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Cutaneous Manifestations of Systemic Disease Flashcards

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1
Q

discoid LE (lupus erythematosus)

  • has what other name
  • pathogenesis
  • demographics
  • presentation
  • dx
  • complications
A

= chronic cutaneous LE

  • pathogenesis: UVB radiation triggers a exlusively cutaneous reaction
  • demographics: AA women
  • presentation:
    • indurated erythematous plaques on face/neck/scalp ears with:
      • scarring
      • surrounding hair loss
      • follicular plugging
    • NO systemic sx!
  • dx: only 5% ANA positive
  • complications: can progress to -> SLE
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2
Q

subacute cutaneous LE (SCLE)

  • pathogenesis
  • demographics
  • presentation
  • dx
  • complications
A
  • pathogenesis: sun exposure triggers cutaneous disease + some internal involvement
  • demographics: female caucasion
  • presentation:
    • polycyclic plaques on sun-exposed areas that are:
      • annular with central clearing
      • no scarring
  • dx:
    • 60-80% ANA
    • anti-Ro/SSA (overlaps with sjogrens)
  • complications: can progress to SLE
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3
Q

compare and contract discoid LE and SCLE in terms of

  • location of lesions
  • presence of scarring
  • presence of systemic sx
  • lab findings
  • progression to SLE
A
  • discoid LE (chronic cutaneous LE)
    • localized to face / neck / scalp / ears
    • heals with scarring
    • no systemic sx
    • lab findings: 5% ANA
    • less likely to progress to SLE
  • subacute cutaneous LE (SCLE)
    • lesions on sun exposed areas
    • NO scarring
    • mild systemic sx - arthalgia/arthritis
    • lab findings: 80% ANA + anti-Ro/SSA
    • more likely to progress to SLE
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4
Q

neonatal lupus erythema

  • pathogenesis
  • demographics
  • presentation
  • dx
  • complications
A
  • pathogenesis: transplacental passage of maternal anti-Ro/SSA Abs
  • demographics: neonates
  • presentation: similar to SCLE, but more facial involvement
    • annular lesions
    • periorbital erythema
  • dx: anti-Ro / SSA antibodies
  • complications: heart block
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5
Q

acute cutaneous lupus (ACLE)

  • pathogenesis
  • demographics
  • presentation
  • dx
  • complications
A
  • pathogenesis:
  • demographics: AA women
  • presentation:
    • skin: malar erythema (“butterfly rash”) + dorsal hands
      • malar erythema: overal nasal bridge & bilateral malar cheeks + spares nasolabial fold
      • dorsal hands: spares the knuckles
    • systemic: kidney, heart
  • dx: ANA, anti-dsDNA, anti-Smith
  • complications: can progress to SLE (more likely than DLE or SCLE)
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6
Q

systemic lupus erythematous (SLE)

  • pathogenesis
  • demographics
  • presentation
  • dx
  • complications
A
  • pathogenesis: autoimmune, predipsposed by complement deficiency
  • demographics: AA females
  • presentation: SOAP-BRAIN MD
    • S - serositis (pleuritis, pericardiits)
    • O - oral ulcers
    • A - alopecia
    • P - photosensitivity
    • B - blood
    • R - reynauds / acronyanosis
    • A - arthritis
    • I - immune: ANA, anti-dsDNA, anti-Smith
    • N - neurologic
    • M - malar rash
    • D - discoid rash
  • dx: ANA, anti-dsDNA, anti-Smith
  • complications:
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7
Q

drug induced SLE

  • m/c causes?
  • dx?
A
  • drugs
    • hydralazine
    • procainamide
    • isoniazid
    • quinidine
  • dx: anti-histone positive & dsDNA negative
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8
Q

which variations are lupus are most / least likely to progress to lupus?

A

in order of most to least:

acute cutaneous lupus (ACLE) > subacute cutaneous lupus (SCLE) > discloid lupus (DLE)

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9
Q

summarize the lab findings for each type of lupus

A
  • DLE (chronic): 5% ANA
  • SCLE/neonatal: 80% ANA, + anti-Ro/SSA
  • ACLE/SLE: 99% ANA, + anti-Sm + anti-dsDNA
  • drug induced SLE: + anti-histone, - anti-dsDNA
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10
Q

lupus - management

A
  • prevention: sunscreen !!
  • treatment: hydrochloroquine (systemic) + topical steroids (cutaneous)
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11
Q

what are the variations of scleroderma?

