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Home , Granuloma, Granuloma annulare, Keratosis, Necrobiosis, Necrobiosis lipoidica, Seborrheic keratosis

from the Inside Out Dyanne P. Westerberg, DO

8/6/2014

Dermatological Manifestations of Systemic

Dyanne P. Westerberg, DO. FAAFP Associate Professor and Chair , Department of Family and Community Medicine Cooper Medical School Rowan University Camden, New Jersey

Goals

Certain skin disorders are frequently associated with internal disease. The skin itself may be insignificant but should prompt the clinician to search for possible internal illness. The goal of this lecture is to review several common skin conditions and their possible associated internal disorders. There are many such cutaneous problems. The purpose of this talk is to review more common pathological problems.

Paraneoplastic Syndromes

• Cutaneous symptom which is a consequence of an internal disease i.e • Broad range of • Believed to be due to result of biological active hormones, growth factors immunologic complexes induced by or produced by the tumor

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A 42 yo female patient presents with dry, thickened, scaly or flaky skin and she feels it resembles the scales on a fish;

http://dermnetnz.org/dermatitis/img/ichthyosis-s.jpg

Cutaneous and Internal Malignancy • : It has been described in association with malignancies, drugs, endocrine and metabolic disease, HIV, infection, and autoimmune conditions. – Hodgkins – Lymphoproliferative disorders – of the lung, breast and cervix

A 46 yo male presents to the office with a complaint of abrupt appearance of black ovals on this back. They started to appear about 3 months ago and OTC hydrocortisone cream did not help. On exam you see numerous seborrheic lesions http://www.51qe.cn/pic/30/12/17/41/b/00701.jpg

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Leser-Trelat

• Abrupt appearance of numerous • 3 to 6 months • Types • Most are of the GI tract • Others breast, lung, urinary tract, lymph

A 51 yo female presents to the office with a complaint of itchy skin on the nipple of the left breast. It has been present for 3 months and it has not resolved despite a change in soap and use of OTC hydrocortisone cream. Yesterday a bloody discharge started and she believes this is due to the increased scratching. She has not felt a lump.

http://www.oncoprof.net/Generale2000/g01_HistoireGenerale/Images/PagetSein.jpg

Paget’s Disease of the breast

• Breast cancer: Most women have underlying ductal breast cancer • Appears to be eczema - may be associated with discharge • It is common for the symptoms to disappear for a while, which may make the patient think incorrectly that the condition has cleared up spontaneously. • Most women do not visit the doctor because they take Paget's disease to be minor or eczema • Should encourage mammogram and biopsy • Most patients diagnosed with Paget's disease of the nipple are over age 50

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A 10 yo male presents to the office for evaluation of stomach upset. This has been going on for a few days. He is brought in by his mother who states that the father has some type of stomach problem but she has not seen him in years and doesn’t know what it is. Before you start the exam you notice that the child has darkly pigmented spots on the lips and buccal mucosa.

http://drugster.info/img/ail/3127_3150_3.jpg

Peutz- Jegher Syndrome aka Hereditary Intestional Polyposis Syndrome

• Autosomal dominant • Patches of in the mouth and on the hands and feet and may fade by adulthood • characterized by the development of noncancerous growths called hamartomatous polyps in the gastrointestinal tract (particularly the stomach and intestines) causing abdominal and GI bleeding. • of the stomach, duodenum, pancreas and colon. Also esophagus, ovary, lung, uterus and breast. • Intussusception in 47% of 222 patients with Peutz-Jeghers syndrome in Japan between 1961-1974

A 16 yo female present to the office as a new patient for a physical exam. During the course of the exam you note an overweight female patient. On her neck you find symmetric, hyperpigmented, hyperkeratotic and verrucous plaques. She mother reported that these lesions seem to have gradually erupted over the past few years. Various creams and soaps did not get rid of these lesions.

http://imaging.cmpmedica.com/shared/zone5/0812CFPILEF1.jpg

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Acanthosis nigricans Causes: •Obesity ( majority) •Insulin resistance •Excessive •Drugs i.e Oral contraceptives •Adenocarcinoma •In thin individuals: malignancy

Location: axilla most common , also nape of neck, groin, belt line , aerola, dorsum of fingers

Pathogenesis: •caused by factors that stimulate epidermal and dermal proliferation.

