8 - Cutaneous Signs of Systemic Disease Flashcards
What are some endocrine-related diseases?
- Diabetes
- Hyperthyroidism (including grave’s disease)
- Hypothyroidism
- Addison’s disease (adrenal insuff.)
- Cushing’s disease
What are some skin signs of diabetes mellitus?
- Diabetic dermopathy
- Acanthosis nigricans
- Finea (fungus)
- Candidiasis (yeast)
- Cellulitis
- MRSA
- Neuropathic ulcers
- Peripheral arterial disease (ishemia)
- Various gangrene
What is diabetis dermopathy? Who gets it and where?
Common (30%) in long standing diabetes; marker for poor diabetic control (no effective treatment).
Lower legs, possibly trauma related.
Atrophic, pink and hyperpigmented macules and plaques that look like scars.
How common is acantosis nigricans? What does it look like and what is it a marker of?
Common in type II D; marker for insulin resistance.
Velvety hyperpigmented thickening of the skin. Intertriginous, flexors (not usually extensor or face).
+/- skin tags.
What are the three broad categories that can cause acanthosis nigricans?
AN1: familial
AN2: malignancy (gastric and lung)
AN3: related to obesisty, insulin resistance, and endorinopathy such as T2DM, PCOS, Cushings, and hypothyroidism
What other skin findings are commonly associated with diabetes mellitus DM?
Tinea (fungus), candidiasis (yeast), MRSA infections, and cellulitis.
What peripheral afflictions occur with DM?
Neuropathic ulcers, peripheral arterial disease (ischemia), and various gangrene (ischemia + infection).
What might the skin of someone with hyperthyroidism look/feel life?
- Fine, velvety, smooth skin
- Warm and moist due to increased sweating
- Hyperpigmentation - localized or generalized
- Pruritus
- Hair: fine, thin
- Onycholysis (lifting)
There are not characteristic of hyperthyroidism - many things could cause these.
What might the skin of someone with hypothyroidism look/feel life?
- Dry, rough, coase skin
- Cold and pale skin
- Yellow discoloration from caotenemia
- Thick scale on feet (keratoderma)
- Generalized boggy and edematosu skin (myxedema) - rare
- Hair: dull, coarse, brittle, slow to grow
- Alopecia of the later third of eyebrows
- Nails: thin, brittle, slow to grow
These are not characteristic, many things other than hypothyroidism can cause these.
What skin symtoms is seen in 1-5% of those with hyperthyroidism WITH grave’s disease?
Pretibial myxedema: cutaneous infiltration of skin of shins (rare on other sites) with MUCIN (which accumulates)
Peau d/orange, skin colored to brown red; firm
Can occur from Graves or following treatment.
What is addison’s disease? What is it caused by?
Primary adrenocortical insufficiency (can’t make cortisol)
Autoimmune in 80% (autoantibodies) - opposed to postTB, vascular, neoplstic, genetic.
Difficult to diagnose.
What skin manifestations are seen with Addison’s disease?
Hyperpigmentation (melanocyte stimulating hormone-like effect on ACTH) and mucosal pigmentation- diffuse
Loss of ambisexual hair in post-pubertal women
Fibrosis and calcification of the cartilage (ear) - rare
What is Cushing’s disease/syndrome? What are common characteristics of someone with Cushings?
Overproduction of CORTISOL by the adrenal gland.
- Moon face
- Dorsicervical fat pad (buffalo hump)
- Truncal obesity
- Spindly limbs
- Striae
- Easy bruisability
- Slow wound healing
- Acne and hirsutism (abnormal hair growth).
What are two connective tissue diseases?
Lupus and dermatomyositis
What is lupus? What is the spectrum of disease?
MULTISYSTEM disorder that can prominently affect the skin; has several subsets that are defined by appearance, timing, and pathology.
~80% of systemic lupus (SLE) pts have problems with skin; some (chronic cutaneous lupus) ONLY have problems with their skin.
What are the american college of rheumatology SKIN criteria for SLE (systemic)?
Malar (cheek) erythema
Discoid (chronic, thick) lesions
Oral ulcers
Photosensitivy
Pts must have 4 or more points (one for each affliction) in addition to the multisystem effects, to have a diagnosis of SLE systemic lupus.
What are the american college of rheumatology MULTISYSTEM criteria for SLE (systemic)?
Arthritis
Serositis (serous membrane inflamm)
Nephropathy
CNS disorder
Hematologic disorder
Immunologic
Abnormal ANA (antinucleas antibodies)
Pts must have 4 or more points (one for each affliction) in addition to the skin effects, to have a diagnosis of SLE systemic lupus.
What are risk factors for SLE (ie who is more likely to get this)?
Female > make (6:1)
Young (childbearing age) > old
African american, other dark skin > caucasion (3:1)
What are the subtypes of lupus?
Acute cutaneous lupus erythematosis (ACLE)
Chronic cutaneous lupus erythematosus (CCLE) - aka discoid
Subacute cutaneous lupus (SCLE)
Neonatal lupus
Who gets acute cutaneous lupus erythematosus (ACLE)? How long does it last? What is it associated with?
Females > males (8:1)
Acute - resolves quickly in hrs to days WITHOUT scarring.
Strongly associated with SLE development.
What rash is seen with acute cutaneous lupus erythematous (ACLE) and SLE?
