Scleroderma and CREST syndrome

Terms in this set (17)

What is scleroderma?
-A rare, devastating connective tissue disorder
-There is chronic hardening and tightening of the skin
-Systemic sclerosis: involvement of internal organs--->diffuse or local cutaneous (depending on distribution of skin involvement)
What is the pathophysiology of scleroderma?
-Immune activation of autoantibodies
-Vascular damage (activation of endothelial cells, up regulation of adhesion molecules)
-Excessive production of extracellular matrix with increased collagen deposition (thickness!)
-Cytokine production (mediates proliferation of vascular and connective tissue) to produce more collagen
What are symptoms of scleroderma?
-Fatigue
-Stiff joints
-Loss of strength
-Pain
-Sleep difficulties
-Skin discoloration
-Affects the fingers and hands first, face earliest involvement with hardening and thickening
-Edema and erythema may precede skin indurations
-Common skin features: sclerodactyly, digital ulcers, pitting at the fingertips, telangiectasia, calcinosis cutis (calcium deposits in skin), edema and pruritus in the early stages
Oral manifestations of systemic sclerosis
Skin findings of systemic scleroderma
-Beak like nose
-Shiny skin
-Taut lips
-Salt and pepper hypo/hyperpigmentation
Sclerodactyly in systemic scleroderma
-The skin is very taut
-Painful
-Easy to get digital ulcers
-Disability with work
Telangiectasia in systemic scleroderma
Calcinosis cutis in systemic scleroderma
-These are rock hard
What are clinical manifestations of local scleroderma?
-Sclerosis restricted most commonly to hands and distal forearm
-Prominent vascular manifestations (Raynaud's phenomenon)
What is CREST syndrome?
-Subgroup of scleroderma with limited disease but has all these things:
-Calcinosis cutis
-Raynaud's phenomenon
-Esophageal dysmotility
-Sclerodactyly
-Telangectasia
What is Raynaud's Phenomenon?
-Reversible vasospasm due to functional changes in arteries/arterioles of hands and/or feet
-Pallor=white
-Cyanosis=blue
-Hyperemia=red
-Can result in ischemic ulceration or infarct if prolonged
-In local cutaneous, raynaud's typically precedes scleroderma symptoms
-In diffuse cutaneous, raynaud's typically coincides with scleroderma symptoms
What are clinical manifestations of diffuse scleroderma?
-Sclerotic skin on chest, abdomen, upper arms, and shoulders
-More likely to have internal organ involvement due to fibrosis or ischemic injury
-Raynaud's phenomena
What are some extracutaneous manifestations of scleroderma?
-GI (90%)-->esophageal hypo motility, incompetent lower esophageal sphincter (it gets sclerotic as well and it doesn't move well), dysphagia, choking, pyrosis, hoarseness, cough with swallowing, bloating, altered bowel movements, fecal incontinence
-Pulmonary (70%)-->interstitial lung disease, 5 fold increase of lung cancer (leading cause of mortality)
-Renal (60-80%)-->renal disease and renal crisis, there is a significant increase with BP
-Cardiac-->pericarditis, heart failure, MI
-Musculoskeletal-->swelling of hands, arthralgia, myalgia, fatigue
-GU-->ED, vaginal dryness
What are some ddx to scleroderma?
-Host vs graft reaction
-Amyloidosis
-Drug-induced (chemo)
-Myxedema
-DM
-SLE
-Environment exposures (farming chemicals)
-BUT if Raynaud's phenomenon is present then we can exclude these DDX
How do you diagnose scleroderma?
-Clinical: presence of skin thickening, hardening, puffy fingers, digital ulcers and presence of extracutaneous features
-CBC with diff
-Creatinine
-CK will usually be elevated bc of the breakdown
-U/A will show albumin
-ANA to help support but it is not confirmatory
-ACA antibodies in local cutaneous
-Anti-DNA topoisomerase in diffuse cutaneous (increases interstitial lung disease risk)
-Skin biopsy is not needed to confirm but best used if in doubt of diagnosis (not first line therapy bc they're really hard to heal. Maybe do if they had a lot of autoimmune disorders)
How do you treat scleroderma?
-Refer to rheumatologist
-Patient education
-Psychological support
-Pretreatment evaluation-->assess organ involvement and treatments are organ specific
-Localized cutaneous=UV-A light therapy (softens lesions), highly potent topical steroids, Vit D, methotrexate
-Diffuse cutaneous=treat pruritus with antihistamines, low-dose oral steroids, telangiectasia with green makeup/laser treatment, and calcinosis with a dentist drill (derm)
-Raynauds=calcium channel blocker
-GI=empirically give PPI to prevent strictures and erythromycin for esophageal hypo motility
-Musculoskeletal=APAP, NSAIDs for symptomatic relief
What is the prognosis of scleroderma?
-Increased premature death with systemic scleroderma
-#1 is pulmonary disease
-The more cardiac, GI, and pulmonary HTN involvement they have, the more significant disease
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