Dermatology from the Inside Out Dyanne P. Westerberg, DO 8/6/2014 Dermatological Manifestations of Systemic Disease 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 lesion 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 neoplasm • Broad range of diseases • Believed to be due to result of biological active hormones, growth factors immunologic complexes induced by or produced by the tumor 1 8/6/2014 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 lesions and Internal Malignancy • Ichthyosis: It has been described in association with malignancies, drugs, endocrine and metabolic disease, HIV, infection, and autoimmune conditions. – Hodgkins – Lymphoproliferative disorders – Cancer 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 keratosis lesions http://www.51qe.cn/pic/30/12/17/41/b/00701.jpg 2 8/6/2014 Leser-Trelat • Abrupt appearance of numerous seborrheic keratosis • 3 to 6 months • Types • Most are adenocarcinomas of the GI tract • Others breast, lung, urinary tract, lymph tissue 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 contact dermatitis or eczema • Should encourage mammogram and biopsy • Most patients diagnosed with Paget's disease of the nipple are over age 50 3 8/6/2014 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 hyperpigmentation 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 pain and GI bleeding. • Adenocarcinoma 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 4 8/6/2014 Acanthosis nigricans Causes: •Obesity ( majority) •Insulin resistance •Excessive corticosteroids •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 keratinocyte and dermal fibroblast 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 Diabetes Association established acanthosis nigricans 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, 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 5 8/6/2014 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 atrophies •ulcers form •Treatment •Steroids •Pentoxifylline •Aspirin and dipyridamole •Skin grafting 6 8/6/2014 Bowen Disease Bowen’s disease is a very early form of skin cancer that appears as a slow-growing, red and scaly skin patch. In Bowen’s disease, the skin cancer is located only in the epidermis, the uppermost layer of the skin. Rarely, the skin cancer can invade into the dermis and then it is called an invasive squamous cell carcinoma. 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 7 8/6/2014 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 tuberculosis skin tests 12% of patient have DM •Differential: Tinea Corporis •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 Ulcer • Carotenodermia • Acanthosis Nigricans • Diabetic Bullae • Gas Gangrene • Diabetic Dermopathy • Granuloma Annulaire • Diabetic Thick Skin • Insulin Lipodystrophy • Erythema • Necrobiiosis Lipoidica • External Otitis • Yellow Nails • Finger Pebbles • Perforating disorders • Eruptive Xanthomas •syndrome of painless nodules that occur over the pretibial areas Myxedema •subcutaneous accumulation