Skin & Vascular Disorders Flashcards
Symptoms of polymyalgia rheumatica
Pain and stiffness in proximal muscles (eg, shoulders, hips), often with fever, malaise, weight loss
Does not cause muscular weakness
Epidemiology of polymyalgia rheumatica + association
More common in females > 50 years old; associated with giant cell (temporal) arteritis
Lab findings in polymyalgia rheumatica
Increased ESR & CRP, normal CK
Tx of polymyalgia rheumatica
Rapid response to low-dose glucocorticoids
Epidemiology of Fibromyalgia
Most common in females 20–50 years old
Define fibromyalgia
Chronic, widespread musculoskeletal pain associated with “tender points,” stiffness, paresthesias, poor sleep, fatigue, cognitive disturbance (“fibro fog”)
Lab findings of fibromyalgia
Normal inflammatory markers like ESR
Tx of fibromyalgia
Regular exercise, antidepressants (TCAs, SNRIs), neuropathic pain agents (eg, gabapentin)
Lab findings in non/specific polymyositis/dermatomyositis
Nonspecific: ⊕ ANA, increased CK
Specific: ⊕ anti-Jo-1 (histidyl-tRNA synthetase), ⊕ anti-SRP (signal recognition particle), ⊕ anti-Mi-2 (helicase)
Define polymyositis, involves what
Progressive symmetric proximal muscle weakness, characterized by endomysial inflammation with CD8+ T cells
Most often involves shoulders
Findings in dermatomyositis
Clinically similar to polymyositis, but also involves Gottron papules, photodistributed facial erythema (eg, heliotrope edema of the eyelids), “shawl and face” rash, mechanic’s hands (thickening, cracking, irregular “dirty” appearing marks due to hyperkeratosis of digital skin)
Dermatomyositis increases the risk of what
Occult malignancy
Inflammation in dermatomyositis
Perimysial inflammation and atrophy with CD4+ T cells
Define myositis ossificans
Heterotopic ossification involving skeletal muscle (eg, quadriceps)
Associated with blunt muscle trauma
Presentation of myositis ossificans
Painful soft tissue mass
Imaging and histology for myositis ossificans
Imaging: eggshell calcification.
Histology: metaplastic bone surrounding area of fibroblastic proliferation
Benign, but may be mistaken for sarcoma.
Name the large vessel vasculitis
Giant cell (temporal) arteritis & Takayasu arteritis
Epidemiology/presentation of giant cell arteritis
Females > 50 years old.
Unilateral headache, possible temporal artery tenderness, jaw claudication
May lead to irreversible blindness due to anterior ischemic optic neuropathy
Associated with polymyalgia rheumatica
Lab findings of giant cell arteritis
Most commonly affects branches of carotid artery
Focal granulomatous inflammation
Increase in ESR
IL-6 levels correlate with disease activity
Tx of giant cell arteritis
High-dose glucocorticoids prior to temporal artery biopsy to prevent blindness
Epidemiology/presentation of Takayasu arteritis
Usually Asian females < 40 years old. “Pulseless disease” (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances
Lab findings of Takayasu
Granulomatous thickening and narrowing of aortic arch and proximal great vessels
Increased ESR
Tx of Takayasu
Glucocorticoids
What are the medium-vessel vasculitis
Buerger disease (thromboangiitis obliterans), Kawasaki disease, Polyarteritis nodosa
Epidemiology/presentation of Buerger disease (thromboangiitis obliterans) and definition
Heavy tobacco smoking history, males < 40 years old
Intermittent claudication
May lead to gangrene, autoamputation of digits, superficial nodular phlebitis
Raynaud phenomenon is often present
Segmental thrombosing vasculitis with vein and nerve involvement
Tx of Buerger disease (thromboangiitis obliterans)
Smoking cessation
Epidemiology/presentation of Kawasaki disease
Usually Asian children < 4 years old.
Bilateral nonexudative bulbar Conjunctivitis, Rash (polymorphous = desquamating), Adenopathy (cervical), Strawberry tongue (oral mucositis), Hand-foot changes (edema, erythema), fever.
