Five things not to miss in Dermatology Dr Judy Wismer Associate Clinical Professor Michael G DeGroote School of Medicine
Key Descriptives Fever, skin pain Purpura, necrosis Bullae, Mucosal, Skin sloughing erythroderma
Dermatologic Medical Emergencies Staphlococcal toxic shock syndrome Angioedema Erythroderma Necrotizing fasciitis Vasculitis Calciphylaxis Purpura Fulminans DRESS Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Lyme Disease
Terminology Skin pain: burning, hyperesthesia, dysesthesia, (distinguish from pruritus) Petechiae Purpura Nikolsky s Sign Erythroderma
Five Things Not to Miss Blisters and Denuded Skin Atypical Ulcers Purpura Erythroderma with Pustules Five quick clinicals
Five things: blisters and denuded skin
55 y o with new onset of superficial bullae, No mucosal fever, leukocytosis No new meds, previously well,now depression, suicidal ideation
Ddx: Pemphigus, TEN, paraneoplastic pemphigus. SLE, drug rxn Skin biopsies H&E, DIF Cultures Pemphigus, pemphigoid serology, autoimmune work-up
Blisters, denuded skin Pemphigus vulgaris Paraneoplastic Pemphigus Stevens Johnson Syndrome Bullous Pemphigoid Pemphigus Foliaceous
Blistering and Denuded Skin Biopsy H&E, DIF, Pemphigus Foliaceous Imperative nursing care and skin dressing changes Burn Unit High Dose Solumedrol, Methotrexate, IVIG, rituximab
Pemphigus vulgaris
Bullous Pemphigoid
Patient #2 27 year old HIV male presents with one week history conjunctivitis, crusting lip, atypical targetoid lesions acral, trunk
Erythema Multiforme/Stevens Johnson Syndrome
Toxic Epidermal Necrolysis Fever, systemic toxicity, skin painful, Nikolsky s sign Conjunctivitis, Mucositis, GI, Resp Anemia and lymphopenia sepsis, multiorgan failure, GI hemorrhage
Toxic Epidermal Necrolysis (TEN)
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis SJS and TEN are rare, life-threatening, bullous cutaneous diseases generally considered as immune-mediated reactions to drugs. These severe cutaneous adverse reactions (SCAR) are characterized by epidermal necrosis, extensive detachment of the epidermis, erosions of mucous membranes and severe constitutional symptoms.
SJS/TENS TEN Virtually always represents idiosyncratic reaction to medication Allopurinol, Phenytoin, Phenobarbital, Carbamazepine,Sulfonamide antibiotics, NSAIDS, Quinolones,Lamotrigine, Greatest risk in first two months of RX Other: (SJS) vaccinations, Mycoplasma pneumoniae, Herpes Simplex, chinese herbals, peudoephedrine
Estimates of odds ratio for 7 drugs most often associated with SJS or TEN Drug Patients Control subjects (%) n = (%) n = 1505 379 [ ] Allopurinol Adjusted OR (95% CI) 66 (17.4) 28 (1.9) 18 (11-32) Carbamazepine 31 (8.2) 4 (0.3) 72 (23-225) Clotrimoxazole 24 (6.3) 1 (0.1) ND Nevirapine 21 (5.5) 0 [ ] Phenobarbital ND 20 (5.3) 5 (0.3) 16 (5.0-50) Phenytoin 19 (5.0) 3 (0.2) 17 (4.1-68) Lamotrigine 14 (3.7) 0 [ ] ND
Effect of treatments on the mortality of SJS and TENS Retrospective European Study No treatment modality established as standard Neither intravenous immunoglobulins nor corticosteroids showed any significant effect in comparison with supportive care Thalidomide RCT negative (TNF-a) JAAD Vol 58 (Jan 2008)
Management SJS/TEN Skin biopsy Differential DX: Drug-induced Bullous Pemphigoid, DrugInduced Pemphigus, Staphylococcal Scalded Skin, Paraneoplastic Pemphigus, Acute GVHD Discontinue Drug TEN admit to burn unit Fluid management, eyes, resp tract, electrolytes, nutrition, infection and pain relief
Five things: unusual ulcers Ecthyma Gangrenosum: Pseudomonal, immune supressed
Unusual Ulcers Squamous cell ca
