SM_268a: Connective Tissue Disease in Skin Flashcards Preview

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Flashcards in SM_268a: Connective Tissue Disease in Skin Deck (39)
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Systemic lupus erythematosus cutaneous presentations include ____, ____, and ____

Systemic lupus erythematosus cutaneous presentations include chronic cutaneous lupus erythematosus (discoid lupus erythematosus), subacute cutaneous lupus erythematosus, and acute cutaneous lupus erythematosus


Describe discoid lupus erythematosus

Discoid lupus erythematosus

  • Head and upper body (sun exposed areas)
  • Scaly pink brown plaques
  • Heal with atrophic scars and dyspigmentation
  • Annular appearance (central clearance)
  • Scalp lesions result in alopecia
  • More common in African Americans and women
  • Skin manifestation of chronic cutaneous lupus erythematosus


Describe subacute cutaneous lupus erythematosus

Subacute cutaneous lupus erythematosus

  • Photosensitive 
  • Mucocutaneous locations: lateral face, upper trunk, extensor upper extremities (sun exposed areas but can generalize)
  • Scaling annular pink papules and plaques (polycyclic)
  • No scarring
  • Anti-SSA (Ro)

(do detailed drug history)


Acute cutaneous lupus erythematosus involves a classic _____

Acute cutaneous lupus erythematosus involves a classic malar rash


(butterfly rash, cheeks extending onto nose, spares nasolabial folds)


Describe acute cutaneous lupus erythematosus

Acute cutaneous lupus erythematosus

  • Classic malar rash: butterfly rash, cheeks extending onto nose, spares nasolabial folds
  • Appearance variable: mild erythema, significant edema, telangiectasia, erosions
  • Transient eruption
  • Triggered by sun exposure
  • Oral ulcerations
  • Often associated with antibodies: anti-dsDNA
  • Must evaluate for systemic disease


People with acute cutaneous lupus erythematosus must be evaluated for ___

People with acute cutaneous lupus erythematosus must be evaluated for systemic disease


Describe neonatal lupus erythematosus

Neonatal lupus erythematosus

  • Infants whose mothers have anti-Ro antibodies
  • Similar to subacute lupus erythematosus: annular pink scaly plaques, periorbital location
  • Sun exposure not needed
  • Dyspigmentation persists


Describe cutaneous manifestations of dermatomyositis

Cutaneous manifestations of dermatomyositis

  • Heliotrope eroption
  • Gottron's papules
  •  Nail changes: mechanic's hands
  • Poikiloderma
  • Shawl sign
  • Calcinosis cutis
  • Anti-MDA-5 disease


Heliotrope rash occurs in ____ and involves is ____, involves ____, and can be ____

Heliotrope rash occurs in dermatomyositis and is violaceous, involves periorbital edema, and can be subtle


(patients often treated for atopic dermatitis or allergic contact dermatitis if subtle)


Describe Gottron's papules

Gottron's papules

  • Occur in dermatomyositis
  • Extensor joints: MCP, IP, elbows
  • Pink to violaceous
  • Papules and plaques
  • Often scaling
  • Ulceration in MDA-5


Nail changes in dermatomyositis include ____, ____, and ____

Nail changes in dermatomyositis include mechanic's hands, dilated capillary loops, and capillary loop dropout


Mechanic's hands occur in ____ and involve ____, ____, and ____

Mechanic's hands occur in dermatomyositis and involve ragged cuticules (proximal nail folds), cuticular overgrowth, and hyperkeratosis / fissuring of the lateral fingers


Poikiloderma occurs in ____ and involves ____, ____, ____, and ____

Poikiloderma occurs in dermatomyositis and involves hyperpigmentation, hypopigmentation, telangiectasia, and epidermal atrophy


Shawl sign occurs in ____ and involves ____, can be ____, and is ____

Shawl sign occurs in dermatomyositis, involves anti Mi2 antibodies, can be pruritic, and is photodistributed poikiloderma (V of neck, upper back)


