Cutaneous signs of systemic conditions Flashcards
What skin conditions are often seen with diabetes mellitus type II
Acanthosis nigricans
Diabetic dermopahty
tinea fungus
candidiasis
cellulitis
MRSA infections
peripheral neuropathy-neuropathic ulcers
vascular disease-peripheral arterial disease/ gangrene
What is diabetc dermopathy?
- common, 30% in long standing diabetes,
- lower legs, possibly trauma related
- atrophic pink and hyperpigmented macules and plaques (looks like scars)
- marker for poor diabetic control
- no effective treatment
What is acanthosis nigricans? what is it a marker of and what is seen?
- common marker in type II DM
- marker for insulin resistance
- velvety hyperpigmented thickening of the skin
- intertriginous (inguinal/axillary folds)
- flexures
- not usually extensor surfaces or face
- sometime skin tags
WHat are the 3 types of acanthosis nigricans and what are they associated with?
AN1-familial
AN2-malignancy (especially gastric and lung)
AN3- related to obesity , insulin resisitance and endocrinopathy (Type II DM, PCOS, cushings, hypothyroidism)
What symptoms are often associated with hyperthyroidism?
- fine, velvety, smooth skin
- warm and moist increased sweating
- hyperpigemntation: localized or generalized
- pruitus
- fine, thin hair (mild, diffuse alopecia)
- onycholysis ( of nails)
what symptoms are associated with hypothyroidism?
- dry, rough, course skin
- cold and pale skin
- yellow discoloration from carotenemia
- thick scale on feet (keratoderma)
- generalized boggy edematous skin (myxedema)
- dull, coarse, brittle, slow growing hair
- alopecia of the lateral third of eyebrows
- thin, brittle slow growing nails
What is graves diseases associated with?
Pretibial myxedema
- occurs in some with Grave’s disease (about 1-5% but not everyone with hyperthyroidism)
- cutaneous infiltration of skin of shins with MUCIN
- “Peau d’ orange” skin colored to brown red, firm
- can occur during Grave’s or following treatment
What is addison’s disease?
- primary adrenocortical insufficiency (cannot make cortisol)
- autoimmune in 80%- autoantibodies (vs post-TB, vascular, neoplastic, gentic)
- delay in diagnosis common bc difficult to diagnose
What are the skin manifestations association with addison’s disease?
- hyperpigmentation (MSH like effect of ACTH which builds up bc adrenal gland isn’t making cortisol)
- diffuse, sun-exposed, sites of trauma, axillae, perineum, nevi, mucous membranes, hair, nails
- loss of ambisexual hair in ost-pubertal women
- fibrosis and calcification of the cartilage (ear)
What is cushing’s disease?
overproduction of cortisol by adrenal gland?
- Moon facies
- Dorsicervical fat pad (“buffalo hump”)
- truncal obesity
- spindly limbs
- striae distensae
- easy bruisability
- slow wound healing
- acne and hirsutism
What are 2 connective tissue diseases?
lupus
dermatomyositis
What are the 2 facets to lupus erythematosus effects?
- multisystem-disorder that can prominently affect the skin
- cutaneous- lupus has several recognizable subsets defined in part by apperance, timing and pathology
- over 80% of systemic lupus (SLE) patients haev problems with skin
- some lupus patients (chronic cutaneous lupus) ONLY have problems with skin
What are the skin related symptoms wth SLE (systemic)
malar (cheek) erythema
discoid (chronic/thick) lesions
oral ulcers
photosensitivity (sun sensitivity)
WHat are some multisystem effects of SLE?
arthritis
serositis
neohrology
CNS disorder
hematologic disorder
immunologic disorder
abnormal ANA
WHat are risk factors for SLE?
Femal
young (childbearing age)
African American
What are the subtypes of lupus?
- Acute cutaneous lupus erythematous (ACLE)
- Chronic cutaneous lupus erythematous (CCLE)
- aka discoid lupus
- subacute cutaneous lupus (SCLE)
- Neonatal lupus
What is seen in people with acute cutanous Lupus erythematous (ACLE)
- acute=resolves quickly in hours to days without scarring
- femal: male is 8:1
- generalized or photodistributed, exanthematous (rashy) eruption
- often brought on by sun but not always
- “malar rash”=”butterfly rash”
- strongly associated with SLE
Who gets chronic cutaneous lupus erythematosus (CCLE)?
aka discoid lupus
- most common form of CCLE
- 15-30% of SLE pts have some of these lesions
- only 5% of those with CCLE develop systemic lesions
- female:male is 3:1
What is seen with CCLE?