1 2

Image 1:http://www.skinsight.com/images/dx/webChild/acanthosisNigricans_22933_lg.jpg Image 2: http://www.cssd.us/images/diagnoses/endo7.jpg

• In 2000, the American Association established as a formal risk factor for the development of diabetes in children. • Sinha S, Schwartz RA. Juvenile acanthosis nigricans. J Am Acad Dermatol. Sep 2007;57(3):502-8

Acanthosis nigricans

• I hereditary – benign • II benign – – associated with endocrine disease usually insulin-resistance state such as polycystic ovarian disease, lipodystrophies, type 2 diabetes mellitus, and several genetic disorders – about 15% of adults with obesity and Acanthosis Nigricans have an endocrine abnormality e.g Cushing’s Disease • III pseudo - complication of obesity (rapid weight gain) • IV drug-induced - oral contraceptives, nicotinic acid, corticosteroids, subcutaneous insulin, testosterone, diethylstilbestrol, triazinate (a folate antagonist with antitumor activity) and topical fusidic acid (Fucidin, used for gram-positive bacterial skin infections) • V malignant - usually gastric adenocarcinoma, also seen with endocrinologic and lung malignancies, lymphoma, , sarcomas, and genitourinary tract – Type V seen most often in • non-obese patients with sudden onset, • severe or rapidly progressive involvement • mucous membrane or prominent palm and sole involvement • no easily discernible cause

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Acanthosis nigricans

• I hereditary – benign • II benign – – associated with endocrine disease usually insulin-resistance state such as polycystic ovarian disease, lipodystrophies, type 2 diabetes mellitus, and several genetic disorders – about 15% of adults with obesity and Acanthosis Nigricans have an endocrine abnormality e.g Cushing’s Disease • III pseudo - complication of obesity (rapid***** weight gain) • IV drug-induced - oral contraceptives, nicotinic acid, corticosteroids, subcutaneous insulin, testosterone, diethylstilbestrol, triazinate (a folate antagonist with antitumor activity) and topical fusidic acid (Fucidin, used for gram-positive bacterial skin infections) • V malignant - usually gastric adenocarcinoma, also seen with endocrinologic and lung malignancies, lymphoma, melanoma, sarcomas, and genitourinary tract cancers*****

– Type V seen most often in • non-obese patients with sudden onset, • severe or rapidly progressive involvement • mucous membrane or prominent palm and sole involvement • no easily discernible cause

A 32 yo female presents to the office with a lesion on the left anterior fibula. It has gradually gotten worse over the years. On exam you notice slightly raised shiny red-brown patches. The centers are yellowish.

http://t3.gstatic.com/images?q=tbn:ANd9GcSaXLUmj5P2q_rRFsBT86X02mx3MMLfXfv2W620YrITfox16ZoV5Q

Necrobiosis lipoidica

•Unknown origin •>50% DM •May appear years prior to the onset of DM •Commonly in 3rd and 4th decade •Most commonly females •Most anterior surfaces of the legs •Starts as small ovals •Waxy yellow skin with telangiectasia •eventually the skin •ulcers form •Treatment •Steroids •Pentoxifylline •Aspirin and dipyridamole •Skin grafting

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Bowen Disease Bowen’s disease is a very early form of that appears as a slow-growing, red and scaly skin patch. In Bowen’s disease, the skin cancer is located only in the , the uppermost layer of the skin. Rarely, the skin cancer can invade into the and then it is called an invasive squamous cell .