Dermatomyositis involves ____ in contrast to lupus but cannot tell difference between the two on ____

Dermatomyositis involves nasolabial folds in contrast to lupus but cannot tell difference between the two on skin biopsy


Describe calcinosis cutis

Calcinosis cutis

  • Juvenile disease
  • Adults with NXP-2 antibodies
  • Advanced disease
  • Cutaneous, deep fascia, intramuscular connective tissue
  • Hard nodules favoring sites of trauma
  • May drain a chalky material
  • Painful
  • Treatment is difficult


Describe anti-MDA-5 disease

Anti-MDA-5 disease

  • Clinical clues to this serologic phenotype: punched out ulcers over Gottron's papules and nail folds, prominent alopecia, more oral ulcers, tender palmar ulcers (classic tender areas on flexural joints)
  • High mortality due to interstitial lung disease: pulmonary function tests w/ diffusion capacities at presentation and every 3 months


Dermatomyositis internally manifests as ____

Dermatomyositis internally manifests as malignancy


  • Associated with anti-TIF1-gamma antibodies
  • Associated with adult cases: not juvenile
  • May precede malignancy


Describe dermatomyositis


  • Mild facial erythema that includes nasolabial folds (in contrast to lupus)
  • Skin almost always precedes muscle symptoms
  • Skin biopsy cannot distinguish between dermatomyositis and lupus (need to look at patient)
  • Risk of malignancy highest in first year of presentation and remains elevated for three years


Describe relapsing polychondritis

Relapsing polychondritis

  • Cartilaginous structures
  • Erythema, edema, pain of cartilaginous ear but lobe is spared
  • Nasal chondritis in 70% 
  • Respiratory tract
  • Arthritis
  • Ocular inflammation
  • Death is possible
  • Evaluate for systemic disease: other autoimmune diseases, myelodysplasitc syndrome


Earlobe is ___ in relapsing polychondritis

Earlobe is spared in relapsing polychondritis


Describe systemic sclerosis (scleroderma)

Systemic sclerosis (scleroderma)

  • Skin, blood vessels, internal organs
  • Two major clinical subtypes: limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis


Describe diffuse cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

  • Anti Scl70 antibodies
  • Raynaud's phenomenon
  • Generalized fibrotic changes
  • Starts in hands
  • Spreads to forearms, arms, trunk, face, lower extremities


Describe limited cutaneous systemic sclerosis

Limited cutaneous systemic sclerosis

  • Anti-centromere antibodies
  • Fibrotic changes limited to fingers, hands, and face
  • CREST syndrome: calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia


CREST syndrome is also known as ____ and includes ____, ____, ____, ____, and ____

CREST syndrome is also known as limited cutaneous systemic sclerosis and includes calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia


Describe early stages of systemic sclerosis

Early stages of systemic sclerosis

  • Hands and fingers
  • Edema or "puffiness" of hands: change in ring size
  • Skin hardens
  • Taut and shiny skin


Describe Raynaud's phenomenon

Raynaud's phenomenon

  • Occurs in systemic sclerosis
  • Red, white and blue: numbness when white, ischemia when blue, re-warming when red
  • May be painful
  • Severe cases may result in ulcerations


Describe sclerodactyly


  • Fibrosis of the skin on the hands
  • Taut and tight skin
  • Results in contractures
  • Loss of digital pulp
  • Tapering of the digits
  • Nail changes


Describe CREST


  • Occurs in limited cutaneous systemic sclerosis: anti-centromere antibody
  • Calcinosis cutis
  • Raynaud's
  • Esophageal dysmotility
  • Telangiectasia


Describe morphea


  • Distinct from systemic sclerosis
  • No Raynaud's
  • No fibrosis of internal organs
  • No edema of the hands
  • Erythematous to violaceous expanding indurated plaques
  • Centrally more sclerotic
  • Can lead to loss of range of motion
  • Can be disfiguring
  • En coup de sabre / Parry-Romberg (progressive hemifacial atrophy)

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