Connective Tissue & Bullous Disorders of the Skin - MD Flashcards

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1
Q

What are characteristics of Lupus Erythematosus?

A
  • Chronic, multisystem Dz
  • Autoimmune
  • Genetic predisposition
  • Women > men
  • MC in Af. Americans & Hispanics
  • Onset 16-55 y.o.
  • Triggers include UV light, EBV, smoking
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2
Q

What are S/S of SLE?

A
  • Fatigue (MC)
  • Myalgia/weakness
  • Low grade fever
  • Photosensitivity, alopecia, oral/nasal ulcerations
  • Raynauds
  • Weight loss/gain
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3
Q

What are the 3 different types of SLE mucocutaneous rashes?

A

1) Malar rash: occurs when SLE is active, characterized by edema/photosensitivity
2) Discoid Rash (looks like psoriasis): erythematous & chronic
3) Subacute Cutaneous: Erythematous papules/plaques with slight scaling mimicking psoriasis

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4
Q

What are characteristics of acute cutaneous LE?

A
  • occurs on sun-exposed areas
  • Associated with systemic Dz
  • Nonpuritic, erythematous rash on exposed parts of the body
  • Classic “butterfly” or “malar” rash on face
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5
Q

What are characteristics of DLE (Discoid Lupus)?

A

-like acute cutaneous LE but lower incidence of systemic disease. and can lead to severe scarring

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6
Q

What are the characteristics of subacute cutaneous LE?

A
  • Falls b/w chronic destructive DLE & erythema of acute cutaneous LE
  • Two forms → papulosquamous & annular
  • lesions heal without scarring
  • associated with arthralgia/myalgia
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7
Q

What positive lab result is the cardinal feature in SLE diagnosis since it occurs in over 90% of cases?

A

Positive for the presence of ANA

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8
Q

What are aspects of a treatment plan for a patient with LE?

A
  • modifying CV risk factors (leading cause of morbidity and mortality)
  • Avoid sunlight
  • Calcium + Vit D
  • Hydroxychloroquine (Plaquenil) → protects against organ damage
  • Methotrexate for persistent arthritis
  • Immunosuppresive Tx
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9
Q

What is the difference between polymyositis and dermatomyositis?

A

Dermatomyositis is inflammatory muscle and skin Dz

Polymyositis is same but without skin involvement

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10
Q

What are characteristics of Dermatomyositis?

A

1) Heliotrope erythema of the eyelids
- pathognomonic
- Periorbital edema and violet discoloration
2) Gottron’s papules:
- pathognomonic
- smooth, violaceous-to-red, flat topped papules, occuring over the knuckles, along with sides of fingers.

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11
Q

What findings would assist in making a Dermatomyositis Dx?

A
  • Symmetrical weakness of limb girdle muscles
  • Elevated muscle enzyme levels
  • Myopathic pattern on EMG
  • Muscle Biopsy evidence on inflammation
  • Skin rash
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12
Q

What is the Tx for dermatomyositis?

A
  • Steroid taper (first line)
  • Azathioprine/ Methotrexate (second line)
  • Exercise
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13
Q

What are characteristics of limited scleroderma (CREST Syndrome)?

A
C – Calcinosis 
R – Raynaud's phenomenon 
E – Esophageal dysfunction 
S – Sclerodactyly 
T – Telangiectasia
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14
Q

What are the most common manifestations of Systemic Scleroderma?

A

Most common symptoms:

  • Raynaud’s,
  • diffuse swollen hands,
  • arthralgias/myalgias,
  • fatigue
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15
Q

What is the Tx plan for a patient with scleroderma?

A
  • Treat symptoms

- No disease modifying agents available

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16
Q

What are the characteristics of Pemphigus Vulgaris?

A
  • Group of rare blistering Dz of skin and mucous membranes
  • Organ specific-autoimmune Dz
  • Start in oral mucosa but spreads over months to form bullous eruptions
17
Q

What is the Tx of Pemphigus Vulgaris?

A

oral steroids & tapering

18
Q

What are the characteristics of Dermatitis Herpetiformis?

A

Grouped, pruritic, erythematous papulovesicles
Extensor surface skin involvement
Elbows, knees, buttocks, lower back, scalp
Virtually ALL patients have gluten-sensitivity enteropathy on bowel biopsy (but GI sx in 25%)
Gluten-free diet successful > 90% of cases
Associated with thyroid disorders

19
Q

What is the Tx for Dermatitis Herpetiformis?

A

Dapsone 25 mg PO daily until symptoms improve

Gluten-free diet