Pyoderma gangrenosum
Pyoderma Gangrenosum Painful ulceration, worsened by trauma, 50% association with underlying Crohn s, Ulcerative Colitis, rheumatoid arthritis, hematologic, malignancy Clinical Dx. R/O: vascular, vasculitis, neoplastic, infectious, pancreatic, drugs, cryoglobulins, antiphospholipid etc Treat pain, secondary infection, oral steroids,(12mg/kg/day) immunosuppressive (MTX, Mycophenolate,CyA, Imuran, Infliximab) avoid debridement
Five Things: Purpura Purpuric lesions are the clinical expression of passage of erthrocytes from the vascular compartment to the extravascular one Cutaneous, self limiting Systemic, life threatening
Five Things: Purpura Meds, Travel Pain, Fever, malaise, headache arthralgias, GI, hematuria, neuropathy Resp, sinusitis, Cardiac Leg pain, cold hands and feet
Tumbler test
Vasculitis Spectrum DINCA
Vasculitis Atypical ulcerations, necrosis, livedo Consider medium vessel vasculitis ie PAN cutaneous
Henoch Schonlein Purpura Children 3-7 yrs Post upper resp tract illness Petechiae coalesce, buttock, legs Young children facial swelling Fever, Abdominal pain, Renal : hematuria, HT, failure IgA mediated Corticosteroids symptomatic relief Plus azathiprine, Cyclosporin A severe renal
Purpura Fulminans Haematological emergency, vessel thrombosis skin necrosis and disseminated intravascular coagulation May progress to multiorgan failure PF may complicate severe sepsis or may occur as an autoimmune response to previous infection
Purpura Fulminans PR in severe sepsis Systemic activation of coagulation and complement pathways and endothelial dysfunction Microvascular thrombosis Loss of anticoagulant and anti-inflammatory proteins, Protein C, Protein S, Antithrombin III Menigococcus, Group A and B strep, Haemophilus, Staphlococcus
Purpura Fulminans in severe sepsis Typically develops distal extremities, progressing proximally Neisseria meningitis (children and adolescents) Streptococcus (adults) Multiorgan failure
Post infectious purpura fulminans PF lesions appear a few days or weeks after a febrile illness Lower body, thighs, lower legs, buttock, genitals Varicella, Streptococcus PF and DIC in otherwise well pt Acquired deficiency PS
Heritable PC and PS deficiency PF is presenting feature of severe heritable deficiency of PC or PS Partial PC deficiency risk factor for adultonset and childhood thromboembolic disease and PF
Acute Management of PF Haematologic multi-organ thrombotic injury Broad spectum antibiotic coverage DIC support, FFP Recombinant Activated PC adults Phase II trial: children APC no survival benefit Surgical management fasciotomies,debridement, amputation Critical Care Med 2003, 2008
Five things: 4. Erythroderma and Pustules Erythema, pustules, arthralgias Mild leukocytosis Recently started Humira for UC Previously no skin disease
Pustular Psoriasis Ddx: pustular drug reaction Recognize new cutaneous SE biologics AVOID systemic corticosteroids Mtx, Mycophenolate Mofetil, UV, Cyclosporine
Five more things: perianal rash May present as irritation, persistant diaper rash, painful defecation Itch, tenderness Group A betahemolytic strep cellulits
Staphylococcal Scalded Skin Syndrome Less than 6 yrs (98%) Desquamation 3 to 5 days Staphylococcal epidermolytic toxin Cultures negative Fluid loss, risk for infection
Periumbilical, Periorificial desquammation
Flared Atopic S. Aureus Herpes Simplex
Periorbital Edema DRESS SYNDROME DERMATOMYOSITIS
Deadly lumps SQUAMOUS CELL CA MELANOMA
Five Things Not to Miss Blisters and Denuded Skin Atypical Ulcers Purpura Erythroderma with Pustules Five quick clinicals
Thank you!