cutaneous manifestations of systemic disease · disorder of the heme pathway which causes buildup...

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

kathleenhaycraft@yahoo.com

5737952808