Differential

Stasis Dermatitis • insufficient venous return • can lead to increased pressure on capillaries in the extremities • blood collects in the intracellular spaces rather than being drawn back into the circulatory system

: http://t2.gstatic.com/images?q=tbn:ANd9GcReB7FykYvQzkqR7jtssOLLLPMaZyGPY96oRurIxrwPmqJRdB7cSg

Cellulitis

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Granuloma Annulare

•Appearance: •reddish bumps arranged in a circle or ring. • Types: • localized, disseminated, subcutaneous, and perforating. •Unknown etiology : •shown to follow trauma, malignancy, viral infections (including human immunodeficiency virus [HIV], Epstein-Barr virus, and herpes zoster), insect bites, and skin tests 12% of patient have DM

•Differential: •Treatment •None unless bothered by appearance •Triamcinolone injection, topical steroids etc

http://images.medicinenet.com/images/image_collection/skin/granuloma-annulare.jpg

Diabetes Mellitus

• Candida • Foot • Carotenodermia • Acanthosis Nigricans • Diabetic Bullae • Gas Gangrene • Diabetic Dermopathy • Granuloma Annulaire • Diabetic Thick Skin • Insulin Lipodystrophy • • Necrobiiosis Lipoidica • External • Yellow Nails • Finger Pebbles • Perforating disorders • Eruptive

•syndrome of painless nodules that occur over the pretibial areas Myxedema •subcutaneous accumulation of mucopolysaccharide-rich material •Stimulation of • A complication of Graves Disease: 1-4% but also seen in primary hypothyroidism or Hashimoto's thyroiditis •Associated with Graves’ophthalmopathy,

http://2.bp.blogspot.com/-WRLc3o-VTfs/TbvtZ-ezbSI/AAAAAAAAABE/sGENUs5uppo/s1600/myxedema.jpg

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A 54 yo female present s to the office with a complaint of flat yellow lesions around her eyes. You note that they are soft to the touch and have sharp edges.

Xanthomas •Lipid deposits in the skins and tendons due to elevated serum lipids

http://dermatology.cdlib.org/123/case_presentations/xanthoma/1.jpg

Types

Type clinical abnormality Xanthelasma Inner and outer canthus, May not have lipid problem plane or papular Eruptive Yellow on red base High triglycerides, DM Butt, elbows, knees Type I,II, IV hyperlipidemia Plane Palms and face, neck, chest Biliary , Type III Tuberous Nodular on elbows and High triglycerides, Type II knees and III biliary cirrhosis Tendinous Nodules on elbow and Type II knees, Achilles tendon , hands and feet

Types of Xanthomas

Eruptive Xanthomas Tuberous Xanthomas Image 1 Image 2

Image 1: http://www.healthcare.uiowa.edu/dermatology/Images%5CClin%5CErupXan-01-low.jpg Image 2: http://2.bp.blogspot.com/_v2ER6lx2y4w/TRwxzxSOsPI/AAAAAAAAACU/g__C8DdET28/s1600/xanthoma_tuberous.JPG

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Types of

Plane Xanthosis Tendinous Xanthomas Image 1 Image 2

Image 1:http://www.dermnet.org.nz/systemic/img/xanth-palm-s.jpg Image 2 http://3.bp.blogspot.com/_xzqs4DHM8as/TLSpR90RV-I/AAAAAAAAAGo/2KiF6LfV_ng/s320/xanthoma+tendon.jpg

Café- au - lait macules Image 1 Image 2

Malignant degeneration to neurofibromasarcoma or malignant schwannoma can occur

Image 1 http://www.hardsweat.com/wp-content/plugins/wp-o-matic/cache/60d73_cafe-au-lait-spot_1.jpg

