Cutaneous Manifestations of Systemic Disease

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Cutaneous Manifestations of Systemic Disease

  1. 1. Cutaneous Manifestations of Systemic Disease<br />Holly Edmonds, MD<br />Chief Resident<br />Department of Dermatology<br />
  2. 2. Brief Review of terminology…<br />
  3. 3. Zits???<br />
  4. 4. Red Rash???<br />
  5. 5. “Nasty… just call the derm service and get a consult!”<br />
  6. 6. You have to learn the language!<br />
  7. 7. Description of a skin lesion:<br />Type<br />Shape<br />Arrangment <br />Distribution<br />OH!!! DATS what that rash is!!!<br />
  8. 8. TYPE<br />Primary lesion<br />Secondary lesion<br />Color <br />Palpation<br />
  9. 9. MACULE/PATCH<br />
  10. 10.
  11. 11. PLAQUE<br />
  12. 12.
  13. 13. PAPULE<br />
  14. 14.
  15. 15. NODULE<br />
  16. 16.
  17. 17. VESICLE/BULLA<br />
  18. 18.
  19. 19.
  20. 20. PUSTULE<br />
  21. 21.
  22. 22. WHEAL<br />
  23. 23.
  24. 24. SCALE<br />
  25. 25.
  26. 26. CRUST<br />
  27. 27.
  28. 28. LICHENIFICATION<br />
  29. 29.
  30. 30. EROSION<br />
  31. 31.
  32. 32. ULCER<br />
  33. 33.
  34. 34. ATROPHY<br />
  35. 35.
  36. 36. EXCORIATION<br />
  37. 37.
  38. 38. FISSURE<br />
  39. 39.
  40. 40. SCAR<br />
  41. 41.
  42. 42. Color<br />
  43. 43. Color<br />
  44. 44. Color<br />
  45. 45. Palpation<br />Consistency <br />Firm, Soft, Fluctuant, Boardlike<br />Temperature deviation (hot or cold)<br />Mobility<br />Presence of tenderness<br />
  46. 46. Margination<br />Ill-defined<br /> vs.<br /> Well-defined<br />
  47. 47. SHAPE<br />
  48. 48. SHAPE<br />
  49. 49. SHAPE<br />
  50. 50. SHAPE<br />
  51. 51. SHAPE<br />
  52. 52. ARRANGEMENT<br />
  53. 53. ARRANGEMENT<br />
  54. 54. ARRANGEMENT<br />
  55. 55. ARRANGEMENT<br />
  56. 56. ARRANGEMENT<br />
  57. 57. DISTRIBUTION<br />
  58. 58. DISTRIBUTION<br />
  59. 59. DISTRIBUTION<br />
  60. 60. DISTRIBUTION<br />
  61. 61. DISTRIBUTION<br />
  62. 62. Cutaneous Manifestations <br />Diabetes Mellitus <br />Renal Disease<br />Gastrointestinal disorders <br />Rheumatologic disease<br />Hepatitis C<br />Thyroid disease<br />Paraneoplastic disease<br />Nutritional disease<br />
  63. 63. Diabetes Mellitus<br />Acanthosis Nigricans<br />Diabetic Dermopathy<br />Bullosis Diabeticorum<br />Necrobiosis Lipoidica<br />Diabetic Foot Ulcers<br />
  64. 64. Acanthosis Nigricans<br />African Americans and Hispanics > Caucasians<br />Associated with obesity, insulin resistance<br />Hyperpigmented velvety plaques of the flexures<br />Genetic sensitivity of the skin to hyperinsulinemia<br />Malignant form a/w gastric ACA<br />
  65. 65. Acanthosis Nigricans<br />
  66. 66. Acanthosis Nigricans<br />
  67. 67. Diabetic Dermopathy<br />AKA “shin spots” or pigmented pretibial papules<br />Most common cutaneous manifestation of diabetes<br />Benign asymptomatic red brown macules on shins<br />No treatment needed<br />
  68. 68. Diabetic Dermopathy<br />
  69. 69. Bullosis Diabeticorum<br />Rapid onset painless, tense blisters on hands and feet<br />Trauma and microvascular disease may play a role<br />Spontaneous healing in 2-5 weeks<br />
  70. 70. Bullosis Diabeticorum<br />
