CUTANEOUS MANIFESTATIONS OF SYSTEMIC DISEASE
Kathleen Haycraft, DNP, FNP/PNP-BC, DCNP, FAANP,
AANP BOD, REGION 7
• 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
Conflicts of Interest:
■ Celgene
■ Lilly
■ Pfizer
■ Abvie
■ Valeant
■ Novartis
■ Sanofi/Aventis/Regeneron
■ None will influence the discussion today.
Cutaneous Manifestations R/TEndocrine
ACAAcanthosis Nigricans
© kathleen haycraft
© kathleen Haycraft
Acanthosis Nigricans on hands
© kathleen haycraft
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
Granuloma Annulare
© kathleen haycraft
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.
Concerns
■ Associated with DM and thyroid autoimmune
disease
Diabetes Necrobiosis Lipoidica
Lichen Planus
© susan Voss
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
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
Concerns
■ Hepatitis C
■ Hepatitis B
■ LFTs
■ Low but existent risk for DM
Xanthelesma
© kathleen haycraft
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
Cutaneous ManifestationsRenal
Perforating Dermatosis
© Susan Voss
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.
Concerns
■ Renal Failure
■ Diabetes Mellitus
■ Refer to Dermatology
Calciphylaxis
© kathleen haycraft
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.
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
Cutaneous GI
Seborrheic Keratosis
© kathleen Haycraft
Sign of Leser Trelat
kathleen haycraft
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.
Concern:
■ Pigmented SK can mimic melanoma
■ If suddenly occur screen for genitourinary and
gastrointestinal malignancy
■ If concerned may refer to dermatology
Porphyria Cutanea Tarda
© Kathleen Haycraft
PCT
Woods light on PCT Urine
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
Concerns
■ Strong link to hepatitis C
■ May develop hepatocellular carcinoma
■ Modest risk HIV
■ Disease treatment will eliminate PCT
Palmar Erythema
© Kathleen Haycraft
Concerns
■ May be a normal variant
■ Look for alcohol abuse
■ Look for underlying liver disease
Dermatitis Herpetiformis
© Susan Voss
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
Concerns
■ Related to Celiac Disease
Cutaneous manifestations related to internal malignancies
Muir Torre Syndrome
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
Concern
■ REFER TO DERMATOLOGY AND INTERNIST FAMILIAR
WITH DISEASE for appropriate screening
Puetz-Jehgers Syndrome
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
Concerns
■ Small intestine malignancy
■ Refer to Dermatology GI and Oncology
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
Dermatomyositis
© kathleen haycraft
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
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
Bullous Pemphigoid
© kathleen haycraft
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
Pemphigus Vulgaris
© kathleen haycraft
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.
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
Pruritis/Chronic Lichen Simplex
© kathleen haycraft
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
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
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
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
Systemic Disease with common cutaneous disorders
Acne
kathleen haycraft
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
Rosacea
© kathleen haycraft
Rosacea
■ Genetic link to increase central vasculature of face
■ Initial face is erythrotelengectasia, papular-pustular,
granulomatous, ocular rosacea can occur at any
point
Concern
■ More than double risk of Parkinsons, DM, Celiac,
MS, Rheumatoid Arthritis and atherosclerosis
■ Screen for both and refer if concern
Photosensitivity
© kathleen haycraft
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
Dermatofibroma
© Kathleen Haycraft
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.
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
Psoriasis
© kathleen haycraft
Psoriasis
■ Autoimmune disease that affects many cytokinines
that cause hyperkeratinization and angiogenesis
■ Pathways include IL 12, 17, 23, TNF alpha
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
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
Sebaceous Cyst with SCC
Rapid growth of cyst, painful, expresses white material
© Kathleen Haycraft
Concerns
■ Look for cancer in rapidly expanding lesion
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
Good evening
And Good Night
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
Thank you.
■ Kathleen Haycraft, DNP, FNP/PNP-BC, DCNP, FAANP
■ 300 Lovers Leap Dr
Hannibal, MO 63401
5737952808