1. Dermatologic Emergencies Amy Y-Y Chen, MD, FAAD amychen@bu.edu Boston University School of Medicine Internal Medicine Noon Conference July 26th 2013

2. Conflicts of Interests No Conflicts of Interests

3. Objectives Identify clinical clues to the diagnosis of potentially life-threatening dermatologic conditions Describe the clinical presentation of important dermatologic emergencies Discuss infectious and pharmacologic causes of life-threatening dermatoses

4. Outline Introduction Infections –Bacterial –Viral Life threatening drug eruptions Others

5. Introduction ~15-20 % of visits to primary care physicians and emergency departments are due to dermatologic complaints It is important to be differentiate simple skin conditions from the more serious, life threatening conditions that require immediate intervention

6. Clues to the Presence of a Potential Dermatologic Emergency Fever and rash Fever and blisters or denuding skin Rash in immunocompromised Palpable purpura “Full body redness”

7. Inpatient Consults CLEAR AND DEFINED question

8. Inpatient Consults Have seen and examined the patient and able to provide some pertinent hx Not acceptable: –“Saw a derm note in EMR” –“Something on the skin” –“Patient being discharged today, needs stat consult” MD to MD contact If patient has pre-existing derm problem and was doing well on therapy, consider keeping them on therapy when they are admitted

9. Inpatient Consults Benefits and limitation of biopsy – Not black and white –Takes a few days to come back –Tissue cultures can take a few weeks –Suture removal Follow up on recommendation –Topical therapy takes a few days to a week to work

10. Infections - Bacterial - Viral

11. Staphylococcal Scalded Skin Syndrome Etiology –Toxin-mediated cleavage of the skin at granular layer resulting in a split –Risk factors: newborn, children or adults w/ renal failure

12. Staphylococcal Scalded Skin Syndrome Dermatologic findings –Erythema periorificially on the face, neck, axilla, groin. Then generalized within 48 hrs as the color deepens –Skin tenderness –Flaccid bullae w positive Nikolsky sign –Within 1-2 days, flexural areas begin to slough off –Complete re-epithelialization in 2 weeks

13. Nikolsky Sign Positive when a blister occurs on normal appearing skin after application of lateral pressure w/ a finger Occurs in any superficial blistering process

14. Staphylococcal Scalded Skin Syndrome

15. Clinical presentation –Prodrome of fever, malaise, sore throat Complication –Mortality rate is 3% in kids, > 50% in adults and 100% in adults with underlying diseases –If in newborn nursery, needs isolation –Identify possible staph carrier

16. Necrotizing fasciitis Etiology –Necrosis of subcutaneous tissue due to infection Type I : mixed anaerobes, gram negative aerobic bacilli and enterococci Type II: group A streptococci –Risk factors: diabetes, peripheral vascular disease, immunosuppression Dermatologic findings –Diffuse edema and erythema of the affected skin-> bullae-> burgundy color-> gangrene –Severe pain, anesthesia. crepitus, exudates

17. Necrotizing fasciitis

18. Clinical presentation –Shock and organ failure Management –Also need surgical debridement of the necrotic tissue

19. Meningococcemia Etiology –Neisseria meningitides (gram neg diplococcus) spread by respiratory route –Often seen in young adults and children –Risk factor: asplenia, immunoglobulin or terminal complement deficiencies Dermatologic findings –Abrupt onset of maculopapular or petechial eruption on acral surface, trunk or lower extremities -> progression to purpura in hours –Angular edge with “gun metal gray” center –+/- mucosal involvement

20. Meningococcemia Clinical presentation –Flu like symptoms: fever, chills, malaise –DIC, shock, death

21. Meningococcemia

22. Rocky Mountain Spotted Fever Etiology –Rickettsia Rickettsii carried by ticks –Only 60% aware of tick bites –Geographic location

23. Rocky Mountain Spotted Fever Dermatologic findings –Purpuric macules and papules –Starts on the wrists and ankles within 2 weeks-> spread to palms, soles-> to trunk and face –Over 2-4 days, the skin will become hemorrhagic and petechial –May have eschar at site of bite

24. Rocky Mountain Spotted Fever First starts on wrists and ankles

25. Rocky Mountain Spotted Fever

27. Clinical presentation –Triad: fever, headache and rash (only in 60%) –Can have variety of organ involvement (cardiogenic shock, hepatic failure, renal failure, meningismus and DIC) Management –Mortality is 30-70% if untreated vs 3-7% if treated –Ideally should start within 5 days of infection –DOXYCYCLINE ! Even in kids

28. Infections -Bacterial -Viral

29. Eczema Herpeticum Kaposi’s varicelliform eruption Etiology –Herpes virus: HSV1 > HSV2 –Risk factor: any diseases w impaired skin barrier Dermatologic findings –2-3 mm umbilicated vesicles-> punched out erosions-> hemorrhagic crusts –If severe, may have systemic involvement

30. Eczema Herpeticum

31. Varicella Infection Etiology –Varicella Zoster Virus (VZV or HSV3) –Causes of chicken pox (primary infection) and shingles (reactivation) Dermatologic findings –Primary Pruiritic erythematus macules and papules-> vesicles with clear fluid surrounded by narrow red halos (dew drops on a rose petal) Lesions in all stages of development

32. Varicella Infection

33. Dermatologic findings –Zoster Follows dermatome distribution

34. Varicella Infection Zoster Prodrome in 90% Disseminated lesions (> 20 vesicles outside of the area of primary or adjacent dermatomes) and/or visceral involvement seen in approximately 10% of immunocompromised patients V1 Distribution

35. Management Treatment of underlying infections –Antibiotics, broad spectrum until organism identified –Antiviral Supportive care with fluid and electrolyte management

