Dermatology 5th year, 3rd lecture (Dr. Faraedon Kaftan)


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The lecture has been given on Apr. 10th, 2011 by Dr. Faraedon Kaftan.

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Dermatology 5th year, 3rd lecture (Dr. Faraedon Kaftan)

  1. 1. Dermatologic Signs of Systemic Diseases5th yearLecture By Dr. FaraedonKaftanConsultant DermatologistCollege of MedicineUniversity of Sulaimani April. 10th2011<br />
  2. 2. The skin is often a window to systemic disease.<br />By recognizing cutaneous manifestations of systemic diseases, the internist can often determine the appropriate diagnosis and therapy or the need for referral to a dermatologist.<br />1- Common cutaneous disorders (Seb D, Seb K, Urt,EM, V, EN)<br />2- Blistering diseases (P, Pd, EBA)<br />3- Internal malignancies (Cut. Metastases, Pag. Dis, AN, Amyloid)<br />4- Cardiovascular disease (PXE, EDS)<br />5- Pulmonary disease (Sarcoidosis)<br />6- Rheumatic disease (Ps. A, LE, Sclerod., DM )<br />7- Gastrointestinal disease (DH, ACE, LP …)<br />8- Renal disease (Nephrogenicfibrosingdermopathy) <br />9- Endocrine and metabolic disease (Porphyria and Diabetes Mellitus-Related Skin Conditions)<br />
  3. 3. 1- Common cutaneous disorders <br />Seborrheic dermatitis<br />may be associated with <br />systemic disease, such as <br />1-Parkinson's disease<br />2- (HIV: Human immunodeficiency virus) infection. <br />3- (CVA: cerebrovascular accident ) can develop unilateral seborrheic dermatitis on the scalp corresponding to the affected hemisphere, (The pathophysiology of this phenomenon is not completely understood.)<br />
  4. 4. SeborrheicKeratoses<br />the most common benign cutaneous neoplasms, <br />are warty, age-related hyperkeratotic papules and plaques that appear anywhere on the body, most commonly the trunk.<br />(Sign of Leser-Trélat):Rarely, if seborrheickeratoses appear suddenly in great numbers indicate an underlying adenocarcinoma of the GI tract<br />
  5. 5. Urticaria or Hives<br />are pruritic, edematous, <br /> evanescent wheals that <br /> resolve within 24 hours.<br />Acute urticaria typically lasts <br /> less than 6 weeks. <br />is most often caused by: <br />1- Medication (commonly penicillin or <br /> other antibiotics, sulfa drugs, aspirin) <br />2- Food (shellfish, nuts, chocolate), <br />3- infection: less often<br />
  6. 6. ErythemaMultiforme (EM)<br />Is a cutaneous hypersensitivity reaction characterized by Iris (Target) lesions on the face, hands and feet. <br />is usually caused by: <br />1- Infection (herpes simplex virus or Mycoplasmapneumoniae) <br />2- and less commonly by drug sensitivity (sulfonamides, barbiturates, antibiotics). <br />
  7. 7. Vitiligo<br />commonly occurs in periorificial areas (mouth, orbits, vagina, anus) or at sites of trauma (hands, elbows, knees). <br />Vitiligo is often associated with: <br />1- Autoimmune thyroid disease, <br />2- diabetes mellitus (Insulin-dependent) <br />3- Pernicious anemia, or <br />4- Addison's disease.<br />
  8. 8. ErythemaNodosum (EN)<br />Painful, erythematous nodules on the shins and occasionally elsewhere, in young women, between 20 and 40 years, lasting an average of 3 to 6 weeks.<br />Causes of EN: 3 S + Tb<br />1- Streptococcal pharyngitis is the most common . <br />2- Tuberculosis,<br />3- (GI) infections with Yersinia, Salmonella, or Shigella, and <br />4- Systemic fungal infections. <br />5- Less common causes: drug sensitivity (sulfonamides, salicylates, iodides, oral contraceptives or hormone replacement therapy), and a variety of systemic diseases, most often inflammatory bowel disease (Crohn's disease more than ulcerative colitis) and sarcoidosis.<br />
  9. 9. 2- Blistering diseasesPemphigusVulgaris<br />painful mucosal erosions and flaccid skin blisters (bullae) that become erosive. occurs in the 40th to 60th of life, <br />Mortality is significant, even with treatment.<br />
  10. 10. BullousPemphigoid<br />characterized by large, tense subepidermal blisters, which are often pruritic. Mucosal disease is rare.<br />in the elderly, 65 and 75 years of age. <br />
  11. 11. EpidermolysisBullosaAcquisita: EBA<br />uncommon bullous disease characterized by skin fragility, milia (small cysts), scarring alopecia, and nail dystrophy. primarily on the hands, feet, elbows, and knees.<br />EBA may be associated with other autoimmune diseases, most frequently inflammatory bowel disease. <br />