Generalized or photodistributed exanthematous (rashy) eruption; often brought about by sun but not always.
“Malar rash” = butterfly
(malar rash seen with ACLE and SLE)
Who gets chronic cutaneous lupus erythematosis (CCLE)? What is the likelihood that they will develope SLE?
AKA discoid lupus; most common form.
Female > male (3:1)
Only 5% of those with CCLE develope SYSTEMIC SLE (especially likely if lesions are widespread).
15-30% of SLE patients have some of these lesions.
Where do you see lesions caused by chronic cutaneous (discoid) lupus erythematosus? What can these cause?
Face, ears, scalp, arms.
Mucosal (lips and mouth) involvement in 25%
Atrophic (thin) scarring with telangiectasia, follicular scales, too much or too little pigment.
Leaves scars! (challenging with scarring alopecia because hair will not grow back)
____ alopecia is seen in discoid lupus.
Scarring alopecia
What is subacute cutaneous lupus erythematosus? Where does it occur and what does it look like?
Newest subset of skin disease in lupus; may also meet criteria for SLE.
Usually positive for different ANA (antinuclear antibody) types than SLE.
Annular, polycyclic rash. Very photosensitive; not usually on face.
What causes neonatal lupus? Why is this dangerous?
SSA/B antibodies crossing the placenta from mom with subacute cutenaous lupus erythematosus (ECLE) to baby.
Puts baby at risk for heart block.
What are characteristics of dermatomyositis of the skin?
- Heliotrope rash (eyelids)
- Photosensitive “poikiloderma”/dermatitis
- Gottron’s papules (scaley purple papules on knuckles)
- Positive ANA (+/-)
- Elevated CRP/ESR
What are characteristics of dermatomyositis of muscle?
- Proximal muscle weakness
- Elevated CK (creatinine kinase) and aldolase
- Abnormal EMG (electromyography)
- Abnormal MRI of muscles
- Myositis on biopsy
What is dermatomyositis associated with? (ie why do we can about it)?
Cancer in adults 10-50%
GU, ovarian, and colon most common; also breast, lung, pancreatic, and lymphoma
Always consider OVARIAN cancer in women with DM.
Other than cancer, what else can dermatomyositis be associated with?
Diabetes mellitus (may overlap with other connective tissue disorders)
Interstitial lung disease - may be fatal
What organ systems are most commonly involved in sarcoidosis?
Pulmonary (90%) - can be asymptomatic
Skin (20-35%) - easy to biopsy
Pretty much able to involve any organ.
What is seen histologically in someone with cardoidosis?
Non-caseating granulomas - ie granulomas with no necrosis in the center
What is the cutaneous disease of carcoidosis most commonly seen? What does this indicate that you should do next?
Most common at onset of disease; if present, look for systemic disease.
Skin can be specific with granulomas on pathology, or nonspecific.
Usually cutaneous disease is asymptomaic and is of no prognostic significance.
What is the appearance of cutaneous sarcoidosis granulomas?
Brown-red papules and plaques are characteristic.
Lesions can be small papules or large plaques.
PEriorbital granulomatous papules
Red/brown color on diascopy.
What type of sarcoidosis can be diagnosed without a tissue sample?
Lofgren’s syndrome
- hilar adenopathy (big mediatinal lymph nodes)
- erythema nodosum
- Fever
- Iritis, arthritis
Better prognosis - will often resolve.
What are specific (characteristic) symptoms associated with inflammatory bowel disease (crohn’s disease and ulcerative colitis)?
Crohns disease with granulomas in the skin
Involvement of oral mucosa with granulomatous inflammation
Fistulae to skin
What are non-specific symptoms associated with inflammatory bowel disease (crohn’s disease and ulcerative colitis)?
Erythema nodosum
Pyoderma gangrenosum
Nutritional problems
These may make you think they have crohns but are not necessarily characteristic of the disease itself.
Describe the appearance of pyogerma gangrenosum seen with inflammatory bowel disease? What other diseases can it be associated with?
Steroil, rapid ulceration of the skin caused by neutrophilic infiltration.
Undermined, dusky borders.
Assocaited with IBD but also other conditions such as inflammatory arthritis, hematologic problems, malignancies, and idopathic.
What is dermatitis herpetiformis? How common is it?
Reaction to gluten in grains (gliabin proteins) causing an immune reaction in the small bowel that alters the mucosa there.
Leads to Ab formation that enter the blood and attach to skin (anti-transglutaminase Abs) causing the rash.
Much less common than celiacs.
Dermatitis herpetiformis is ricidulously _____ and the bowel disease may be ______.
Ridiculously pruritic
Bowel disease may be asymptomatic.
What is the best treatment of dermatitis herpetiformis?
Complete avoidance of gluten; after 3mo the TTG antibody will decrese, the mucosa of the gut will normalize, and symptoms of DH leave.
Dapson also effective, but does not effect gut or production of autoantibodies.
What is porphyria cutanea tarda (PCT)? What is it caused by?
Metabolic disease caused by a defect in uroporphyrin decarboxylase which breaks down gene proteins.
Other causes: HepC, alcoholism, gemachromatosis (iron overload), and some drugs. All of these are due to enzyme stress.
What cutaneous lesions are seen with porphyria cutanea tarda?
Fragile blisters from trauma and sun on hands.
Hypertrichosis of the face.