36. Life Threatening Drug Eruptions

37. Risk factors: –HIV or immunosuppressed patients –Elderly (polypharmacy) –Genetic predisposition Management –Stop the medication –Supportive care

38. Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) Pathophysiology: –Drug induced mucocutaneous reaction –Culprit medications: Sulfonamides, anticonvulsants, allopurinol, NSAIDs. Usually given 1-3 weeks before onset –Genetic susceptibility SJS and TEN are continuum –SJS: BSA < 10% –SJS/TEN overlap: BSA 10-30% –TEN: BSA > 30%

39. SJS/TEN +/- Clinical presentation –Prodrome: fever, chills, malaise –Stinging eyes, difficulty swallowing and urinating Dermatologic findings –Skin tenderness –Dusky erythema –Epidermal detachment and desquamation –Mucosal involvement

40. SJS/TEN

42. Management –Burn unit, ICU –Ophthalmology, urology –IVIG –Systemic steroid is controversial

43. DRESS DRESS: Drug Reaction with Eosinophilia and Systemic Symptoms –Anticonvulsant hypersensitivity syndrome –Drug-induced hypersensitivity syndrome –Hypersensitivity syndrome –Drug-induced delayed multi-organ hypersensitivity syndrome Pathophysiology: –Idiosyncratic, problem with drug detoxification –Drug exposure to onset of symptoms 2-6 wks –Common culprit: aromatic anticonvulsant, sulfonamides, minocycline, allopurinol, antiretroviral drugs, NSAIDS, CCB

44. DRESS Dermatologic findings –Maculopapular (morbilliform) and urticarial eruption most common –Vesicles, bullae, pustules, purpura, targetoid lesions, erythroderma –Facial edema (mistaken for angioedema)

45. DRESS Clinical presentation –Fever, eosinophilia, lymphadenopathy, –Hepatic damage (can be fulminant), endocrinopathy, myocarditis Management –Systemic corticosteroid with slow taper

46. Others

47. Angioedema Pathophysiology –Increased intravascular permeability Dermatologic findings –Well circumscribed acute cutaneous edema due to increased intravascular permeability –Face, lips, extremities, genitalia –Painful, usually not pruritic Clinical presentation –Abdominal pain –Respiratory distress

48. Angioedema Etiology: –Often idiopathic –Medications angiotensin-converting- enzyme inhibitor in 10-25% of cases Penicillin NSAID –Allergens (foods, radiographic contrast media) –Physical agents (cold, vibration, etc) –C1 esterase inhibitor deficiency: hereditary vs associated with autoimmune disorder or malignancy

49. Angioedema Management –Airway management –Antihistamines –Cool compresses –Avoid triggers –For pts with C1 esterase inhibitor deficiency: –Acute management vs short term vs long term prophylaxis: androgens (danazol and stanozolol), C1 esterase inhibitor concentrate, antifibrinolytics, icatibant (selective antagoist of bradykinin B2 receptor)

50. Erythroderma Dermatologic findings –Generalized erythema involving 90% of BSA –Pruritus Clinical presentation –Fever, malaise –Excessive vasodilatation-> protein and fluid loss Hypotension, electrolyte imbalance, congestive heart failure Etiology: –50% due to preexisting dermatoses Seborrheic dermatitis, contact dermatitis, lymphoma (CTCL), leukemia, atopic dermatitis, psoriasis, pityriasis rubra pilaris, idiopathic, drugs (esp in HIV pts) –Search for clues on physical examination

51. Erythroderma

52. Management –Supportive care with fluid and electrolyte –Need to search for underlying causes-> treatment of underlying dermatoses (topical corticosteroids, emollients) –Abx of signs of infection –Mortality is 18%

53. Question 1: This patient presents with few days history of malaise and decrease oral intake. What is the most appropriate therapy? A)Topical antibiotics B)Oral antibiotics C)IV antiviral D)Topical antiviral E)Topical steroids

54. Question 2: This patient was given sulfonamides two weeks ago for an UTI. She now presents to the ED with painful skin, which one of the following is the most important first step? A)Start IVIG B)NSAID for pain control C)Start high dose systemic steroids D)Stop the sulfonamides E)Call dermatology

55. Question 3: The patient in Question 2 is now stabilized and in the Burn unit. What organ system(s) can potentially be involved in the disease process? A)Eyes B)Aerodiguestive track C)Urinary tract D)All of the above E)None of the above

56. Question 1: This patient presents with few days history of malaise and decrease oral intake. What is the most appropriate therapy? A)Topical antibiotics B)Oral antibiotics C)IV antiviral D)Topical antiviral E)Topical steroids

57. Question 2: This patient was given sulfonamides two weeks ago for an UTI. She now presents to the ED with painful skin, which one of the following is the most important first step? A)Start IVIG B)NSAID for pain control C)Start high dose systemic steroids D)Stop the sulfonamides E)Call dermatology

58. Question 3: The patient in Question 2 is now stabilized and in the Burn unit. What organ system(s) can potentially be involved in the disease process? A)Eyes B)Aerodiguestive track C)Urinary tract D)All of the above E)None of the above

59. Selected Future Reading 1.Usatine RP and Sandy N. Dermatologic Emergencies. Am Fam Physician. 2010; 82: (7): 773-780 2.Kress DW. Pediatric dermatology emergencies. Current Opinion in Pediatrics. 2011; 23:403-406. 3.Freiman A, Borsuk D and Sasseville D. Dermatologic emergencies. CMAJ. 2005; 173 (11): 1317-1319. 4.OR you can rotate with us !! References (including images) 1)Dermatology 2)Fitzpatrick’s Dermatology 3)Fitzpatrick’s color atlas and synopsis of clinical dermatology 4)DermNet.NZ 5)eMedicine

60. THANK YOU FOR YOUR ATTENTION ! amychen@bu.edu