cutaneous manifestations of systemic disease · disorder of the heme pathway which causes buildup...
TRANSCRIPT
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CUTANEOUS MANIFESTATIONS OF SYSTEMIC DISEASE
Kathleen Haycraft, DNP, FNP/PNP-BC, DCNP, FAANP,
AANP BOD, REGION 7
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• Identify cutaneous manifestations related to /endocrine
dysfunction.
• Identify cutaneous manifestations related to renal failure.
• Identify cutaneous manifestations related to
gastrointestinal..
• Identify cutaneous manifestations related to internal
malignancies.
Objectives
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Conflicts of Interest:
■ Celgene
■ Lilly
■ Pfizer
■ Abvie
■ Valeant
■ Novartis
■ Sanofi/Aventis/Regeneron
■ None will influence the discussion today.
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Cutaneous Manifestations R/TEndocrine
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ACAAcanthosis Nigricans
© kathleen haycraft
© kathleen Haycraft
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Acanthosis Nigricans on hands
© kathleen haycraft
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Acanthosis Nigricans
■ Concern if sudden:
– Growth Hormone
– Contraception
– Malignancy
■ Pearl: Signs of malignancy include:
Rapid, pruritic, and extensive acanthosis nigricans in a patient of normal or low BMI
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Granuloma Annulare
© kathleen haycraft
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Granuloma Annulare
■ Granuloma annulare (GA) is common, self limited, dermatosis of the dermis and subcutaneous tissue.
■ May be local lesions or disseminated
■ May last years or decade
■ Interesting new treatment Augmentin 875 bid two weeks.
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Concerns
■ Associated with DM and thyroid autoimmune
disease
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Diabetes Necrobiosis Lipoidica
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Lichen Planus
© susan Voss
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Lichen Planus■ Self-limiting pruritic inflammatory condition
■ Abnormal immune reaction provoked by a viral infection.
■ Strong association with Hepatitis C virus
■ Prognosis is good with the majority of cases resolving within 18 months.
■ The 5 P’s Pruritic, Planar, Polyangular, Purple Papules
■ Wickham’s Striae
■ 2mm-1cm
■ Triggered by Hep B, C, Flu vaccine, Nsaids
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Lichen Planus
■ Aspartame
■ Graft Vs Host
■ Primary Biliary Cirrhosis
■ May be beta blockers, plaquenyl, thiazide diuretics,
furosemide, spironolactone, metformin penicilin,
NSAIDs, Hep B, C disease, Flu vaccine
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Concerns
■ Hepatitis C
■ Hepatitis B
■ LFTs
■ Low but existent risk for DM
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Xanthelesma
© kathleen haycraft
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Xanthelesma/Xanthoma
Xanthomas develop from a deposition of cholesterol rich materials.
Yellowish in color due to the yellow color of cholesterol. They are common
Usually associated with hyperlipidemia. They can occur anywhere on the body and when
a xanthoma occurs near the eyelids it is referred to as a xanthelesma. The lesion is soft
and usually has sharp demarcations.
Concern: Monitor lipids…less necessary if Xanthelsma. Screen for family history of
cardiovascular disease
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Cutaneous ManifestationsRenal
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Perforating Dermatosis
© Susan Voss
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Perforating Dermatoses
• Umbilicated dome-shape papules on the arms and legs
• Papules hyperkeratotic, ranging in size from 2 to 10mm
• Varying degrees of pruritus
• Tends to be distributed on trauma-prone areas and often can be reproduced by scratching.
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Concerns
■ Renal Failure
■ Diabetes Mellitus
■ Refer to Dermatology
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Calciphylaxis
© kathleen haycraft
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Calciphylaxis
• Rare systemic Rare syndrome involving vascular calcification and skin necrosis.
• Seen with chronic renal failure with dialysis.
• Accumulation of calcium deposits in the tunica media of the walls of small arterial vessels is responsible for the presentation of calciphylaxis.
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Concerns
• High mortality rate of 60-80%. The one year and
five year survival rates have been reported at 45%
and 35% ,respectively.
• The most common cause of death is usually
secondary sepsis
• REFER TO DERM ASAP
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Cutaneous GI
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Seborrheic Keratosis
© kathleen Haycraft
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Sign of Leser Trelat
kathleen haycraft
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Seborrheic Keratosis
■ The most common cutaneous neoplasm.
■ Correlated with senescence and genetics.
■ Increase after age 40.
■ Associated with failure of keratinocyte repressor
gene (FGFR3 and P13K genes).
■ Common sites face, chest, back, and friction sites.
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Concern:
■ Pigmented SK can mimic melanoma
■ If suddenly occur screen for genitourinary and
gastrointestinal malignancy
■ If concerned may refer to dermatology
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Porphyria Cutanea Tarda
© Kathleen Haycraft
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PCT
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Woods light on PCT Urine
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Porphyria Cutanea Tarda
■ Disorder of the heme pathway which causes buildup
of porphyrins which are activated by UV light
■ Genetic predisposition that has trigger of alcohol
abuse, oral contraceptives, viral illnesses eg HIV and
Hep C
■ Fragile skin with vesicles and bullae of sun exposed
areas, classically pull hands out of pants and
blisters open.
