SM_268a: Connective Tissue Disease in Skin Flashcards
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
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
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
CREST
- Occurs in limited cutaneous systemic sclerosis: anti-centromere antibody
- Calcinosis cutis
- Raynaud’s
- Esophageal dysmotility
- Telangiectasia
Describe morphea
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)
Describe anti-phospholipid antibody syndrome
Anti-phospholipid antibody syndrome
- Livedo reticularis
- Livedo racemosa
- Skin necrosis and ulceration
Livedo reticularis occurs in ____ and is characterized by ____, ____, ____, and ____
Livedo reticularis occurs in anti-phospholipid antibody syndrome and is characterized by pink to reddish-blue color, netlike appearance, triggering by cold, and resolution with warmth
Livedo racemosa occurs in ____ and involves ____, ____, ____, ____, and ____
Livedo racemosa occurs in anti-phospholipid antibody syndrome and involves, fixed livedo vascularis, irregular networks, broken lace patterns, no variation with temperature changes, and association with systemic disease
(requires treatment)
Vasculitis is caused by ____ and results in ____ and ____
Vasculitis is caused by immune complex deposition and/or antibody deposition and results in inflammatory cascade and destruction of vessel walls
Describe leukocytoclasic vasculitis
Leukocytoclasic vasculitis
- Etiology: iiopathic, drug, infection, connective tissue, inflammatory diseases, malignancy, cryoglobulins, Henoch-Schonlein purpura (IgA vasculopathy)
- Crops of palpable purpuric papules
- May be necrotic
- May have constitutional symptoms
- Suspicion for systemic involvement
Henoch-Schonlein pupura is caused by a ____ and involves ____ deposits in vessel walls
Henoch-Schonlein pupura is caused by a preceding URi and involves IgA immune deposits in vessel walls
(acute onset: palpable purpura, arthralgias, colicky abdominal pain; renal vasculitis)
Granulomatosis with polyangiitis is manifests as ____ and involves ____, ____, and ____
Granulomatosis with polyangiitis manifests as +c-ANCA with anti-PR3 and involves granulomatous inflammation of upper and lower respiratory tracts, systemic mecrotizing small vessel vasculitis, and glomerulonephritis
Cutaneous features of granulomatosis with polynagiitis include ____, ____, ____, ____, and ____
Cutaneous features of granulomatosis with polynagiitis include palpable pupura, oral ulcers, subctuaneous nodules, papulonecrotic lesions on extensor surfaces, and strawberry gums
Eosinophilic granulomatosis manifests as ____ and has ____ phases
Eosinophilic granulomatosis manifests as +p-ANCA to myeloperoxidase and has three phases
- First: allergic rhinitis, nasal polyps, asthma
- Second: peripheral eosinophilia, respiratory tract infections, and GI symptoms
- Third: systemic vasculitis with granulomatous inflammation, palpable purpura