  12. 12. 3- Internal malignancies<br />Cutaneous Metastases<br />Any malignant neoplasm can metastasize to the skin. <br />in men: Cutaneous metastases are from cancers of the lung, large intestine, and kidney.<br />in women: Cutaneous metastases are from cancers of the breast and large intestine.<br />Flesh-colored to violaceous nodules in close proximity to the primary neoplasm; <br />Most common sites are the <br /> - Head (scalp), <br /> - Neck, and <br /> - Trunk.<br />
  13. 13. Paget's Disease<br />1- Mammary Paget's disease: Unilateral eczematous <br />plaque of the nipple and areola. <br />- is strongly associated with an underlying <br />invasive Ca. of the breast<br />2- Extramammary Paget's disease: <br />* persistent, eczematous plaque of the <br />anogenital or axillary regions: <br />* is often associated with an underlying <br />- adnexal (apocrine gland) carcinoma or <br />- cancer of the genitourinary tract or distal GIT.<br />
  14. 14. AcanthosisNigricans (ANs)<br />smooth, velvet-like, hyperkeratotic plaques in intertriginous areas (e.g., groin, axillae, neck).<br />Type I (Malignant AN): is associated with malignancy. May be adenocarcinoma of the GI tract (60% gastric) <br />has a sudden onset and more extensive distribution, including the face, palms, and trunk. <br />Type III AN is associated with obesity and insulin resistance and is the most commonly occurring type.<br />AN can develop following the use of drugs: <br /> systemic Cs, <br /> nicotinic acid, <br /> diethylstilbestrol, and <br />isoniazid (INH).<br />
  15. 15. Amyloidosis<br />Purpuric and Ecchymotic Papules on the eyelids and extremities due to amyloid infiltration in the vessels. <br />may be a sign of multiple myeloma. <br />
  16. 16. 4- Cardiovascular diseasePseudoxanthomaElasticum: PXE<br />Brittle (Broken)and calcified elastic fibers. <br />Yellow papules over redundant skin folds on the neck, abdomen, and groin, giving the skin the appearance of plucked chicken skin. <br />Angioid streaks in Bruch's membrane of the eye.<br />Associated signs of PXE : <br />1- Hypertension (HTN) <br />2- Peripheral vascular and coronary artery disease <br />3- Retinal and GI hemorrhage<br />4- Stroke.<br />
  17. 17. Ehlers-Danlos Syndrome: EDS<br /><ul><li>abnormalities in collagen biosynthesis, </li></ul> which can affect multiorgans<br />Joint hyperextensibility, <br />Hypermobility (Hyperelasticity) <br />Skin and Vessel fragility, and <br />fish-mouth scars. <br />* (7-11) types of EDS identified associated with:<br />mitral valve prolapse, blue sclerae, vascular aneurysm, aortic dissection, hernias, angina, GI bleeding (perforation), and peripheral vascular disease. <br />* Patients with vascular (type IV) EDS are prone to arterial rupture and have the highest mortality.<br />
  18. 18. EDS<br />
  19. 19. 5- Pulmonary disease<br />Sarcoidosis<br />is a multisystem, granulomatous disease of the <br /> lungs, bones, CNS, lymph nodes, eyes, and skin. <br />more in women and in African Americans. <br />Skin disease, affecting 25-35% of patients, includes <br />(Lupus pernio): Red to purple indurated plaques of the nose <br />midfacial papules <br />annular plaques or nodules on the trunk and extremities. <br />predilection for scars. <br />Erythemanodosumis the most common manifestation.<br />
  20. 20. 6- Rheumatic diseasePsoriatic Arthritis: (PsA)<br />Affects 5-10% of patients with psoriasis. <br />Asymmetric fusiform swelling of the distal interphalangeal joints (sausage digits) in 70%. <br />
  21. 21. Lupus Erythematosus: DLE, SLE & SCLE<br />(DLE) usually localized to the head or neck, is characterized by atrophic, scarring plaques on sun-exposed areas. <br />5% of patients develop SLE.<br />
  22. 22. SLE<br />cutaneous manifestations include <br />Malarerythema (The butterfly rash): is the most common expression of SLE.<br />Photosensitivity<br />Oral ulcers<br />Discoid plaques <br />Bullae<br />Purpura<br />Calcinosis cutis <br />alopecia.<br />
  23. 23. systemic or generalized Scleroderma<br />Are of 2 forms:<br />1- CREST syndrome: (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias). <br />- has a better prognosis<br />2- Progressive systemic sclerosis. <br />
  24. 24. Dermatomyositis<br />Symmetric proximal muscle weakness (myositis); <br />photosensitivity; <br /><ul><li>Heliotrope: periorbital edema with a violaceous hue. </li></ul>- Gottron's papules: papules and plaques on the hands, elbows, and knees and <br />Telangiectatic plaques with atrophy and hypopigmentation (poikiloderma) on the face, neck, trunk, and extremities; <br />Nail abnormalities (periungualtelangiectases and cuticular hypertrophy).<br />In adults: Dermatomyositis has a strong association with neoplasm of the breast, GIT, or lung.<br />