A
  • localized scleroderma
    • morphea
    • linear
  • systemic scleroderma
    • limited aka CREST syndrome
    • diffuse aka progressive
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12
Q

localized scleroderma

  • pathogenesis
  • demographics
  • presentation
  • treatment
A
  • pathogenesis: CT disease characterized by excess collagen deposition in skin/organs
  • demographics: female predominant
  • presentation: plaques of expanding erythema -> become hairless
    • morpheoa form: trunk + proximal extremities
    • linear form: lower extremities (l for lower)
      • “en coupe de sabre” is a linear lesion on the forehead
  • treatment: steroids; if severe - METHOTREXATE
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13
Q

limited systemic sclerosis

  • pathogenesis
  • demographics
  • presentation
  • diagnosis
A
  • pathogenesis: excessive collagen deposition
  • demographics: female predominant
  • presentation: = CREST syndrome
    • C = calcinosis cutis
    • R = reynaud’s phenomenon
    • E esophageal dysmotility
    • S = sclerodactyolyl
    • T = telagiectasia
  • diagnosis: anti-centromere antibodies
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14
Q

diffuse scleroderma

  • pathogenesis
  • demographics
  • presentation
  • diagnosis
  • treatment
A
  • pathogenesis: CT disorder characterized by excess collagen deposition
  • demographics: female predomoinant
  • presentation:
    • skin:
      • shiny, “leather like” skin - “loss of wrinkles”
      • beaked nose
      • fingers:
        • edema
        • sclerodactyl
        • digital pitting ulcers on tips
    • systemic (more involvement than limited)
      • esophageal dysfunction (m/c)
      • renal & pulmonary
  • diagnosis: anti-Scl-70 (anti-topoisomerase)
  • complications: bilateral basilar pulmonary fibrosis is the m/c cause of death
  • treament: most important to control internal organ involvement
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15
Q

reynaud’s syndrome

  • can present in what systemic-cutaneous disorders?
  • is treated how?
A
  • disorders
    • SLE
    • systemic scleroderma (limited & diffuse)
  • treamtment:
    • first line:
      • AVOID COLD
      • SMOKIN CESSATION
    • next: vasodilating drugs (calcium channel blockers)
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16
Q

what features do limited scleroderma and diffuse scleroderma and diffuse scleroderma share? what are their differences?

A
  • both present with
    • esophageal dysmotility
    • reynaud’s
    • sclerodactyl
  • limited scleroderma only:
    • calcinosis
    • telangectiasis
  • diffuse scleroderma only:
    • finger edema w/ ulcers at the tip
    • significant renal & pulmonary involvement:
      • m/c cause of death = bilateral pulmonary dibrosis
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17
Q

dermatomyositis (DM)

  • pathogenesis
  • demographics
  • presentation
  • diagnosis
  • complications
  • treatment
A
  • pathogenesis: autoimmune CT disease
  • demographics: biomodal distribution - juvenile, adult forms
  • presentation: skin findings → muscle weakness
    • ​skin findings:
      • ​grotton’s papules: lichenoid papules over IP joints including knukcles
      • helitrope sign: red eyelids surrounded by white circle
      • shawl sign: redness on neck
    • muscle weakness that is
      • proximal
      • painless
      • disruptive of rising from seated position
  • diagnosis:
    • elevated CK (>1000)
    • antibodies
      • ​anti-Jo L (histadyl tRNA synthetase) - specific
      • Anti-Mi-2 (helicase) - if skin only, no muscle px
  • treatment:
    • ​skin only = photoprotection + topical steroids
    • muscle = prednisone until CK normalizes (if skin also: add MTX & azahthioprine = steroid sparing)
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18
Q

what are the muscle dysfunctions seen in dermatomyotisis?

A
  • proximal weakness
  • painless
  • diffuctly rising / walking up stairs
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19
Q

which antibodies are seen in dermatomyositis? explain.

A
  • anti-Jo (histadyl tRNA syntase) - highly specific
  • anti-Mi-2 (helicase) - in skin presentation only
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20
Q

how to tx dermatomyotisis with both skin & muscle presentation?