■ Facial hypertrichosis
■ REFER TO DERMATOLOGY
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Concerns
■ Strong link to hepatitis C
■ May develop hepatocellular carcinoma
■ Modest risk HIV
■ Disease treatment will eliminate PCT
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Palmar Erythema
© Kathleen Haycraft
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Concerns
■ May be a normal variant
■ Look for alcohol abuse
■ Look for underlying liver disease
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Dermatitis Herpetiformis
© Susan Voss
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Dermatitis Herpetiformis
■ Chronic, itchy, burning, blistering rash.
■ Lesions are found symmetrically on the extensor surfaces of the knees, elbows, back, and buttocks.
■ Seen with Celiac Disease
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Concerns
■ Related to Celiac Disease
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Cutaneous manifestations related to internal malignancies
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Muir Torre Syndrome
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Muir Torre Syndrome
■ Cutaneous sebaceous adenoma or carcinoma and
Keratocanthoma with visceral malignancy GU, GI,
small intestinal, some thyroid
■ Lynch syndrome
■ Mismatch repair gene
■ Isot and interferon have been used to reduce risk
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Concern
■ REFER TO DERMATOLOGY AND INTERNIST FAMILIAR
WITH DISEASE for appropriate screening
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Puetz-Jehgers Syndrome
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Puetz-Jeghers Syndrome
■ Autosomal dominant inherited with
hyperpigmentation and polyps
■ Early marked freckling in unusual places eg. Lips,
soles of feet and palms. They are blue gray
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Concerns
■ Small intestine malignancy
■ Refer to Dermatology GI and Oncology
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Concerns
■ Nanoparticles may be carcinogenic black and red
ink are worse
■ Tattoo removal may increase spread
■ Infections esp Hep C as well as unusual bacteria
■ Allergic Reactions
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Dermatomyositis
© kathleen haycraft
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Dermatomyositis■ Rare autoimmune disease that can affect skin,
joints, muscles and many organs
■ Muscle weakness
■ Scaly or psoriaform plaques on forehead and scalp
■ Gottron papules, violaceous hues like violet
eyeshadow
■ Pigmentary changes and telangectasias follows
shawl sign on sun exposed areas
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Concerns■ Myopathies
■ Esophageal involvement
■ Cardiac arrhythmias including conduction abnormalities
■ 10% have interstitial lung disease
■ Esophageal involvement
■ Dependent upon genetic type determines risk for cancer
types, always screen for ovarian
■ Cancer risk in first five years with highest in first year.
■ Refer to dermatology also consider rheumatology
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Bullous Pemphigoid
© kathleen haycraft
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Bullous Pemphigoid■ Disease is a tense blistering disease that is bellow
the epidermis and is a chronic inflammatory illness
that persists for months or years It can have
remissions and exacerbations. It can be fatal.
■ Blisters are firm and antibodies attack the area
below the epidermis
■ BP 230 and 180 are circulating antibodies that
target the hemidesmosome and can be measured
for disease severity
■ May be triggered by medications eg diuretics,
captopril, antibiotics and neuroleptic agents
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Pemphigus Vulgaris
© kathleen haycraft
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Pemphigus Vulgaris
■ Autoimmune blistering disease where the antibodies
target the epithelial cell and mucous membrane
■ Blisters are soft
■ No known cause is linked to some medications and
may be linked to cancers.
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Concerns:■ Treatments side effects of immune suppression and ulcers.
