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MyDermatology-Bolognia > Ch. 43 Systemic sclerosis > Flashcards

Ch. 43 Systemic sclerosis Flashcards

1
Q

What is the difference between limited cutaneous SS and diffuse cutaneous SS

A

Limited-up to elbows and knees, face (most often just hands and feet)

Diffuse-trunk, upper arms and legs

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2
Q

Name the 7 main conditions on ddx for sclerodermoid conditions

A
  1. Scleroderma
  2. Generalized morphea
  3. Scleredema
  4. Scleromyxedema
  5. Eosinophilic fasciitis
  6. Chronic GVHD
  7. Nephrogenic systemic fibrosis

Drugs:
-Bleomycin exposure

Paraneoplastic: 
-POEMS
-carcinoid
-amyloidosis
-Paraneoplastic scleroderma-like syndrome
Immunologic:
-Fibroblastic rheumatism

Metabolic:

  • PCT-porphyria
  • Diabetic cheiroroarthropathy

Neoplastic:
-Carcinoma en cuirasse

Neurologic:
-reflex sympathetic dystrophy, spinal cord injury

Toxin mediated:

  • Toxic oil syndrome
  • eosinophilic myalgia (L tryptophan)
  • silicosis

Drug induced: bleomycin, taxanes, vinyl chloride, chlorinated hydrocarbons

other: lipodermatosclerosis

Genetics: Stiff skin syndrome ad many others…

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3
Q

Incidence and prevalence systemic sclerosis

A

20 per 1 million

275 per 1 million

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4
Q

Average age onset scleroderma

A

35-50

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5
Q

Name 5 RF for worse prognosis systemic sclerosis

A 
African american
Male sex
Older age
ILD/internal organ involvment
More diffuse disease (trunk involvement)
Elevated ESR
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6
Q

What are 3 pathogenic mechanisms causing systemic sclerosis

A
  1. Vascular dysfunction with increased VEGF
  2. Immune activation with autoantibody production
  3. Extra-cellular matrix dysregulation: sclerosis characterized by deposition of collagen and other extracellular matrix proteins
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7
Q

What are the diagnostic criteria for systemic sclerosis

A

Skin thickening proximal to MCPS = automatic dx
Otherwise need 9 points

Skin thickening distal to MCPS or skin puffiness

Finger tip lesions-fingertip scar or ulcers

Nailfold capillary changes

Telengiectasias

Raynauds

PAH or ILD

Autoantibodies

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8
Q

What are the 8 diagnostic criteria for systemic sclerosis

A

Skin thickening proximal to MCPS = automatic dx
Otherwise need 9 points

Skin thickening distal to MCPS or skin puffiness

Finger tip lesions-fingertip scar or ulcers

Nailfold capillary changes

Telengiectasias

Raynauds

PAH or ILD

Autoantibodies

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9
Q

What 3 antibodies are most specific for scleroderma and what are their clinical correlates

A

Anti-centromere with CREST/limited and PAH

Anti SCL70 (Topoisomerase I) with diffuse SS and ILD

RNA polymerase III with rapidly progressive diffuse skin disease and renal involvement

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10
Q

What are 5 major differences (beyond skin) between limited and diffuse sclerosis

A

1) Lungs
- PAH more in limited, ILD more in diffuse

2) Raynauds
- longstanding hx raynauds in limited vs. acute onset in systemic

3) Skin progression
- slowly progressive skin disease in limited, quickly in diffuse (peak in 1-1.5 yrs

4) onset internal organ involvement
- 10-15 yrs limited, most in first 5 yrs in diffuse

5) more kidney/SRC in diffuse

Prognosis worse in diffuse

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11
Q

What are the 4 major internal organs affected in systemic sclerosis

A

Lungs: PAH, ILD

GI: GERD, esophageal dysmotility

Renal: renal crisis, htn

Cardiac: fibrosis/restrictive pericarditis, CHF from PAH

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12
Q

Leading cause death scleroderma

A

lung disease

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13
Q

What are 5 features that suggest secondary raynauds

A 
Later onset > age 25 
Digital ulcerations
Fingertip scars 
Abnormal nail fold capillaroscopy
Sclerodacltyl
ANA+
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14
Q

Name 12 physical findings of scleroderma

A
  1. Puffy/edematous fingers–>skin thickening and sclerodactlyl
  2. Raynauds
  3. Nailfold changes –> alternating dilation with drop out
  4. Salt and pepper skin (leukoderma with hypopigemntation with preservation follicular pigment)
  5. Calcinosis cutis-often over joints, extremities
  6. Telengiectasias-face, lips, palms
  7. Fingertip pitted scars
  8. Microstomia and peri-oral wrinkles
  9. Beaked nose
  10. Tendon friction rubs
  11. Flexion contractures
  12. Dry skin, loss sweating,
  13. Ulcers-f fingertip and over joints
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15
Q

Name 6 pathology findings scleroderma

A
  1. compact or hyalinized collagen
  2. excessive deposition of collagen
  3. atrophic eccrine and pilosebaceous glands,
  4. loss of subcutaneous fat
  5. sparse lymphocytic infiltrate in the dermis and subcutis
  6. Trapped adnexal structures
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16
Q