A

steroids (prednisone) + steroid sparing agents: MTX + azothioprine

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21
Q

peutz-jeghers syndrome

  • pathogenesis
  • presentation
  • complications
A
  • pathogenesis: STK11 mutation (tumor suppressor gene)
  • presentation:
    • skin: pigmented papules on oral mucosa
    • systemic: harmatomatous GI polyps - esp in jejunem
  • complications: increased risk of GI and non-GI malignancies
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22
Q

gardner syndrome

  • pathogenesis
  • presentation
  • complication
  • treatment
A
  • pathogenesis: APC gene mutation (adenematous polypolsis coli)
  • presentation:
    • systemic: premalignant colon polyps by age 20 -> GI bleeding + abdominal pain
    • skin: cysts:
      • osteomas - in mandible
      • odontogenic cysts
      • epidermoid / desmoid cysts
    • other: CHRPE (congenital hypertrophy fo the retinal pigment)
  • complications: 100% risk of GI adenocarcinoma
  • treatment: prophylactic total colectomy
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23
Q

what is the treatment for gardner syndrome?

A

prophylactic total colectomy

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24
Q

what two systemic-cutaneous diseases increase risk of GI malignancies?

  • what mutations do they result from?
  • what are their skin manifestations?
  • what specific risks do they each pose?
A
  • peutz-jegher:
    • STK-11 mutation
    • skin: pigemented macules on oral mucosa
    • risk: inc risk of GI and NON-GI malignancies
  • gardner syndrome:
    • APC gene mutation
    • skin: cysts - osteomas (mandible, maxilla), odontogenic cysts, epidermoid cysts
    • risk: 100% chance of developing GI adenocarcinoma without total prophylactic colectomy
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25
pyoderma gangrenosum * pathogenesis * presentation * diagnosis * treatment
* pathogenesis: **m/c IBD** (or other underlying systemic inflammatory dz) * presentation: * systemic: IBD symptoms skin: **_painful_ ulcer that is surrounded by a irregular, undermined, violaceous border** * diagnosis: diagnosis of exclusion * treatment: steroids good wound care
26
dermatitis herpetiformis * pathogenesis * presentation * diagnosis * treatment * complications
* pathogenesis: formation of antibodies against transglutaminase (TTG), which found in the skin & GI tract * presentation: **_extremely pruritic_ papulovesicular eruptions on** * _buttocks_ * _extrensor surfaces_ * diganosis: **anti-TTG** * treatment**: strict gluten free diet (no wheat, rye, barley)** * complications: **increased risk of MALT lymphoma**
27
porphyria cutanea tarda (PCT) * pathogenesis * presentation * treatment
* pathogenesis: **defect in _heme synthesis_ d/t** **uroporphyrinogen decarboxylase** * presentation: tense bullae + erosions + hyperpigmentation + scarring on sun exposed skin * treatment: photoprotection + hydroxychloroquine
28
what are the _skin manifestations_ of hyper and hypothyroidism? how is each treated?
* hyperthyroidism (low TSH, elevated T3 & T4) * manifestations: * **pretibial myxedema:** i**ndurated red-brown pretibial plaques** * **smooth / moist skin (even hyperhyrdosis)** d/t _hypermetabolic state_ * **vilitgo** * tx: anti-thryoid drugs, radioactive iodine * hypothryoidism * manifestations: * **loss of lateral eyebrows (madarosis)** * **course / dull / brittle skin** d/t _hypometabolic state_ * levothyroxine
29
acanthosis nigricans * pathogenesis * presentation * treatment
* pathogenesis: associated with insulin resistance (DM) * presentation**: thickening of the neck / axilla that is** * **symmetrical** * **_velvety_** * **_grayish-brown_** * treatment: topical retinoids +/- ammonium lactate
30
necrobiosis lipoidica * pathogenesis * presentation
* pathogenesis: cell death due to **microangiopathy of diabetes** * presentation: **patches that are** * **yellow orange -\> red brown** * **associated with telangectasias** * **over the _pretibial areas_**
31
what major cutaneous presentations are associated with diabetes?