■ Dermatologic emergency as affects many mucous membranes in body Sepsis, Treatments put patients at risk for ulcers, immune suppression
■ High risk of sepsis
■ Involves the mucousa in 25%
■ HPN, MI, DM. thromboembolism, neurologic disorders and cancer risks are elevated
■ Optical lesions can result in blindness
■ Cancer development secondary to immune suppression
■ Refer to dermatology
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Pruritis/Chronic Lichen Simplex
© kathleen haycraft
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Concerns:
■ Drugs associated with pruritis:
■ Nsaids, steroids, testosterone, opiates, allopurinol,
antidepressants, ED drugs, Statins, tamoxifen,
antibiotics, ACE, ARB, Beta blocker, Calcium channel
blocker, neuroleptics, heparin, amiodarone,
biguanides, sulfonylurea derivative diabetic agents
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Concerns
■ If pruritis has been treated for a reasonable time
with cool creams, low potency steroid creams, four
time dose antihistamines, negative immuno-cap
testing, negative patch testing, gabapentin DO A
WORK UP
■ Work up;
■ CMP, TSH, HIV, CBC, Sed, SPEP, CXR,
■ Consider psych referral and further malignancy work
up
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Sweet’s Syndrome
■ Painful violaceous juicy plaque often on back of
hand
■ May have a nipple like look
■ May have dusky papules and plaques there may be
vesicles and bulla
■ Believed to be a hypersensitivity reaction to drugs or
disease
■ Frequently have had a preceding infectious disease
that is respiratory
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Concerns■ Previous trigger eg. Infection or meds eg.,
isotretinoin, oral contraceptives, sulfa, furosemide,
cyclins, quinolones and cyclins
■ Risks include:
– Pyoderma granulosum
– Bullous disease
– Genitourinary cancer
– Hematologic malignancy
– REFER TO DERMATOLOGY
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Systemic Disease with common cutaneous disorders
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Acne
kathleen haycraft
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Concerns:
■ The issue of depression, inflammatory bowel
disease is likely a comorbid of nodular cystic acne
and not due to isotretinoin or doxycycline
■ Screen for GI and depression/suicide
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Rosacea
© kathleen haycraft
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Rosacea
■ Genetic link to increase central vasculature of face
■ Initial face is erythrotelengectasia, papular-pustular,
granulomatous, ocular rosacea can occur at any
point
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Concern
■ More than double risk of Parkinsons, DM, Celiac,
MS, Rheumatoid Arthritis and atherosclerosis
■ Screen for both and refer if concern
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Photosensitivity
© kathleen haycraft
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Photosensitivity/polymorphous light eruption
■ Exposure to sun results in sunburn like reaction, more edematous and itchy to painful
■ MED relationship: quinolones, sulfa, furosemide, HCTZ, isotretinoin, antifungals, NSAIDS, phenothiazines, cyclins
■ Polymorphous light eruption usually idiopathic exposure occurs on extensor surfaces and face. A significant proportion of individuals with photosensitivity develop Lupus
■ Screen for arthralgias, ANA with titer annually. Refer if either is significant
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Dermatofibroma
© Kathleen Haycraft
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Appearance■ Asymptomatic but occasionally pruritic or tender.
■ Small, firm, exophytic papule on the lower
extremities of adults.
■ The color may be flesh or have tan or brown
pigmentation.
■ Hypertrophy of the overlying epidermis may exist.
■ Dermatofibromas characteristically have a dimple
sign (Fitzpatrick sign) that occurs when placing
lateral pressure with the thumb and forefinger.
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Dermatofibrosis
■ Concerns:
■ Multiple dermatofibromas (greater than 6) are associated with an altered immune state. The most common is systemic lupus but other disorders include: myasthenia, AIDS, and malignancies.
– Even though benign, overlying epidermis has increase risk for BCC..rapid growth is a clue
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Psoriasis
© kathleen haycraft
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Psoriasis
■ Autoimmune disease that affects many cytokinines
that cause hyperkeratinization and angiogenesis
■ Pathways include IL 12, 17, 23, TNF alpha
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ConcernsMedications can trigger: Beta blocker, Lithium, antimalarials
Severe stress, bacterial and viral infections
Reduces 5 years life span
Comorbidities:
■ Psoriatic arthritis
■ CVD,
■ HPN
■ Hyperlipidemia
■ Abdominal aortic aneurysm
■ Stroke
■ Fractures
■ Hypertension
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Concerns:■ Obesity
■ Diabetes
■ Depression
■ Malignancy
■ Insomnia
■ Anxiety
■ PEARL Nictotine stimulates the TNF alpha pathway
■ If psoa, severe disease, genitalia, scalp, palmar plantar REFER TO DERMATOLOGY
■ The new world of biosimilars
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Sebaceous Cyst with SCC
Rapid growth of cyst, painful, expresses white material
© Kathleen Haycraft
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Concerns
■ Look for cancer in rapidly expanding lesion
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Just a pearl for a pearl’s sake
■ Primary presentation of Zika is itchy papular or
macular rash…NO FEVER
■ Powasa virus in ticks is scary
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Good evening
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And Good Night
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References:
■ Bolognia, Jean L., Jorizzo, Josep L., & Shaffer, Julie V. (2012). Dermatology: 2-Volume Set: Expert Consult Premium Edition (3rd ed). Philadelphia, PA: Saunders.
■ DermNet NZ: the dermatology resource. (2016). Retrieved from http://www.dermnetnz.org/
■ Habif, Thomas B. (2015). Clinical Dermatology (5th ed.). Philadelphia, PA: Mosby.
■ Medscape Reference: Drugs, Diseases, and Procedures. (2016). Retrieved from http://reference.medscape.com/
■ James, William D., Berge, Timothy, & Elston, D. (2015). Andrews' Diseases of the Skin, 11th Edition (11th ed.). Philadelphia, PA: Saunders.
■ Cutis Journal Years 2015-2016
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Thank you.
■ Kathleen Haycraft, DNP, FNP/PNP-BC, DCNP, FAANP
■ 300 Lovers Leap Dr
Hannibal, MO 63401
5737952808