  71. 71. Necrobiosis Lipoidica<br />20-35% of patients with NLD have diabetes, only about 1-3% of diabetics have NLD<br />Start as red-brown papules and progress to well defined yellow-brown atrophic plaques with irregular violaceous borders and telangiectasias. Shins #1 site. <br /> Ulceration in 35%. <br />Glucose control will not clear NLD<br />
  72. 72. Necrobiosis Lipoidica Diabeticorum<br />
  73. 73. Diabetic Eruptive Xanthomas<br />Seen in uncontrolled diabetes, hypertriglyceridemia<br />Sudden crops on firm, non-tender yellow papules with a red rim on extensors<br />Control glucose and lipid reduction will reduce lesions<br />
  74. 74. Diabetic Eruptive Xanthomas<br />
  75. 75. Diabetic Neurotropic Ulcers<br />Peripheral neuropathy leads to unnoticed trauma<br />Vascular complications may lead to ulcers and complicate ulcer healing<br />Risk of amputation goes up 8x once these develop<br />
  76. 76. Diabetic Neurotropic Ulcers<br />
  77. 77. Renal disease<br />Pruritis<br />Perforating dermatosis<br />Calciphylaxis<br />Nephrogenic Fibrosing Dermopathy<br />
  78. 78. Pruritis<br />Most common cutaneous manifestation of renal disease<br />Seen in both peritoneal and hemodialysis patients<br />Unknown mechanism, unsatisfactory therapy- UVB helps the most<br />
  79. 79. Pruritis<br />
  80. 80. Perforating Disorder<br />Acquired Perforating Dermatosis of ESRD<br />Umbilicated papules/nodules with central hyperkeratotic core<br />
  81. 81. Perforating dermatosis<br />
  82. 82. Perforating dermatosis<br />
  83. 83. Calciphylaxis<br />Painful purpuric plaques and retiform purpura<br />More proximal lesions = poorer prognosis<br />
  84. 84. Calciphylaxis<br />
  85. 85. Nephrogenic Fibrosing Dermopathy<br />Woody, indurated plaques with peau d’orange appearance<br />Usually spares the face, palms, soles<br />Associated with gadolinium contrast for MRIs<br />
  86. 86. Nephrogenic Fibrosing Dermopathy<br />
  87. 87. Gastrointestinal disorders<br />Henoch Schonlein Purpura<br />Dermatitis Herpetiformis<br />Inflammatory Bowel Disease<br />
  88. 88. Henoch Schonlein Purpura<br />Palpable purpura, urticaria, necrotic ulcers on buttocks, distal legs<br />Symmetric<br />IgAvasculitis<br />GI symptoms + arthritis, long term concern for hypertension and renal involvement<br />Usually under 20, following an URI<br />
  89. 89. HSP<br />
  90. 90. HSP<br />
  91. 91. Dermatitis Herpetiformis<br />Symmetric, grouped vesicles on extensors<br />Very pruritic!<br />All patients have gluten-sensitive enteropathy, only 20% symptomatic<br />Associated with HLA-DQ2, Hashiomoto’s thyroiditis, lymphoma, IDDM<br />Cutaneous findings are due to autoantibodies to epidermal transglutaminase<br />Treatment- rapidly responsive to dapsone<br />
  92. 92. Dermatitis Herpetiformis<br />
  93. 93. Inflammatory Bowel Disease<br />Crohn’s and Ulcerative Colitis<br />
  94. 94. Oral Crohn’s<br /><ul><li>Linear ulcerations, cobblestoned oral mucosa
  95. 95. UC may have aphthous ulcers that develop as IBD flares</li></li></ul><li>Metastatic Crohn’s<br /><ul><li>See fissures and fistulas with Crohn’s
