear Ab)
  • Skin related
    • malar (cheek) erythema
    • discoid (chronic/thick) lesions
    • oral ulcers
    • photosensitivity
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14
Q

What are some risk factors for SLE?

A

**systemic lupus erythematosus;

  • female>male (6:1)
  • young (childbearing age)>old
  • african american/dark skinned>caucasian (3:1)
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15
Q

What are 4 important subtypes of cutaneous lupus?

A
  • ACLE= acute cutaneous lupus erythematosus
  • CCLE= chronic cutaneous lupus erythematosus
    (aka discoid lupus)
  • SCLE= subacute cutaneous lupus
  • neonatal lupus
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16
Q

Describe ACLE

A

ACLE= acute cutaneous lupus erythematosus

  • resolves quickly in hours to days WITHOUT scarring
  • female:male= 8:1
  • generalized or photodistributed, exanthematous (rashy) eruption often brought on by the sun or stress but not always
  • “malar rash”= butterfly rash
  • strongly associated with SLE
17
Q

Describe CCLE

A

CCLE= chronic cutaneous lupus erythematosus

  • aka discoid lupus
  • 15-30% of SLE patients have these lesions
  • only 5% with CCLE develop SLE
  • female:male= 3:1
  • scarring of the face/ears/scalp/arms
    • 25% have lip/mouth mucosal involvement
    • atrophic/thin scarring with telangiectasia, follicular scales, and too much/too little pigment
18
Q

Describe SCLE

A

SCLE= subacute cutaneous lupus erythematosus

  • newest subset of lupus; patient may also meet criteria for SLE although many don’t
    • usually positive for different ANA (antinuclear Abs) than SLE
  • very photosensitive yet usually not on face
19
Q

Describe neonatal lupus

A
  • due to SSA/B antibodies crossing the placenta
  • often affects the face causing “racoon eyes”
    • interesting; recall mother’s reaction spares the face
  • can cause long term heart block
  • antibodies go away and rash resolves as the baby grows
20
Q

What is dermatomyositis?

A

An inflammatory disease marked by muscle weakness and skin rash

21
Q

What are the symptoms of dermatomyositis?

A
  • Skin
    • heliotrope rash (eyelids)
    • photosensitive “poikiloderma”/dermatitis (lacy thin brown/white/pink rash on trunk/extremities)
    • gottron’s papules (scaly purple papules on knuckles)
    • positive ANA
    • elevated CRP/ESR
  • Muscle
    • proximal muscle weakness
    • elevated CK and aldolase
    • abnormal EMG and MRI of muscles
    • muositis on biopsy
22
Q

What are some comorbidities for dermatomyositis?

A
  • associated with cancer in adults 10-50%
    • GU, ovarian, colon most common
    • also breast, lung, pancreatic, and lymphoma
  • may overlap with other connective tissue diseases
  • interstitial lung disease may be fatal
23
Q

What is sarcoidosis?

A
  • caused by noncaseating granulomas in multiple organ systems (skin in 25% of cases)
  • incidence is higher in African Americans and men, with onset typically in young adulthood
24
Q

What are the clinical symptoms of sarcoidosis?

A
  • most common presentation is red-brown macules and papules on the face **Around eyes and nose
  • Erythema nodosum may be associated
  • organ involvement can be asymptomatic, all patients with cutaneous sarcoidosis should undergo a work-up for internal disease
  • “The great pretender”; can look like a lot of diseases
25
Q

What is Lofgren’s syndrome?

A

Very common syndrome of sarcoidosis;

  • hilar adenopathy (without lung disease)
  • erythema nodosum
  • fever
  • iritis, arthritis

**often will resolve

26
Q

What are the clinical symptoms of inflammatory bowel disease?

A
  • Specific (diagnostic)
    • Crohns disease with granulomas in the skin
    • involvement of oral mucosa with granulomatous inflammation
    • fistulae to skin
  • Non-specific
    • erythema nodosum
    • pyoderma gangrenosum (fast moving, rapidly ulcerating skin)
    • nutrional problems
    • other
27
Q

What is the mechanism of dermatitis herpetiformis?

A
  1. reaction to gluten in grains
  2. immune reaction in the small bowel alters the mucosa there and leads to antibody formation
  3. antibodies can enter blood and attach to skin (anti-transglutaminase Abs)

**Results in DH; a rediculously pruritic rash on extensor surfaces

28
Q

What if the treatment for dermatitis herpetiformis?

A
  • best treatment= avoidance of gluten… after 3 months;
    • TTG Ab levels decrease
    • mucosa of gut normalizes
    • symptoms of DH abate
  • dapsone is effective treatment but does not effect the gut or production of autoantibodies
29
Q

What is porphyria cutanea tarda?

A
  • a metabolic disease caused by defective uroporphyrin decarboxylase (breaks fown heme proteins)
  • common causes (all due to enzyme stress);
    • Hep C
    • alcohol and some drugs
    • hemachromatosis (iron overload)
  • causes fragile blisters from trauma and sun on hands especially