  25. 25. 7- Gastrointestinal disease<br />Dermatitis Herpetiformis (DH)<br />AcrodermatitisEnteropathica (ACE)<br />Necrolytic Migratory Erythema(glucagonoma syndrome)<br />Lichen planus (LP)<br />Hereditary Hemorrhagic Telangiectasia (HHT)<br />Peutz-Jeghers Syndrome<br />PyodermaGangrenosum<br />
  26. 26. Dermatitis Herpetiformis (DH)<br />is a chronic, intensely pruritic blistering disease <br />Most patients have an asymptomatic gluten-sensitive enteropathy<br />less commonly thyroid disease. <br />
  27. 27. AcrodermatitisEnteropathica (ACE)<br />Is an inherited or acquired condition due to zinc deficiency. <br />Acral and periorificial: pustules, bullae and scaling.<br />Most patients have diarrhea.<br />Treatment is zinc supplementation.<br />
  28. 28. Necrolytic Migratory Erythema(glucagonoma syndrome)<br />is rare, characterized by erythematous, scaly plaques <br /> on Acral, Periorificial or Intertriginous areas + <br /> hyperglycemia, diarrhea, weight loss, and atrophic glossitis.<br />Is in association with an islet cell tumor of the pancreas. <br />Treatment is rmoval of the tumor.<br />
  29. 29. Lichen planus<br />violaceous, flat, polygonal papules on the flexor aspects of the wrists, trunk, medial thighs, genitalia, and oral mucosa. <br />May occur with: <br /> - Primary biliary cirrhosis <br /> - Hepatitis B virus immunization or <br /> - Gold therapy <br /> - In hepatitis C patients: <br /> Oral erosive lichen planus.<br />
  30. 30. Hereditary Hemorrhagic Telangiectasia<br />is an autosomal dominant disorder <br />characterized by numerous telangiectases on the skin and oral mucosa. <br />Recurrent epistaxisis the most common (85 - 90%) presenting manifestation. <br />Telangiectases can involve the lungs, liver, brain, eyes, and GIThemorrhage can occur at any site. <br />Pulmonary arteriovenous fistulae and central nervous system angiomas can also occur.<br />
  31. 31. Peutz-Jeghers Syndrome<br />lentigines (Lentigo: a brown pigmented spot ) on the skin (periorbital region, dorsal surfaces of the fingers and toes) and mucosa (lips, buccal mucosa) <br />Associated with hamartomas (polyps) of the stomach, small intestine, and colon. <br />
  32. 32. PyodermaGangrenosum<br />painful ulcers with boggy, undermined edges and a border of gray or purple pigmentation. <br />The ulcers often follow trauma (pathergy) and begin as pustules or nodules that ulcerate and extend centrifugally.<br />legs are the most common site for the ulcers. <br />50% of patients have underlying rheumatoid arthritis or inflammatory bowel disease.<br />
  33. 33. 8- Renal diseaseNephrogenic Systemic Fibrosis(Nephrogenicfibrosingdermopathy) <br />Resembles scleroderma. <br />Thick, indurated plaques on the extremities and the trunk. <br /> can be progressive, leading to joint contractures. <br />In end-stage renal disease<br />Patients on dialysis.<br />Acute renal failure or <br />After kidney transplantation. <br />
  34. 34. 9- Endocrine and metabolic disease<br />Porphyrias: are inherited or acquired disorders of heme biosynthesis. Erythropoietic, Hepatic, or mixed in nature. <br />Porphyriacutaneatarda (PCT): is hepatic & the most common porphyria, <br />Precipitating factors: alcohol ingestion, estrogen administration, certain hepatotoxins (dinitrochlorobenzene, carbon tetrachloride), HIV infection, hemochromatosis, and hepatitis C infection. <br />Manifestations of PCT include: <br />- photosensitivity<br />- skin fragility <br />- bullae and erosions on sun-exposed skin <br /> (especially dorsal hands), and hypertrichosis. <br />Treatment includes phlebotomy and antimalarial drugs.<br />
  35. 35. Diabetes Mellitus-Related Skin Conditions<br />Cutaneous Manifestations of Diabetes Mellitus: from most to least common.<br />30-50% of diabetic patients develop skin disease. <br />1- Diabetic dermopathy (shin spots): <br />Atrophic, hyperpigmentedpapules and plaques on the legs<br />
  36. 36. 2- Diabetic thick skin: <br />3- Acanthosisnigricans: Common with obesity and diabetes<br />4- Yellow nails and skin (palms and soles): <br />5- Acquired perforating disorders <br />6- Calciphylaxis: isvascular calcification, thrombosis and skin necrosis: Painful purpuric plaques with ulceration<br />7- Necrobiosislipoidicadiabeticorum<br />8- Diabetic bullae<br />