Five things not to miss in Dermatology. Dr Judy Wismer Associate Clinical Professor Michael G DeGroote School of Medicine

Transcription

1 Five things not to miss in Dermatology Dr Judy Wismer Associate Clinical Professor Michael G DeGroote School of Medicine

2 Key Descriptives Fever, skin pain Purpura, necrosis Bullae, Mucosal, Skin sloughing erythroderma

3 Dermatologic Medical Emergencies Staphlococcal toxic shock syndrome Angioedema Erythroderma Necrotizing fasciitis Vasculitis Calciphylaxis Purpura Fulminans DRESS Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Lyme Disease

4 Terminology Skin pain: burning, hyperesthesia, dysesthesia, (distinguish from pruritus) Petechiae Purpura Nikolsky s Sign Erythroderma

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6 Five Things Not to Miss Blisters and Denuded Skin Atypical Ulcers Purpura Erythroderma with Pustules Five quick clinicals

7 Five things: blisters and denuded skin

8 55 y o with new onset of superficial bullae, No mucosal fever, leukocytosis No new meds, previously well,now depression, suicidal ideation

9 Ddx: Pemphigus, TEN, paraneoplastic pemphigus. SLE, drug rxn Skin biopsies H&E, DIF Cultures Pemphigus, pemphigoid serology, autoimmune work-up

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11 Blisters, denuded skin Pemphigus vulgaris Paraneoplastic Pemphigus Stevens Johnson Syndrome Bullous Pemphigoid Pemphigus Foliaceous

12 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

13 Pemphigus vulgaris

14 Bullous Pemphigoid

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16 Patient #2 27 year old HIV male presents with one week history conjunctivitis, crusting lip, atypical targetoid lesions acral, trunk

17 Erythema Multiforme/Stevens Johnson Syndrome

18 Toxic Epidermal Necrolysis Fever, systemic toxicity, skin painful, Nikolsky s sign Conjunctivitis, Mucositis, GI, Resp Anemia and lymphopenia sepsis, multiorgan failure, GI hemorrhage

19 Toxic Epidermal Necrolysis (TEN)

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22 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.

23 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

24 Estimates of odds ratio for 7 drugs most often associated with SJS or TEN Drug Patients Control subjects (%) n = (%) n = [ ] 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

25 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)

26 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

27 Five things: unusual ulcers Ecthyma Gangrenosum: Pseudomonal, immune supressed

28 Unusual Ulcers Squamous cell ca

29 Pyoderma gangrenosum

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31 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

32 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

33 Five Things: Purpura Meds, Travel Pain, Fever, malaise, headache arthralgias, GI, hematuria, neuropathy Resp, sinusitis, Cardiac Leg pain, cold hands and feet

34 Tumbler test

35 Vasculitis Spectrum DINCA

36 Vasculitis Atypical ulcerations, necrosis, livedo Consider medium vessel vasculitis ie PAN cutaneous

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39 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

40 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

41 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

42 Purpura Fulminans in severe sepsis Typically develops distal extremities, progressing proximally Neisseria meningitis (children and adolescents) Streptococcus (adults) Multiorgan failure

43 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

44 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

45 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

46 Five things: 4. Erythroderma and Pustules Erythema, pustules, arthralgias Mild leukocytosis Recently started Humira for UC Previously no skin disease

47 Pustular Psoriasis Ddx: pustular drug reaction Recognize new cutaneous SE biologics AVOID systemic corticosteroids Mtx, Mycophenolate Mofetil, UV, Cyclosporine

48 Five more things: perianal rash May present as irritation, persistant diaper rash, painful defecation Itch, tenderness Group A betahemolytic strep cellulits

49 Staphylococcal Scalded Skin Syndrome Less than 6 yrs (98%) Desquamation 3 to 5 days Staphylococcal epidermolytic toxin Cultures negative Fluid loss, risk for infection

50 Periumbilical, Periorificial desquammation

51 Flared Atopic S. Aureus Herpes Simplex

52 Periorbital Edema DRESS SYNDROME DERMATOMYOSITIS

53 Deadly lumps SQUAMOUS CELL CA MELANOMA

54 Five Things Not to Miss Blisters and Denuded Skin Atypical Ulcers Purpura Erythroderma with Pustules Five quick clinicals

55 Thank you!