Dermatologic Emergencies Boston University School of Medicine Internal

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

Conflicts of Interests • No Conflicts of Interests

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

Outline • Introduction • Infections – Bacterial – Viral • Life threatening drug eruptions • Others

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

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”

Inpatient Consults CLEAR AND DEFINED question

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

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

Infections - Bacterial - Viral

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

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

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

Staphylococcal Scalded Skin Syndrome

Staphylococcal Scalded Skin Syndrome • 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

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

Necrotizing fasciitis

Necrotizing fasciitis • Clinical presentation – Shock and organ failure • Management – Also need surgical debridement of the necrotic tissue

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

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

Meningococcemia

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

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

Rocky Mountain Spotted Fever First starts on wrists and ankles

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever • 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

Infections -Bacterial -Viral

Eczema Herpeticum • Kaposi’s varicelliform eruption • Etiology – Herpes virus: HSV 1 > HSV 2 – 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

Eczema Herpeticum

Varicella Infection • Etiology – Varicella Zoster Virus (VZV or HSV 3) – 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

Varicella Infection

Varicella Infection • Dermatologic findings – Zoster • Follows dermatome distribution

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 V 1 Distribution

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

Life Threatening Drug Eruptions

Life Threatening Drug Eruptions • Risk factors: – HIV or immunosuppressed patients – Elderly (polypharmacy) – Genetic predisposition • Management – Stop the medication – Supportive care

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%

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

SJS/TEN

SJS/TEN

SJS/TEN • Management – Burn unit, ICU – Ophthalmology, urology – IVIG – Systemic steroid is controversial

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

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

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

Others

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

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) – C 1 esterase inhibitor deficiency: hereditary vs associated with autoimmune disorder or malignancy

Angioedema • Management – – – Airway management Antihistamines Cool compresses Avoid triggers For pts with C 1 esterase inhibitor deficiency: Acute management vs short term vs long term prophylaxis: androgens (danazol and stanozolol), C 1 esterase inhibitor concentrate, antifibrinolytics, icatibant (selective antagoist of bradykinin B 2 receptor)

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

Erythroderma

Erythroderma • 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%

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

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) B) C) D) E) Start IVIG NSAID for pain control Start high dose systemic steroids Stop the sulfonamides Call dermatology

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

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

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) B) C) D) E) Start IVIG NSAID for pain control Start high dose systemic steroids Stop the sulfonamides Call dermatology

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

Selected Future Reading 1. 2. 3. 4. Usatine RP and Sandy N. Dermatologic Emergencies. Am Fam Physician. 2010; 82: (7): 773 -780 Kress DW. Pediatric dermatology emergencies. Current Opinion in Pediatrics. 2011; 23: 403 -406. Freiman A, Borsuk D and Sasseville D. Dermatologic emergencies. CMAJ. 2005; 173 (11): 1317 -1319. 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) Derm. Net. NZ 5) e. Medicine

THANK YOU FOR YOUR ATTENTION ! amychen@bu. edu