Skin Manifestations Of Systemic Diseases

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skin manifestations of systemic diseases

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Skin Manifestations Of Systemic Diseases

  1. 1. SKIN MANIFESTATIONS OF SYSTEMIC DISEASES BY Professor M.YOUSRY ABDEL_MAWLA
  2. 2. Skin Manifestations of Systemic Disease <ul><li>Skin signs of internal malignancy </li></ul><ul><li>Skin signs of internal disease other than malignancy </li></ul><ul><li>Drug eruptions </li></ul>
  3. 3. Skin signs of internal malignancy <ul><li>cutaneous metastases </li></ul><ul><li>paraneoplastic syndromes </li></ul><ul><li>heritable &quot;cancer syndromes&quot; </li></ul>
  4. 4. Sister Mary Joseph nodule <ul><li>Umbilical metastasis; poor prognosis </li></ul><ul><li>precedes or follows diagnosis of CA </li></ul><ul><li>CA sites (decreasing order of frequency): colon, ovary, pancreas, endometrium, breast, small bowel </li></ul>
  5. 5. Paraneoplastic Syndromes <ul><li>The skin often presents a clue that an internal malignancy is present. The combination of a malignancy and associated signs and symptoms that are seemingly unrelated to the actual tumor is called a &quot;paraneoplastic&quot; syndrome </li></ul>
  6. 6. Erythema gyratum repens <ul><li>&quot;Wood-grain&quot; pattern </li></ul><ul><li>Wavy, erythematous, urticarial bands with scale </li></ul><ul><li>Slowly migrate </li></ul><ul><li>Breast, stomach, bladder, prostate, cervix; occasionally no CA </li></ul>
  7. 7. Glucagonoma syndrome -- necrolytic migratory erythema <ul><li>Alpha cell tumor of the pancreas; occasionally no neoplasm found </li></ul><ul><li>abdomen, thighs and buttocks </li></ul><ul><li>Patchy erythema with flaccid vesicles and bullae </li></ul><ul><li>Glossitis, angular cheilitis, normocytic anemia, low amino acid levels in seum </li></ul>
  8. 8. Sweet’s syndrome (acute febrile neutrophilic dermatosis ) <ul><li>Painful red plaques and papules - face, neck, upper chest, arms, legs </li></ul><ul><li>Usually females (4:1); fever, leukocytosis prominent </li></ul><ul><li>Associations, benign: URIs, strep, RA, Crohns, sarcoidosis, Behcet’s, pregnancy </li></ul><ul><li>Association, malignant: AML, myelodysplasis, lymphoma--may follow by months to years </li></ul>
  9. 9. Trousseau’s sign <ul><li>Superficial migratory thrombophlebitis and neoplasia </li></ul><ul><li>75% of CA in pancreas, stomach, lung, prostate, hematopoietic </li></ul><ul><li>Hypercoagulable state; thrombophlebitis resistant to therapy . </li></ul>
  10. 10. heritable &quot;cancer syndromes <ul><li>Peutz-Jehger </li></ul><ul><li>Multiple hamartomatous polyps in small bowel (most common), stomach and colon; low risk of bowel malignancy </li></ul><ul><li>multiple lentigines (freckle-like) of lips, nose, oral mucosa, fingertips and nail beds </li></ul><ul><li>non-intestinal malignancies increased: lung, ovary, endometrium pancreas, myeloma </li></ul>
  11. 11. Skin signs of internal disease <ul><li>Pyoderma gangrenosum </li></ul><ul><li>Cullen’s sign </li></ul><ul><li>Xanthomas </li></ul><ul><li>Acanthosis nigricans (AN) </li></ul><ul><li>Erythema nodosum </li></ul>
  12. 12. Pyoderma gangrenosum <ul><li>Rapidly expanding ulcer with bluish undermined border; often lower extremities; begin as sterile pustules </li></ul><ul><li>50% no disease association </li></ul><ul><li>1% to 10% of patients with active ulcerative colitis; often (but not always) parallels disease </li></ul><ul><li>Other associations: Crohn’s, chronic active hepatitis, rheumatoid arthritis, HIV infection; acute and chronic granulocytic leukemia (bullous PG) </li></ul><ul><li>Associated with underlying malignancy (leukemia, etc.) </li></ul>
  13. 13. Cullen’s sign <ul><li>Periumbilical purpura associated with acute pancreatitis </li></ul><ul><li>Hematomas dissect along fascial plans from the retroperitoneal site of bleeding to the periumbilical area </li></ul><ul><li>Turner’s sign- -purpura of the left flank; same cause </li></ul>
  14. 14. Xanthomas <ul><li>A sign of systemic metabolic abnormality or a local cellular dysfunction </li></ul><ul><li>Xanthomas may be the first sign of one the hyperlipoproteinemias, rare but serious metabolic diseases </li></ul><ul><li>Xanthelasma : xanthomas of eyelids that may or may not be associated with hyperlipidemia </li></ul>
