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Toronto Notes 2019 Heritable Disorders Differential Diagnosis
• scarletfever,phototoxiceruption,GVHD,SSSS,exfoliativedermatitis,AGEP,paraneoplasticpemphigus
Management
• discontinueoffendingdrug
• admittointermediate/intensivecare/burnunit
• supportivecare:IVfluids,electrolytereplacement,nutritionalsupport,paincontrol,woundcare,sterile
handling, monitor for and treat infection • IVIg or cyclosporine or etanercept
Other
FIXED DRUG ERUPTION
Clinical Presentation
• morphology:sharplydemarcatederythematousovalpatchesontheskinormucousmembranes
• spread:commonlyface,mucosa,genitalia,acral;recursinsamelocationuponsubsequentexposureto
the drug (fixed location)
Epidemiology
• commoncausativeagents:antimicrobials(tetracycline,sulfonamides),anti-inflammatories, psychoactive agents (barbiturates), phenolphthalein
Management
• discontinueoffendingdrug±prednisone1mg/kg/dx2wkforgeneralizedlesions±potenttopical corticosteroids for non-eroded lesions or antimicrobial ointment for eroded lesions
PHOTOSENSITIVITY REACTION
Clinical Presentation
• phototoxicreaction:“exaggeratedsunburn”(erythema,edema,vesicles,bullae)confinedtosun-exposed areas
• photoallergicreaction:pruriticeczematouseruptionwithpapules,vesicles,scaling,andcrustingthat may spread to areas not exposed to light
Pathophysiology
• phototoxicreaction:directtissueinjury
• photoallergicreaction:typeIVdelayedhypersensitivity
Epidemiology
• commoncausativeagents:chlorpromazine,doxycycline,thiazidediuretics,procainamide
Management
• sunprotection±topical/oralcorticosteroids
Heritable Disorders
Ichthyosis Vulgaris
Clinical Presentation
• xerosiswithfinescalingaswellaslargeadherentscales(“fish-scales”)
• affectsarms,legs,palms,soles,back,forehead,andcheeks;sparesflexuralcreases • improvesinsummer,withhumidity,andasthechildgrowsintoadulthood
Pathophysiology
• geneticdeficiencyinfilaggrinproteinleadstoabnormalretentionofkeratinocytes(hyperkeratosis) • scalingwithoutinflammation
Epidemiology
• 1:300incidence
• autosomaldominantinheritance
• associatedwithADandkeratosispilaris
Investigations
• electronmicroscopy:keratohyalingranules
Management
• immersioninbathandoilsfollowedbyanemollientcream,humectantcream,orcreams/oilcontaining urea or α- or β-hydroxy acids
• intermittentsystemicretinoidsforseverecases
Dermatology D23
Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta- analysis
JAMA Dermatol 2017;153:514-22.
Purpose: To examine possible immunomodulating treatments for SJS/TEN and estimate mortality effect compared to supportive care.
Methods: Systematic review of randomized
and nonrandomized studies on systemic immunomodulating therapies or supportive care
for SJS/TEN.
Results: Ninety-six studies with 3,248 patients were included in the final analysis. Glucocorticoids were associated with a survival benefit for
patients (aggregate - OR 0.5, 95% CI 0.3-1.01). Though findings were limited to a small number of patients overall, cyclosporine was associated with significant benefit (unstratified iPD model – OR
0.1; 95% CI 0.0-0.4). No beneficial effects were observed with other therapies, including intravenous immunoglobulins.
Conclusion: Though based on limited evidence, glucocorticoids and cyclosporine were most promising as SJS/TEN immunomdulating therapies.