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J tnoon conf
- 2. © 2016 Virginia Mason Medical Center 2 Objectives DRESS SYNDROME (Drug reaction with eosinophilia and systemic symptoms) • Review diagnostic criteria • Discuss clinical presentation • Discuss diagnostic tests • Review illness script • Discuss treatment
- 3. © 2016 Virginia Mason Medical Center Diagnostic Criteria 3
- 4. © 2016 Virginia Mason Medical Center Clinical Presentation Affected Body Part Symptoms Skin Morbilliform rash, progresses to diffuse, confluent, and infiltrated erythema with follicular accentuation. Face Facial edema & Erythema. Inflammation and pain of muccous membranes Kidney Interstitial nephritis Systemic (Fever) 100.4 to 104 F Liver Hepatitis, leading to jaundice/hepatomegaly or asymptomatic Systemic (Lymphadenopathy) Diffuse lymphadenopathy with 1-2cm tender nodes. Lungs nonspecific symptoms, including cough, fever, and tachypnea/dyspnea Other organs Heart, GI, Pancreas, Thyroid, Brain, Muscle, Peripheral nerves, Eyes 4
- 5. © 2016 Virginia Mason Medical Center 5 right lung nodules and infiltrates
- 6. © 2016 Virginia Mason Medical Center Diagnostic Tests • CBC w/Diff • LFT’s • Serology for viral hepatitis • Serum Creatinine & UA • Skin Biopsy • CXR or CT 6
- 7. © 2016 Virginia Mason Medical Center 7 Severe Cutaneous Reactions Stevens-Johnson syndrome/Toxic epidermal necrolysis • Mostly clinical, SJS/TEN usually 4-28 after drug exposure, 90% have erosions & bleeding on at least 2+ sites • 50% of DRESS have mucosal involvement, usually single site.
- 8. © 2016 Virginia Mason Medical Center Illness Scripts 8 DRESS SJS Pathophysiology Drug Specific Immune Response Drug Specific Immune Response Epidemiology Adults>Children M=F 1-5 per 10,000 Higher incidence in (Lamictal)Lamotrigine HIV w/Cancer>General population M < F 2-7 per 1,000,000 Time course Normally 2-6 weeks after exposure First 8 weeks of treatment Clinical presentation Fever, Malaise, lymphadenopathy, skin eruption Fever, malaise, mucocutaneous lesions, Photophobia, myalgia, Nikolsky sign (Skin sloughing) Diagnostics Labs:CBC w/ leukocytosis and Eosinophillia; Elevated LFT’s, increased creatinine Skin Biopsy: Non-Specific CXR/CT: Interstitial pneumonitis/Pleural effusion Labs: CBC w/ anemia & Lymphopenia. Neutropenia in 1/3 of patients.l Electrolyte abnormalities 2/2 fluid loss Skin biopsy:Non-Specific CXR/CT=Non-specific Therapeutics Withdraw medication, supportive care, general consensus on systemic steroids Supportive care (Burn unit), Etanercept promising
- 9. © 2016 Virginia Mason Medical Center Question #1 In a diagnosis of DRESS, which finding can be absent in as many as 50% of patients A. Elevated LFT’s B. Eosinophillia C. Fever D. Rash 9
- 10. © 2016 Virginia Mason Medical Center Question #2 In DRESS, involvement of at least 1 internal organ occurs in approximately how many patients? A. 40% B. 70% C. 80% D. 90% 10
- 11. © 2016 Virginia Mason Medical Center 11
Editor's Notes
- Title your presentation “Noon Conference” Prevents inadvertently giving away the case.
- Registry of Severe Cutaneous Adverse Reactions (RegiSCAR)
- Chest radiograph or CT scan may provide evidence of interstitial pneumonitis and/or pleural effusion. Drug-specific CD8+ T lymphocytes and eosinophils may be found in broncho-alveolar lavage fluid Other organs — Several other organs can be involved in DRESS, including [10]: ●Heart (eosinophilic myocarditis, pericarditis) ●Gastrointestinal tract (diarrhea, mucosal erosions, bleeding) ●Pancreas (pancreatitis) ●Thyroid (autoimmune thyroiditis, appearing often late, as a sequel of DRESS) ●Brain (encephalitis, meningitis) ●Muscle (myositis, increase in creatine kinase) ●Peripheral nerves (polyneuritis) ●Eye (uveitis)
- The initial laboratory evaluation in a patient suspected to have DRESS is aimed at confirming the diagnosis, excluding other conditions that mimic DRESS, and evaluating the extent and severity of visceral involvement (table 3). Laboratory tests include:
- Diagnosis of SJS/TEN requires clinicopathologic correlation. The appropriate clinical findings include targetoid and atypical targetoid skin lesions and at least two mucosal surfaces involved (ocular, oral, genital, etc.).3,5 Nikolsky's sign, which refers to epidermal detachment occurring with lateral pressure adjacent to bullae, is present and can be a clue to diagnosis. In patients with these appropriate clinical findings, a lesional skin biopsy should be performed to confirm the diagnosis and rule out other possible entities, including staphylococcal scalded skin syndrome, generalized fixed drug eruption, and drug-induced linear IgA bullous dermatosis.7 Lesional biopsies reveal full thickness epidermal necrosis with minimal dermal inflammation
- Skin Biopsy-mild spongiosis and a lymphocytic infiltrate in the superficial dermis, predominantly perivascular, with eosinophils and dermal edema, Etanercept trial of 91 People was associated with a shorter time to complete skin healing and lower mortality rate compared with intravenous prednisolone [1]. Serious adverse events, including sepsis, respiratory failure, and upper gastrointestinal bleeding were less frequent in the etanercept group.
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