Name 8 treatments for raynauds

A
  1. avoid cold, warming packs, stop smoking= 1st line
  2. calcium channel blockers=2nd line
  3. endothelia receptor antagonists- bosentan
  4. PDE-5 inhibitors-sildenafil
  5. ARBs-losartan
  6. alpha adrenergic blockers-prazosin
  7. SSRI-fluoxetine-4th line
  8. botox
17
Q

Name 5 treatments for cutaneous ulcers from raynauds

A

Avoid aggressive debridement, moist adherent dressings

All therapies listed for raynauds

ASA or clopidogrel

Endothelial receptor antagonists- bosentan

IV prostanoid (e.g. epoprostenol)

High dose statins

18
Q

Name 8 treatments for calcinosis cutis

A
  1. excision
  2. shock wave litho
  3. sodium thiosulfate-IV or injection or topical
  4. warfarin
  5. bisphosphonates
  6. CCBs-diltiazem
  7. aluminum hydroxide
  8. probenecid
19
Q

Name 4 treatments cutaneous sclerosis

A

Often not treated unless internal involvement:

  1. Topical steroids
  2. Phototherapy
  3. Methotrexate-if no ILD
  4. MMF -esp if ILD
20
Q

Treatment scleroderma renal crisis

A

ACEi

21
Q

Name 2 major treatment internal organ involvement scleroderma

A

MMF or cyclo

22
Q

What are the clinical features of eosinophilic fascitis

A
  1. Hx strenuous physical activity in 30%
  2. Pain and edema that progresses to fibrosis and psuedo-cellulite appearance, rippling
  3. Distribution symmetric extremities and usually spares the hands, feet and face
  4. Groove sign
23
Q

What is the groove sign

A

“groove sign” refers to linear depressions where veins appear to be sunken within the indurated skin.

24
Q

Name 3 laboratory findings in eosinophilic fascitis

A
  1. Elevated ESR
  2. Peripheral eosinophilia
  3. hypergammaglobulinemia
25
Q

What are 4 conditions that have been associated with eosinophilic fasciitis

A

Pancytopenia
Anemia
Thrombocytopenia
Myeloproliferative d/o –> some advocate for marrow bx

26
Q

What does pathology show for EF?

A

Thickening deep fascia
Lymphocytes and plasma cell infiltrates
Sometimes dermal fibrosis

27
Q

NAme 2 tests you’d order for workup EF?

A

Fascial biopsy

MRI

28
Q

What is first line treatment EF

A

prednisone 1-2 mg/kg daily tapered over 6-24 months

29
Q

Name 6 treatments other than prednisone for EF

A
  1. hydroxychloroquine
  2. cyclosporine
  3. dapsone
  4. methotrexate
  5. PUVA
  6. infliximab
    * may be used alone or in combination with prednisone.

UVA1, alone or in combination with an oral retinoid, can also be considered

30
Q

What 2 risk factors lead to nephrogenic systemic fibrosis

A
  • kidney injury (AKI or ESRD)

- gadolinium

31
Q

How does NSF present on the skin

A

Thick indurated patterned plaques on extremities or trunk,

can have advancing “ameboic” border

Erythematous or hyperpigemented

32
Q

What internal organ involvement can there be in NSF

A

yellow scleral plaques

systemic fibrosis affecting–> the heart, lungs and skeletal muscle.

33
Q

Treatment NSF

A

anecdotal reports:
-imatinib, rapamycin, UVA1 phototherapy, extracorporeal photochemotherapy, photodynamic therapy, plasmapheresis,
high-dose IVIg, discontinuation of erythropoietin.

-case reports with;
improvement renal function with transplant

34
Q

What is stiff skin syndrome ?

A

Genetic condition with heterozygosity for a mutation in the gene that encodes fibrillin-1 ( FBN1 ).

The presence of giant “amianthoid-like” collagen fibrils in affected fascia had been postulated to reflect a disturbance in the organization of collagen and glycosaminoglycans within the extracellular matrix.

35
Q

Describe the clinical features of stiff skin syndrome

A

Rock hard thickening skin and subcutaneous tissues over buttocks and thighs with sparing inguinal folds, sometimes hypertrichsosi

Spares hands and feet

Postures hip and knee flexion and excess lumbar lordosis

36
Q

internal organ manifestations stiff skin?

A

None usually

37
Q

Name 5 drugs/ that can cause sclerodermoid changes

A
  1. Bad L-tryptophan –> eosinophilic myalgia syndrome
  2. Chemo: esp. taxanes and bleomycin, can cause pull fibrosis
  3. Bad rapeseed oil–> toxic oil syndrome
  4. vinyl chloride
  5. organic solvents
  6. epoxy resins
38
Q

How does chemical induced sclerosis present

A
  • insidious acrosclerosis and Raynaud phenomenon (especially with vinyl chloride exposure), but morpheaform plaques and fibrotic nodules can also develop.
  • Acral osteolysis, hepatic toxicity, and pulmonary involvemen fatigue, weight loss, arthralgias, and myalgias.
  • Autoantibodies are absent, and cessation or reversal of the disease process follows removal of the exposure.
39
Q

What chemical exposure has been associated with AI-CTD and scleroderma specifically

A

Silica and silicosis

MyDermatology-Bolognia (31 decks)

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