“CRASH and burn (fever) on a Kawasaki”
What can occur as a result in Kawasaki disease
May develop coronary artery aneurysms ; thrombosis or rupture can cause death
Tx of Kawasaki
IV immunoglobulins, aspirin
Epidemiology/presentation of polyarteritis nodosa
Usually middle-aged males.
Hepatitis B seropositivity in 30% of patients
Fever, weight loss, malaise, headache
GI: abdominal pain, melena
Hypertension, neurologic dysfunction, cutaneous eruptions, renal damage
Vessel involvement in polyarteritis nodosa and inflammation
Typically involves renal and visceral vessels, not pulmonary arteries
Different stages of transmural inflammation with fibrinoid necrosis
Lab findings of polyarteritis nodosa
Innumerable renal microaneurysms and spasms on arteriogram (string of pearls appearance)
Tx of polyarteritis nodosa
Glucocorticoids, cyclophosphamide
What are the small vessel vasculitis
Behçet syndrome, Cutaneous small- vessel vasculitis, Eosinophilic granulomatosis with polyangiitis, Granulomatosis with polyangiitis, IgA vasculitis, microscopic polyangiitis, mixed cryoglobulinemia
Presentation of eosinophilic
granulomatosis with polyangiitis
Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (eg, wrist/foot drop)
Can also involve heart, GI, kidneys (pauci-immune glomerulonephritis)
Inflammation in eosinophilic granulomatosis with polyangiitis + lab findings
Granulomatous, necrotizing vasculitis with eosinophilia
MPO-ANCA/p-ANCA, Increased IgE lvl
Presentation of granulomatosis with polyangiitis
Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis
Lower respiratory tract: hemoptysis, cough, dyspnea
Renal: hematuria, red cell casts
Inflammation in granulomatosis with polyangiitis
Triad:
Focal necrotizing vasculitis
Necrotizing granulomas in lung and upper airway
Necrotizing glomerulonephritis
Lab findings in granulomatosis with polyangiitis
PR3-ANCA/c-ANCA (anti-proteinase 3) CXR: large nodular densities
Tx of granulomatosis with polyangiitis
Glucocorticoids in combination with
rituximab/cyclophosphamide
Epidemiology/Presentation of IgA vasculitis
Most common childhood systemic vasculitis
Often follows URI
Classic triad: hinge pain (arthralgias), stomach pain (abdominal pain associated with intussusception), palpable purpura on buttocks/legs
Definition of IgA vasculitis
Vasculitis 2° to IgA immune complex deposition
Associated with IgA nephropathy (Berger disease)
Tx of IgA vasculitis
Supportive care, possibly glucocorticoids
Presentation of microscopic polyangiitis
Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura
Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement
Lab findings of microscopic polyangiitis
No granulomas
MPO-ANCA/p-ANCA J (anti-myeloperoxidase)
Tx of microscopic polyangiitis
Cyclophosphamide, glucocorticoids
Presentation of mixed cryoglobulinemia
Often due to viral infections, especially HCV
Triad of palpable purpura, weakness, arthralgias
May also have peripheral neuropathy and renal disease (eg, glomerulonephritis)
Definition of mixed cryoglobulinemia
Cryoglobulins are immunoglobulins that precipitate in the Cold
Vasculitis due to mixed IgG & IgM immune complex deposition
Epidemiology/presentation of Bechet syndrome
People of Turkish and Eastern Mediterranean descent
Recurrent aphthous ulcers, genital ulcerations, uveitis, erythema nodosum
Can be precipitated by HSV or parvovirus
Flares last 1–4 weeks
Causes and gene association in Bechet
Immune complex vasculitis
Associated with HLA-B51
Define cutaneous small-vessel vasculitis
Occurs 7–10 days after certain medications (penicillins, cephalosporins, sulfonamides, phenytoin, allopurinol) or infections (eg, HCV, HIV)
Palpable purpura, no visceral involvement
Lab findings and late involvement in cutaneous small-vessel vasculitis
Immune complex-mediated leukocytoclastic vasculitis
Late involvement indicates systemic vasculitis.