  96. 96. Metastatic Crohn’s are nodules, plaques and ulcerations usually in intertriginous areas which can mimic erythema nodosum</li></li></ul><li>Erythema Nodosum<br /><ul><li>Tender red nodules on anterior lower legs, precedes or occurs with IBD flares, UC more common
  97. 97. Most EN is idiopathic, also can be related to oral contraceptives or abx, preceding strep or mycobacterial infxn</li></li></ul><li>Pyoderma Gangrenosum<br /><ul><li>More common in UC
  98. 98. Papules, pustules, hemorrhagic blisters enlarge and ulcerate with dusky undermined edges
  99. 99. Frequently on legs or around stoma sites
  100. 100. Treat with steroids, often gets better as IBD gets better</li></li></ul><li>Rheumatologic Disorders<br />Lupus Erythematosis<br />Dermatomyositis<br />Reiter’s Disease<br />
  101. 101. Lupus Erythematosis<br />Classification:<br />Systemic Cutaneous Lupus (SLE)<br />Subacute Cutaneous Lupus (SCLE)<br />Discoid Lupus (DLE)<br />Neonatal Lupus<br />
  102. 102. SLE<br />+ANA <br />+Sm and dsDNA<br />Butterfly Rash<br />Poikiloderma <br />Photodistrubited erythematous, papular scaling eruption sparing knuckles.<br />
  103. 103. SLE<br />
  104. 104. SLE<br />
  105. 105. SLE<br />
  106. 106. Dermatomyositis<br />Poikiloderma favoring scalp, periocular (Heliotrope rash), and extensor skin sites <br />Nailfold telangiectasias<br />Gottron’s papules<br />
  107. 107. Dermatomyositis<br />
  108. 108. Dermatomyositis(periungal telangiectasias, gottrons papules)<br />
  109. 109. Reiter’s Disease<br />Urethritis, arthritis, ocular findings, and oral ulcers in addition to psoriasiform skin lesions. <br />Keratoderma blenorrhagicum (feet)<br />Balanitis circinata (penis)<br />
  110. 110. Reiter’s DiseaseKeratoderma Blenorrhagicum<br />
  111. 111. Reiter’s Disease(balanitis circinata)<br />
  112. 112. Hepatitis C virus<br />Porphyria Cutanea Tarda<br />Lichen Planus<br />Pruritis<br />
  113. 113. Porphyria Cutanea Tarda<br />Vesicles and bullae on sun-exposed areas, scarring with milia<br />Hypertrichosis<br />Fragile skin with sclerodermoid changes<br />
  114. 114. PCT<br />
  115. 115. PCT(hypertrichosis)<br />
  116. 116. Lichen Planus<br />Purple, pruritic, polygonal papules<br />Koebner phenomenon<br />Wickham’s striae<br />50% with mucosal involvement<br />
  117. 117. Lichen Planus<br />
  118. 118. Mucosal Lichen Planus<br />
  119. 119. Pruritis<br />Excoriations, lichenification, and prurigo nodularis <br />
  120. 120. Prurigo Nodularis<br />
  121. 121. Pruritis<br />
  122. 122. Thyroid disease<br />Graves disease<br />Hyperthyroidism<br />Hypothyroidism<br />
  123. 123. Graves Disease<br />Thyroid dermopathy (pretibial myxedema)<br />Symmetric, non-pitting yellow-brown waxy papules/plaques<br />Due to increased hyaluronic acis in dermis<br />
  124. 124. Thyroid Dermopathy<br />
  125. 125. Hyperthyroidism<br />Warm, moist skin<br />Flushing, palmar erythema<br />Associated with reversible alopecia and vitiligo<br />
  126. 126. Vitiligo<br />
  127. 127. Hypothyroidism<br />Dry, cool skin<br />Generalized myxedema<br />Yellow hue from carotenemia<br />Purpura from delayed wound healing<br />Alopecia, madarosis <br />
  128. 128. Carotenemia<br />
  129. 129. Paraneoplastic Disorders<br />Acanthosis Nigricans<br />Dermatomyositis<br />Cushing’s Disease<br />Sign of Lesser-Trelat<br />Paraneoplastic Pemphigus<br />Hypertrichosis Lanuginosa<br />