Image 2 http://meded.ucsd.edu/clinicalimg/skin_neurofibroma1.jpg

Neurofibromatosis

• Autosomal Dominant Disorders but commonly due to mutation • Affects all neural crest cells • Presumptive Diagnosis – Prepubertal: 6 or more café au lait spots > 5 mm diameter – Post puberty: 6 or more café au lait spots > 15 mm diameter • Types: – neurofibromatosis type 1 (NF1, also called von Recklinghaus disease), – neurofibromatosis type 2 (NF2), associated with hearing loss – schwannomatosis. Associated with intense pain

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Tuberous 1 Sclerosis 2 Skin: Areas of the skin that are white (due to decreased pigment) and have either an ash leaf or confetti appearance Red patches on the face containing many blood vessels (adenoma sebaceum) Raised patches of skin with an orange- 3 peel texture (shagreen spots), often on the back

Image 1 http://t3.gstatic.com/images?q=tbn:ANd9GcS22v7Y-BxpcxJH6Jmygnpi-CKf3y60vYAsJiTJOz6oAmTIzn8t Image 2 :http://dermis.net/bilder/CD014/550px/img0075.jpg Image 3: http://www.uth.tmc.edu/GeneWise/LumpsBumpsSpots/images/slide31.png

Tuberous sclerosis

• Inherited Disorder - Dominant • Mutations in two genes, TSC1 and TSC2 • Affects skin, nervous tissue, kidneys and heart • With proper care, patients do well • Symptoms: – Developmental delays – Mental retardation – Seizures

Urticaria •polymorphic, round or irregularly shaped pruritic wheals •lesions can appear hyperemic in the center with a white halo along the circumference •Size: varies • released from cutaneous mast cells and basophils in response to inciting stimuli •Lab evaluation of little value

http://www.webmd.com/skin-problems-and-treatments/picture-of--urticaria

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Angioedema

•can occur alone or with urticaria •nonpitting, non-pruritic, well-defined, edematous swelling • involves subcutaneous tissues (e.g., face, hands, buttocks, genitals), abdominal organs, or the upper airway (i.e., larynx)

Common causes of angioedema and urticaria • Nonimmunologic causes – Physical stimuli: exposure to sun, water, or temperature extremes; delayed pressure (e.g., wearing a heavy backpack); vibration – Direct mast cell degranulation: opiates, vancomycin , aspirin, radiocontrast media, dextran, muscle relaxants, bile salts, NSAIDs, ACE inhibitors – Foods containing high levels of : strawberries, tomatoes, shrimp, lobster, cheese, spinach, eggplant

Common causes of angioedema and urticaria

What does this have to do with systemic • Immunological Causes – Type I IgE-mediated Disease?? • Foods: tree nuts, legumes, crustacea, mollusks, fish, eggs, milk, soy, wheat • Organic substances: preservatives, latex, hymenoptera venom • Medications: penicillin, cephalosporin, aspirin, NSAIDs • Aeroallergens: dust mites, pollens, molds, animal dander – Type II cytoxic antibody-mediated: transfusion reaction – Type III antigen-antibody mediated: serum sickness reaction – Type IV delayed : medication, food handling, or exposure to animals • Autoimmune disease: Hashimoto's disease, systemic erythematosus, , • Infection: viral (e.g., cytomegalovirus, Epstein-Barr, hepatitis), parasitic, fungal, or bacterial

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Treatment • long-acting, nonsedating histamine H1-receptor antagonist • first-generation H1-receptor antagonist, such as hydroxyzine • H2-receptor antagonist has shown benefit in the management of urticaria in controlled clinical studies • Cyproheptadine (periactin) • Doxepin (adapin) • Leukotriene modifiers such as montelukast or • short-term course of oral glucocorticoids

