Wounds in Rheumatic Diseases 2017 TOWN HALL

Learning Objectives Recognize systemic features of rheumatic diseases causing skin wounds Identify patterns of skin lesions in rheumatic diseases Labs, treatment will NOT be discussed

Overview Crystal-associated skin lesions Monosodium urate crystals: gout Calcium crystals: scleroderma, dermatomyositis Vascular disease – arterial insufficiency Vasculitis: small, medium-sized arteries Vasculopathy may affect any size of arteries

Gout A disease caused by monosodium urate crystal saturation of tissues over time due to high blood levels of uric acid More common in men – post-puberty decrease in renal clearance of uric acid Women can develop gout, but usually 15-20y after menopause NO SYSTEMIC FEATURES

Clinical Features of Gout Intermittent acute mono-arthritis Tophi are crystalline joint and superficial subcutaneous deposits that occur years of hyperuricemia These may erode through the skin and cause wounds draining MSU crystals

Gout: clinical manifestations Peripheral joints, one at a time Lower extremity attack precedes upper extremity Attack lasts 7-10 d. Desquamation is typical

Management of Acute Gout Control joint inflammation Options: Joint drainage and intraarticular steroid NSAIDs Colchicine IV or oral steroids

Tophi Rx Background control of inflammation, THEN… Add a med to reduce uric acid

Tophaceous Gout:

Longterm Prognosis Requires years to mobilize tophaceous uric acid stores Residual joint pain if erosions are present

Calcinosis This is a feature of an underlying autoimmune disease. Associated conditions: scleroderma, dermatomyositis The calcium crystal deposits can irritate the overlying skin, cause a sterile cellulitis, and spontaneously drain. This can be therapeutic. Other treatment options: colchicine, steroids

Juvenile Dermatomyositis Dramatic calcinosis

Diffuse Scleroderma Skin thickening, tightening Vasculopathy

Limited Scleroderma Vasculopathy + Raynaud can cause painful digital ischemia, gangrene Treatment: vasodilators, sympathectomy

Vasculitis Inflammation of the blood vessel walls If CUTANEOUS vasculitis, can compromise blood flow to the skin and cause skin lesions – rash, ulceration. Exam, labs and skin biopsy help to determine the size of vessel involved

Vasculitis classification Based on clinical presentation, +/- labs Once ulcerated, may be very tough to tell! Biopsy can be necessary

Nonblanching rash Petechiae: <3mm Purpura are larger, often palpable Typically result from hemorrhage due to trauma or vasculitis 35 yo with small vessel vasculitis due to an antibiotic. Palpable purpura, lesions sting and burn, surrounding edema. Biopsy: LCV Diseases that can cause this: many autoimmune diseases, including lupus and Sjogren syndrome

Leukocytoclastic Vasculitis (LCV) Inflammation of small artery walls with Yellow arrows: cellular debris Black arrows: fibrin thrombi

LCV Small vessels May be idiopathic or due to underlying disease, often autoimmune such as SLE or ANCA vasculitis; r/o drug reaction Burning/stinging lesions w surrounding edema; may ulcerate Treatment: steroids, colchicine

LCV with complications Obese RN with LCV, Foot drop due to SLE Rx prednisone 60 mg daily with slow taper 50 lb wt gain, T2DM, LE edema Nonhealing ulceration Obese 55 yo lady with T2DM complicated by peripheral neuropathy, biopsy proven small vessel cutaneous vasculitis (LCV) 3 years prior, on Dapsone 100 mg daily and prednisone 15 mg twice a day with pitting edema to knees and a painless 6 x4.5 cm ulcer, clean base.no surrounding erythema. Gained >50 lb on steroids over past 3 years. Typically painful, but this is painless due to neuropathy Resolved with steroid titration and management of EDEMA

Systemic or skin only? Fever, chills, sweats, loss of appetite Raynaud Alopecia, malar rash Joint pain

Lupus Young women, 9:1 Raynaud Alopecia Photosensitive rash

Lupus: alopecia

Lupus: Internal Organs Pericarditis, pleurisy Glomerulonephritis, renal failure CNS: seizures, CVA, meningitis APL: thromboembolic events Premature coronary artery disease Increased mortality: infection, CAD