CUTANEOUS MANIFESTATIONS OF SYSTEMIC DISEASE. Kathleen Haycraft, DNP, FNP/PNP-BC, DCNP, FAANP, AANP BOD, REGION 7

Transcription

1 CUTANEOUS MANIFESTATIONS OF SYSTEMIC DISEASE Kathleen Haycraft, DNP, FNP/PNP-BC, DCNP, FAANP, AANP BOD, REGION 7

2 Objectives 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.

3 Conflicts of Interest: Celgene Lilly Pfizer Abvie Valeant Novartis Sanofi/Aventis/Regeneron None will influence the discussion today.

4 Cutaneous Manifestations R/T Endocrine

5 ACA kathleen Haycraft Acanthosis Nigricans kathleen haycraft

6 Acanthosis Nigricans on hands kathleen haycraft

7 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

8 Granuloma Annulare kathleen haycraft

9 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.

10 Concerns Associated with DM and thyroid autoimmune disease

11 Diabetes Necrobiosis Lipoidica

12 Lichen Planus susan Voss

13 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

14 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

15 Concerns Hepatitis C Hepatitis B LFTs Low but existent risk for DM

16 Xanthelesma kathleen haycraft

17 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

18 Cutaneous Manifestations Renal

19 Perforating Dermatosis Susan Voss

20 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.

21 Concerns Renal Failure Diabetes Mellitus Refer to Dermatology

22 Calciphylaxis kathleen haycraft

23 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.

24 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

25 Cutaneous GI

26 Seborrheic Keratosis kathleen Haycraft

27 Sign of Leser Trelat kathleen haycraft

28 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.

29 Concern: Pigmented SK can mimic melanoma If suddenly occur screen for genitourinary and gastrointestinal malignancy If concerned may refer to dermatology

30 Porphyria Cutanea Tarda Kathleen Haycraft

31 PCT

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34 Woods light on PCT Urine

35 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

36 Concerns Strong link to hepatitis C May develop hepatocellular carcinoma Modest risk HIV Disease treatment will eliminate PCT

37 Palmar Erythema Kathleen Haycraft

38 Concerns May be a normal variant Look for alcohol abuse Look for underlying liver disease

39 Dermatitis Herpetiformis Susan Voss

40 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

41 Concerns Related to Celiac Disease

42 Cutaneous manifestations related to internal malignancies

43 Muir Torre Syndrome

44 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

45 Concern REFER TO DERMATOLOGY AND INTERNIST FAMILIAR WITH DISEASE for appropriate screening

46 Puetz-Jehgers Syndrome

47 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

48 Concerns Small intestine malignancy Refer to Dermatology GI and Oncology

49 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

50 Dermatomyositis kathleen haycraft

51 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

52 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

53 Bullous Pemphigoid kathleen haycraft

54 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

55 Pemphigus Vulgaris kathleen haycraft

56 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.

57 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

58 Pruritis/Chronic Lichen Simplex kathleen haycraft

59 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

60 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

61 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

62 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

63 Systemic Disease with common cutaneous disorders

64 Acne kathleen haycraft

65 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

66 Rosacea kathleen haycraft

67 Rosacea Genetic link to increase central vasculature of face Initial face is erythrotelengectasia, papular-pustular, granulomatous, ocular rosacea can occur at any point

68 Concern More than double risk of Parkinsons, DM, Celiac, MS, Rheumatoid Arthritis and atherosclerosis Screen for both and refer if concern

69 Photosensitivity kathleen haycraft

70 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

71 Dermatofibroma Kathleen Haycraft

72 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.

73 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

74 Psoriasis kathleen haycraft

75 Psoriasis Autoimmune disease that affects many cytokinines that cause hyperkeratinization and angiogenesis Pathways include IL 12, 17, 23, TNF alpha

76 Concerns Medications 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

77 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

78 Sebaceous Cyst with SCC Rapid growth of cyst, painful, expresses white material Kathleen Haycraft

79 Concerns Look for cancer in rapidly expanding lesion

80 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

81 Good evening

82 And Good Night

83 References: Bolognia, Jean L., Jorizzo, Josep L., & Shaffer, Julie V. (2012). Dermatology: 2-Volume Set: Expert Consult Premium Edition (3 rd ed). Philadelphia, PA: Saunders. DermNet NZ: the dermatology resource. (2016). Retrieved from Habif, Thomas B. (2015). Clinical Dermatology (5th ed.). Philadelphia, PA: Mosby. Medscape Reference: Drugs, Diseases, and Procedures. (2016). Retrieved from James, William D., Berge, Timothy, & Elston, D. (2015). Andrews' Diseases of the Skin, 11th Edition (11th ed.). Philadelphia, PA: Saunders. Cutis Journal Years

84 Thank you. Kathleen Haycraft, DNP, FNP/PNP-BC, DCNP, FAANP 300 Lovers Leap Dr Hannibal, MO