- usually face/ears/scalp/arms (or generalized)
- mucosal (lips and mouth) involvement in 25%
- atrophic (thin) scarring with
- telangiectasia (blod vessels)
- follicular scales
- too much or too little pigment
- leaves scars! makes it challenging esp if it scarring alopecia
What is seen with SCLE= subacute cutaneous lups erythematous
- newest subset of skin disease in lupus, described in 1979
- may also meet ARA criteria for SLE
- usually positive for different ANA types than SLE
- polycyclic patchesvery photosensitive yet usually not on face
Describe neonatal lupus
due to SSA/B antibodies crossing the placents
- can cause heart block
What is seen with dermatomyositis of the skin?
- “Heliotrope rash” (eyelids)
- photosensitive “poikiloderma” /dermatitis
- Gottron’s papules (scale purple papules on knuckles)
- positive ANA
- elevated CRP/ESR
**autoimmune, UV triggered
What is seen with dermatomyositis of the muscle
proximal muscle weakness
elevated CK, aldolase and other
abnormal EMG
Abnormal MRI of muscles
Myositis on biopsy
What is this?
heliotrope rash of eyelids associated with dermatomyositis
Wat is this lacey, thin, brown, white, pink pink rash?
poikiloderma rash on trunk and extremeties associated with dermatomyositis
relationship between dermatomyositis and cancer
- associated with cancer in adults 10-50%
- GU, Ovarian, colon
- also breast, lung, pancreatic and lymphoma
- always consider ovarian in women with DM
- May overlap with other connective tissue diseases
- interstitial lund disease may be fatal
How is sarcoidosis considered a multisystem disease
pulmonary (905) can be asymptomatic
skin
lymphatic/hematologic
liver/spleen
ocular
cardiac
musculoskeletal
CNS and nerve palsies
exocrine glands (parotid)
endocrine
constitutional symptoms and signs
what is a key characteristic seen with sarcoidosis
non-caseating granulomas
*8usually persents with red-brown macules and papules on the face aroun the nose and eyes
when is cutaneous disease most commonly seen in the progression of sarcoidosis
onset of disease. if you see it also look for systemic disease
What is physically seen with cutaneous sarcoidosis?
- brown-red papules and plaques are characteristic
- a “great pretender” can look like a lot of things
- use diascopy to reveal the brown red color
- can see periorbital granulomatous papules
what syndrome is associated with sarcoidosis?
Lofgren’s syndrome:
- hilar adenopathy
- erythema nodosum
- fever
- iritis, arthritis
What is characteristic of specific Inflammatory Bowel disease (chrons disease and ulcerative cholitis)
- chrons disease with granulomas in the skin
- involvement of oral mucosa with granulomatous inflammation
- fistulae to skin
What is non-specific to IBS (Chrons and ulcerative colitis)
erythema nodosum
pyoderma gangrenosum
nutritional problems
what is pyoderma gangrenosum?
- a streile, rapid ulceration of the skin caused by neutrophili infiltration
- characteristic undermined, dusky border
- associated with IBD but also many other conditions including inflammatory arthritis, hematologic problems and malignancies. also idiopathic
What is dermatitis herpetiformis?
- less common than celiacs
- bowel disease can be asymptomatic
- reaction in grains leads to immune reaction in the small bowel which alters the mucosa there
- this leads to antibody formation and antibodies enter blood and attach to skin (antitransglutaminase antibodies) which leads to dermatitis and herpetiforms
- ridiculously pruritic
What is the treatment for dermatitis herpetiforms?
- complete avoidance of gluten
- after 3 months: TTG antibody levels decrease, mucosa of gut normalizes, symptoms of DH abate
- dapsne is effective treatment for DH itching but does not effect gut or production of autoantibodies
What causes porphyria cutanea tarda and what does it lead to?
- caused by defect uroporphyrin decarboxylase which breaks down heme proteins
- OR:
- hep C
- alcohol
- hemachromatosis
- some drugs
- enzyme stress
- leads to: fragile blisters (bullae and scars) from trauma and sun on hands especially
