? SLE
? Dermatomyositis
? Systemic sclerosis (scleroderma)
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? Rheumatoid arthritis? Mixed CT disease
? others
? Spectrum ranging from benign cutaneous variants to severe, often
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fatal, multisystem diseases? Inflammation of the connective tissue - resulting in changes in skin,
joints, vasculature and other Organs
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? Antibodies formed against cell components - Serological markers
Dermatomyositis
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? Dermatomyositis (DM) - systemic autoimmune disease characterizedby inflammation and damage to the skin and muscle
? Interstitial lung disease (ILD) - 20%
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? In adults - DM - heralds the diagnosis of a coexisting internalmalignancy in 10% to 20% of cases
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? Characteristic autoantibodies?Antisynthetase
?Anti?Mi-2
?Anti?transcriptional intermediary factor [TIF1]-gamma
?Anti?melanoma differentiation?associated gene 5 [MDA5]
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?Anti?nuclear matrix protein 2 [NXP2]?Anti?small ubiquitin-like modifier activating enzyme [SAE]
? May be useful in identifying clinical subsets
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Violaceous patches and plaques? Characteristic cutaneous feature
? violaceous patches and plaques, varying from a bright pink to a deep
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violet colorThe heliotrope sign
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? Exemplies the pink to purple violet hue of the eruption? color of the flower petals after which the sign named
? The eyelid eruption can be associated with periorbital edema
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The V-neck sign? Confluent violaceous erythema on the sun-exposed areas of the
lower anterior neck and anterior chest
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? The skin changes - often distributed to typical regions on the body
? Trunk involvement is often seen on the posterior neck, upper back,
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and shoulders, known as the shawl signGottron papules
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? The violaceous to pink papules over the IP and MCP jointsGottron sign
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? Symmetric macular violaceous erythema over the IP joints, olecranonprocesses, patellae, and medial malleoli
? May be atrophy and poikiloderma in classic areas of Gottron sign
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The Holster sign
? The violaceous erythema and poikiloderma on the lateral hips and
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lateral thighs
? Often patterned as folliculocentric macules or subtle papules
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"Mechanic's" hands
? Hyperkeratosis and fissuring along the medial thumb and lateral
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second and third digits? A cutaneous clue to the possible presence of ILD
Systemic sclerosis (Scleroderma)
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? Multisystemic autoimmune disease - by vasculopathy, inflammation,and fibrosis of the skin and many other organs due to extensive
activation of fibroblasts
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? Raynaud phenomenon, circulating autoantibodies, and skin sclerosis
are almost always present
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? Important for the early diagnosis? An overlap syndrome, including mixed connective tissue disease ?
characterized by additional clinical features of other RHDs
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? The highest case-specific mortality of any of the autoimmune
rheumatic diseases
? SSc usually starts with a Raynaud phenomenon - precede the disease
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for many years
? The clinical manifestations - diverse with severe fibrosis of the skin
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and all additional cutaneous manifestations? E.g. hardening of the skin, development of contractures, digital
ulcerations and calcifications
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? Multiple patterns of internal organ involvement
Raynaud's phenomenon
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? Earliest symptom to appear in more than 90% of SSc patients? Characterized by painful pallor/ischemia of single or several digits
followed by reactive hyperemia after reheating at the end of a RP
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attack f/b in some cases cyanosis (triphasic RP)? Worse in winter/by emotional stress
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? Eventually leads to:
?Finger tip ulcers
?Loss of finger pulp
?Gangrene
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? An increasing induration and skin thickening (sclerodactyly)
? Depending on the localization of skin thickening restricted mobility of
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joints (dermatogenous contractures)? Facial appearance - characterized by a radial furrowing around the
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mouth, no expression, a stiff and mask-like facial appearance, and
sclerosis of the frenulum
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? Besides cosmetic/aesthetic problems, this causes considerabledifficulties regarding eating and oral hygiene
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Rheumatoid Arthritis
? Affects roughly 1% of the world population
? Skin findings quite varied, including papules, plaques, and nodules
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with multiple histologic types, vasculitis/Bywaters lesions, pyoderma
gangrenosum/Felty ulcers
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Rheumatoid Nodules and Nodulosis? The usual location is over pressure points such as the olecranon, the
extensor surface of the forearms, and the Achilles tendon
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? Benign, they can lead to complications, including ulceration, infection,
joint effusion (rheumatoid chyliform bursitis), and fistulas (fistulous
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rheumatism)? Accelerated nodulosis- Low-dose methotrexate, often used for the
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treatment of RA, may precipitate erythema in and enlargement ofpreexisting rheumatoid nodules
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Rheumatoid neutrophilic dermatosis? Rare cutaneous manifestation
? Lesions are usually chronic, erythematous, and urticaria-like plaques
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and papules; sharply marginated