Strep Group A beta hemolytic

http://www.webmd.com/cold-and-flu/slideshow-anatomy-of-a-sore-throat

– bright red, scarlet on their chest and neck, which then spreads to the rest of their body – rash blanches Scarlet – flushed face – strawberry tongue – fever and general malaise

http://www.goodtoknow.co.uk/health/133316/Scarlet-fever-in-children

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Rheumatic Fever – Arthritis – Carditis – Subcutaneous nodules – Sydenham chorea – Skin rash (erythema marginatum) -- 5% of patients • Erythematous rings • Non pruritic

http://images.rheumatology.org/viewphoto.php?albumId=75683&imageId=2862052

– tender red lumps from 1 to 5 centimeters – most commonly located over the shins – most common form of panniculitis ( of the subcutaneous fat) – The peak incidence of EN occurs between 18– 36 years of age. – Resolves 3 to 6 weeks http://www.beltina.org/health-dictionary/erythema-nodosum-symptoms-treatment.html

• Infections. Streptococcus (most common) • Pregnancy – Cat scratch disease • Sensitivity to certain – Chlamydia medications, including: – – Antibiotics including – Hepatitis B amoxicillin and other penicillins – – Sulfonamides – Leptospirosis – Sulfones – Mononucleosis (EBV) – Birth control pills – Mycobacteria – Progestin – Mycoplasma – Psittacosis – – Tuberculosis – Tularemia – Yersinia

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– salmon-pink drops on the skin – 80% occur after strep infection – about 2-3 weeks after the infection – more common in children and adults Guttate younger than 30 years

Image 2 http://www.webmd.com/skin-problems-and-treatments/psoriasis/slideshow-severe-psoriasis-9

Lyme Disease •a systemic illness resulting from infection with the spirochete Borrelia burgdorferi •most prevalent in children two to 15 years of age and in adults 30 to 59 years of age •The onset of clinical manifestations - within 7 to 10 days after a tick bite, with a reported range of one to 36 days. •Most patients (60 to 80 percent) develop the early, localized form of •May have associated influenza-like symptoms

http://geology.com/articles/ticks-lyme-disease

Dermatitis Herpetiformis

•Duhring's Disease •Chronic Blistering •Extremely itchy •Itch and burning may appear prior to the onset of the rash •Associated with Celiac disease •Will improve with gluten free diet

http://www.primehealthchannel.com/dermatitis-herpetiformis-pictures-symptoms-causes-and-treatment.html

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A patient of any age presents to the office with red or purple macules on the skin that do not blanch on applying pressure.

HENOCK- SCHÖNLEIN PURPURA

Kawasacki Disease Awareness: http://www.facebook.com/note.php?note_id=85263843435

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Petechiae or Purpura Purpura measure 0.3–1 cm (3–10 mm), whereas petechiae measure less than 3 mm, and ecchymoses greater than 1 cm. They can be seen with clotting problems, associated with infections i.e Meningitis, rocky mountain spotted fever and vasculitis i.e Henoch- Scholein Purpura

http://www.noblis.org/MissionAreas/nsi/BackgroundonBiologicalWarfare/BiologicalWarfareOrganisms/Pages/Rickettsiae.aspx

•systemic disease of unknown cause with noncaseating •skin involvement occurs in about 20-35% and may be first presentation •2 forms of cutaneous Sarcoidosis •specific skin lesions contain noncaseating granulomas and may include maculopapules •Plaques •nodules •Lupus perno: indurated, lumpy, lesions on nose, cheeks, lips, and ears • infiltration •alopecia •ulcerative lesions •hypopigmentation •nonspecific skin lesions (without noncaseating granulomas) •calcifications •Prurigo – itchy eruptions of the skin • Most characteristic • clubbing •Sweet syndrome – fever and red bumps on the arm

http://dermatology.cdlib.org/133/case_reports/pernio/3.jpg

Systemic Lupus Erythematosis

•multisystem, autoimmune disorder of connective tissue •Butterfly rash •Erythema Multiforme type rash •Skin lesions may worsen with sun exposure •Alopecia is also common •Discoid Lupus is associated only with skin manifestations

http://www.cedars-sinai.edu/Patients/Health-Conditions/Images/351457_Lupus-1.jpg http://dermatology.cdlib.org/1508/articles/2008102101/1.jpg

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