* aconthosis nigricans * necrobiosis lipoidica * recurrent candidiasis
32
neurofibromatosis type I * pathogenesis * presentation * complications
* pathogenesis: mutation of NF1 gene * presentation: * **cafe-au lait macules and / or neurofibromas** * _crowe's sign:_ freckling in the axillary / inguinal lesion * _lisch nodules_: melanocytic lesions in the iris * complications: * Wilm's tumor * scoliosis * high BP
33
ehlers danlos syndrome (EDS) * pathogenesis * presentation * complications
* pathogenesis: mutation in **type 5 collagen** leads to abnormal collagen structure within skin, joints & vasculature * presentation: * **hyperextensible skin** * **hypermobile joints** * **tendency to bleed**
34
marfan syndrome * pathogenesis * presentation * complications
* pathogenesis: FBN1 (fibrillin) mutation * presentation: * **tall stature** * **long limbs & digits (arachodatylyl)** * **long ears** * etopia lentis - lens subluxation * complications: **cardiac complications - esp aortic aneurysms / mitral valve prolapse**
35
discoid rash (discoid LE) indurated plaques with follicular plugging localized to _neck / face / ears / scalp_ that heal with _scarring_
36
discoid rash (discoid LE) indurated plaques with follicular plugging localized to _neck / face / ears / scalp_ that heal with _scarring_
37
discoid rash (discloid LE) indurated plaques with follicular plugging localized to _neck / face / ears / scalp_ that heal with _scarring_
38
discoid rash (discloid LE) indurated plaques with follicular plugging localized to _neck / face / ears / scalp_ that heal with _scarring_
39
SLCE annular, scaly plaques on sun-exposed regions
40
SCLE annular, scaly plaques on sun-exposed regions
41
nenoatal lupus erythematosis annular lesions w/ central clearing (like SCLE) - but more facial involvement
42
ACLE rash on dorsal hands that _spares_ the knuckles (vs dermatomyotitis - glotton's)
43
ACLE / SLE malar rash that involves nasal ridges + bilateral malar cheeks & spares melolabial folds
44
localized scleroderma - linear scleroderma linear expanding erythema on lower extremities or face (right pic = "en coupe de sabre")
46
localized scleroderma - morphea scleroderma expanding erythema on trunk + proximal extremities
47
diffuse scleroderma
48
polydactyl limited sclerosis / scleroderma (CREST syndrome)
49
dermatomyositis (DM) heliotrope sign
50
dermatomyositis (DM) heliotrope sign
51
dermatomyositis (DM) grotton's papules: lichenoid papules that _include knuckles_ (vs ACLE)
52
peutz-jeghers syndrome (STK11) pigmented mucutaneous macules on oral mucosa
53
gardner syndrome (APC mutation) osteomas on mandible & maxilla
54
gardner's sydnrome (APC mutation) odontogenic cysts
55
gardner syndrome (APC gene mutaiton) CHRPE: congenital hypertorphy of retinal pigment
56
pyoderma gangrenosum (IBD) ulcers with a _irregular, undermined violaceous border_
57
dermatitis herpetiformis (anti-TTG IgA antibodies) extremily pruritic papulovesicular eruptions on EXTENSOR SURFACES + BUTTOCKS
58
porphyria cutanea tarda (uruoporphyrinogen dexcarboxylase) tenase bullae + erosins + hyperpigmentation on sun exposed skin
59
hypothryoidism loss of lateral eyebrow
60
necrobiosis lipoidca (diabetes) atrophic yellow-orange -\> red-brown patches w/ tenalgetctasia _over pretibial areas_
61
acanthosis nigricans (diabetes) symmetrical, hyperpigemented _velvety, gray-ish-grown thickening_ of the neck + axilla + groin
62
recurrent candiasis (associated with DM) beefy-red demarcated plaques surrounded by satellite lesions
63
ndurofibromatosis type I (NF1 gene mutation) plexiform neurofibroma
64
neurofirbromatosis type I (NF 1 gene mutation) lisch nodules
65
ehler's danos syndrome (mutated type 5 collagen ) hypermobile joints
66
ehler's danlos syndrome (type 5 collagen mutation) hypermobile joints
67
marfan syndrome (FBN1 mutation) arachnodactylyl
68
marfan's syndrome (FBN1 mutation) tall stature
69
pretibial myxedema hyperthyroidism
79
neurofibrmotosis type I neurofibromas

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