  130. 130. Acanthosis Nigricans<br />
  131. 131. Dermatomyositis<br />
  132. 132. Cushing’s syndrome(buffalo hump and striae)<br />
  133. 133. Sign of Lesser-Trelat<br />
  134. 134. Paraneoplastic Pemphigus<br />
  135. 135. Erythema Gyratum Repens(associated with cancers above diaphragm)<br />
  136. 136. Hypertricosis Lanuginosa Acquisita<br />
  137. 137. Nutritional Disorders<br />1. Marasmus<br />2. Kwashiorkor<br />3. Pellagra<br />4. Scurvy<br />5. Zinc deficiency<br />
  138. 138. Marasmus<br />Marasmus = protein/calorie malnutrition.<br />Cutaneous Manifestations:<br />Emaciation with thin, lax, and wrinkled skin.<br />Fine scaling with hyperpigmentation.<br />Follicular hyperkeratosis<br />Purpura<br />Thin hair and nails.<br />
  139. 139. Marasmus<br />
  140. 140. Marasmus<br />
  141. 141. Kwashiorkor<br />Decreased protein intake<br />Dyschromia (irregular pigment)<br />Hypopigmentation.<br />Superficial desquamation with areas of erosion (flaky paint)<br />Petechia/purpura<br />Thin hair/nails<br />
  142. 142. Kwashiorkor<br />
  143. 143. Kwashiorkor<br />
  144. 144. Pellagra<br />Niacin deficiency (Vit B3)<br />Triad of dermatitis, diarrhea, dementia<br />Photosensitive eruption around neck known as “Casal’s necklace”<br />
  145. 145. Pellagra<br />
  146. 146. Pellagra<br />Casal’s neckace<br />
  147. 147. Scurvy<br />Vitamin C deficiency<br />Follicular hyperkeratosis with corkscrew hairs<br />Perifollicular hemorrhage<br />Gingival hypertrophy with erosive bleeding gums.<br />
  148. 148. Scurvy<br />
  149. 149. Zinc Deficiency<br />Acquired <br /> -deficient intake, high fiber intake, malabsorption<br />Inherited (acrodermatitis enteropathica) zinc deficiency<br /> -defect in intestinal absorption of zinc <br />Dermatitis, diarrhea, alopecia<br />Periorificial and acral distribution<br />
  150. 150. Acrodermatitis Enteropathica<br />
  151. 151. Acrodermatitis Enteropathica<br />
  152. 152. A couple of randoms you should know…<br />
  153. 153. Sarcoidosis<br />Multisystem granulomatous disease<br />Skin affected in 20-35%<br />
  154. 154.
  155. 155.
  156. 156. Neurofibromatosis<br />Von Reckinghausen’s disease<br />Autosomal dominant<br />Neurofibromin gene, Chr 17<br />
  157. 157.
  158. 158.
  159. 159.
  160. 160. Tuberous Sclerosis<br />Autosomal Dominant<br />Hamartin and Tuberin (TSC 1 and 2)<br />MR, Seizures (variable)<br />
  161. 161.
  162. 162.
  163. 163.
  164. 164.
  165. 165.
  166. 166.
  167. 167. QUIZ TIME!!!<br />
  168. 168. Very itchy.<br />
  169. 169. Lichen Planus (HCV)<br />
  170. 170. Anterior lower leg<br />
  171. 171. Necrobiosis Lipoidica (DM)<br />
  172. 172. ?<br />
  173. 173. Pyoderma Gangrenosum (IBD)<br />
  174. 174. ?<br />
  175. 175. Porphyria Cutanea Tarda<br />
  176. 176. ?<br />
  177. 177. SLE<br />
  178. 178. ?<br />
  179. 179. Basal Cell Carcinoma<br />
  180. 180.
  181. 181. Nodulocystic Acne<br />
  182. 182.
  183. 183. Xanthelasma<br />
  184. 184.
  185. 185. Herpes Labialis<br />
  186. 186.
  187. 187. Nevus Sebaceous<br />

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