  15. 15. Acanthosis nigricans (AN) <ul><li>Velvety thickening and darkening (hyperpigmentation) of the skin, especially on the nape of the neck, axillae and other body folds   </li></ul><ul><li>Causes </li></ul><ul><li>hereditary </li></ul><ul><li>acquired, : obesity; drugs; &quot;malignant&quot;acanthosis nigricans; Diabetes Mellitus </li></ul><ul><li>hyperinsulinemia is a common denominator </li></ul>
  16. 16. Erythema nodosum <ul><li>Deep erythematous painful nodules, symmetrically on the lower legs; female predominance; a hypersensitivity panniculitis </li></ul><ul><li>Fever, chills, malaise, leukocytosis </li></ul><ul><li>Disease associations: streptococcal infections, drugs (OCPs, sulfonamides, iodides), pregnancy, TB, deep mycoses, acute sarcoidosis, inflammatory bowel disease </li></ul>
  17. 17. Skin Manifestations of other Systemic Diseases <ul><li>Gastrointestinal Disease </li></ul><ul><li>Diabetes mellitus </li></ul><ul><li>Liver disease </li></ul><ul><li>Kidney Diseases </li></ul><ul><li>Other disorders </li></ul><ul><li>Sarcoidosis </li></ul><ul><li>Leucocytoclastic vasculitis in SLE </li></ul>
  18. 18. Gastrointestinal Disease <ul><li>Inflammatory Bowel Disease </li></ul><ul><li>Pyoderma gangrenosum </li></ul><ul><li>Erythema nodosum </li></ul><ul><li>Aphthous stomatitis . </li></ul><ul><li>Mucosal nodularity (cobblestoning). </li></ul><ul><li>Pyostomatitis vegetans (erythematous thickened mucosa) </li></ul>
  19. 19. <ul><li>Gastrointestinal disorders Cutaneous disorders </li></ul><ul><li>Inflammatory bowel disease </li></ul><ul><li>Ulcerative colitis Neutrophilic tissue reactions </li></ul><ul><li>Crohn’s disease Pyoderma gangrenosum </li></ul><ul><li>Bowel – bypass syndrome Sweet’s syndrome </li></ul><ul><li>Pustular vasculitis </li></ul><ul><li>Erythema nodosum </li></ul><ul><li>Aphthous stomatitis </li></ul>
  20. 20. <ul><li>Malabsorption </li></ul><ul><li>Gluten-sensitive enteropathy </li></ul><ul><li>Alcoholic liver disease </li></ul><ul><li>Acrodermatitis enteropathica </li></ul><ul><li>Dermatogenic enteropathy </li></ul><ul><li>Pancreatic panniculitis </li></ul><ul><li>Porphyria cutanea tarda </li></ul><ul><li>Dermatitis herpetiformis </li></ul>Nutritional and Metabolic disorders
  21. 21. Infections <ul><li>Hepatitis B and C </li></ul><ul><li>Helicobacter pylori </li></ul><ul><li>Porphyria cutanea tarda </li></ul><ul><li>Erosive or oral lichen planus </li></ul><ul><li>Sweet's syndrome </li></ul>
  22. 22. Malignancies <ul><li>Glucagonoma </li></ul><ul><li>Carcinoid </li></ul><ul><li>Esophageal carcinoma   </li></ul><ul><li>Nonspecific cutaneous signs of gastrointestinal malignancy </li></ul><ul><li>Necrolytic migratory erythema </li></ul><ul><li>Flushing </li></ul><ul><li>Bazex's syndrome </li></ul><ul><li>Palmar-plantar hyperkeratosis </li></ul><ul><li>Koilyonychia </li></ul><ul><li>Glossitis </li></ul><ul><li>Acanthosis nigricans </li></ul><ul><li>Erythema nodosum </li></ul><ul><li>Metastatic skin lesions (eg adenocarcinoma of colon </li></ul><ul><li>Sister Mary Joseph's nodules) </li></ul>
  23. 23. Malignancies <ul><li>Gastrointestinal polyposis syndromes and gastrointestinal cancer </li></ul><ul><li>Peutz-Jeghers syndrome </li></ul>
  24. 24. Gastrointestinal hemorrhage <ul><li>Vascular disorders </li></ul><ul><li>Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) </li></ul><ul><li>Kaposi's sarcoma </li></ul><ul><li>Necrotizing angiitis </li></ul><ul><li>Pseudoxanthoma elasticum </li></ul><ul><li>Ehlers-Danlos syndrome </li></ul><ul><li>Degos, disease </li></ul><ul><li>Henoch-Schönlein purpura </li></ul>
  25. 25. Dermatologic Manifestations of liver disease <ul><li>Vascular </li></ul><ul><li>a. Spider telangiectasia, </li></ul><ul><li>b. Palmer erythema, </li></ul><ul><li>c. Corkscrew scleral vessels, </li></ul><ul><li>d. Caput medusae, </li></ul><ul><li>e. Plethoric facies, </li></ul><ul><li>f. Flushing, </li></ul><ul><li>g. Unilateral nevoid telangiectasia. </li></ul><ul><li>Jaundice, </li></ul><ul><li>Pruritus, </li></ul><ul><li>Urticaria, </li></ul><ul><li>Nail changes – </li></ul><ul><li>Koilonychia, Clubbing. </li></ul><ul><li>Hyperpigmentation , </li></ul><ul><li>Skin cancer, </li></ul><ul><li>Lichenoid dermatitis, </li></ul>
  26. 35. Skin manifestations of Renal diseases <ul><li>Uremic pruritus :generalized or localized </li></ul><ul><li>Excoriations, lesions of lichen simplex chronicus, and prurigo nodularis. </li></ul><ul><li>Mediated by various chemical mediators neurotransmitters: (pentapeptide enkephalins, proteases, </li></ul><ul><li>serotonin, and histamine..) </li></ul>
