Cutaneous Signs of Systemic Conditions Flashcards
1
Q
What systemic diseases often have skin symptoms?
A
- Endocrine
(diabetes, thyroid, adrenal) - Connective tissue
(lupus, dermatomyositis) - Inflammatory
(sarcoidosis, IBD, dermatitis herpetiformis) - Metabolic disease
(porphyria cutanea tarda)
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
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
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
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
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)
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!)
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
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
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)
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)
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
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
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)
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
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
- reaction to gluten in grains
- immune reaction in the small bowel alters the mucosa there and leads to antibody formation
- 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