  27. 36. Skin manifestations of Renal diseases <ul><li>2-Xerosis : the dry or roughened skin texture </li></ul><ul><li>A direct relation between xerosis and stratum corneum water content. </li></ul><ul><li>A thicker though fractured stratum corneum leads to increase transepidermal water loss. </li></ul><ul><li>3-Cutaneous pigmentation:pallor , brown-to-slate-gray discoloration, yellowish (sallow) hue, and brownish hyperpigmentation in sun-exposed areas. </li></ul><ul><li>Due to increased levels of melanocyte stimulating hormone (MSH) </li></ul>
  28. 37. Skin manifestations of Renal diseases <ul><li>4-Calciphylaxis: a life-threatening condition of progressive cutaneous necrosis due to small- and medium-sized vessel calcification. </li></ul><ul><li>A complication of secondary hyperparathyroidism in chronic renal failure. </li></ul><ul><li>Firm, bilaterally symmetric non-ulcerating painful </li></ul><ul><li>preinfarctive ischemic plaques that appear as mottling or violaceous discoloration on the extremities developing into necrotic areas. ---_Flaccid or hemorrhagic bullae may form over ischemic tissue </li></ul>
  29. 38. Diabetes mellitus <ul><li>Vascular microangiopathies and rubeosis </li></ul><ul><li>Recurrent skin infections :, either due to fungi (e.g. genital candidiasis ) or bacteria (e.g. folliculitis). </li></ul><ul><li>Blisters on the feet </li></ul><ul><li>Wet gangrenes </li></ul><ul><li>Peripheral neuropathy and trophic skin changes </li></ul><ul><li>Brown macules sometimes develop on the shin </li></ul><ul><li>Plaques with dark red or purple edges, atrophic centres, and surface telangectasia (Necrobiosis lipoidica diabeticorum ). </li></ul>
  30. 39. Drug eruptions <ul><li>Urticaria </li></ul><ul><li>Morbilliform </li></ul><ul><li>Fixed drug eruption </li></ul><ul><li>Erythema multiforme </li></ul><ul><li>Stevens-Johnson Syndrome </li></ul><ul><li>Toxic epidermal necrolysis </li></ul>
  31. 40. Urticaria (hives) <ul><li>Drugs (e.g., penicillins) are a common cause of urticaria, but urticaria can be precipitated by other internal and external factors </li></ul><ul><li>Primary lesion is a wheal, a flesh-colored to pink, well circumscribed plaque caused by dermal edema; itchy! </li></ul><ul><li>Individual lesions last only a few hours, never more than 24 hours </li></ul><ul><li>When caused by drugs, may be IgE mediated, triggering mast cell granule release; or drug may directly cause mast cell granule release </li></ul>
  32. 41. Morbilliform eruption (exanthematous drug eruption, maculopapular drug eruption) :   <ul><li>&quot;morbilliform&quot; refers to a resemblance to the rash of measles (morbilli is Latin for measles); measles is a rare disease now, but morbilliform eruptions are common </li></ul><ul><li>a morbilliform eruption is symmetrically distributed on the trunk and proximal extremities, and consists of bright pink macules and slightly raised papules (&quot;maculopapular&quot;) </li></ul>
  33. 42. Fixed drug eruption <ul><li>Fixed&quot; in that it occurs at same sites with each episode </li></ul><ul><li>OTC drugs containing phenolphthalein, pseudoephedrine, etc. common culprits </li></ul><ul><li>tetracyclines, barbiturates, phenothiazines, sulfonamides </li></ul><ul><li>oval, itchy or burning dusky red plaque </li></ul>
  34. 43. Erythema multiforme <ul><li>A form of cutaneous reaction to an underlying condition. In 50% of cases, a cause can’t be identified </li></ul><ul><li>Common causes : </li></ul><ul><li>Drugs (sulfonamides, phenytoin, barbiturates, penicillin, etc.); infections (esp. herpes simplex and Mycoplasma); inflammatory bowel disease </li></ul><ul><li>Eruption usually lasts for a week or two, then spontaneously remits </li></ul><ul><li>the &quot;target&quot; lesion is approximately 1cm dull-red macule or papule with a central area of blistering or hemorrhage </li></ul>
  35. 44. Toxic epidermal necrolysis (TEN) <ul><li>it is unclear whether TEN is a severe form of erythema multiforme or a distinct disease </li></ul><ul><li>80% of cases have a strong association with a specific drug (list is similar to that for erythema multiforme) </li></ul><ul><li>TEN is a medical emergency, on the order of a total body burn </li></ul>
  36. 45. THANK YOU

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