2/3/2014 Deadly Derm Kathleen Cowling, MS, DO, FACEP MCEP Winter Symposium Boyne Mountain 2014 Case #1 • 20 y.o. male college student presents with rash, fever, and headache. He appears ill. • PMHx: none • SocHx: Sexually active, recent travel to NC • Vitals: BP 110/60, RR 18, HR 110, T 101.8° 1 2/3/2014 Rocky Mountain Spotted Fever • • • • Etiology: Tick vector for Rickettsia rickettsi Where: Eastern 2/3 of the US Incidence: varies by State Clues: maculopapular palmar rash, starts distally and spreads centrally, progresses to palpable petechial (vasculitis) • Caveats: only ~50% remember the tick • Diagnosis: Clinical, DO NOT WAIT TO TREAT Rocky Mountain Spotted Fever • M&M: Death 30% untreated, which drops to 5% with ABX. 15% have permanent deficits • Treatment: Doxycycline even for PEDS and OB Case #2 • 18 y.o. woman with confusion and fever, and evolving rash, college student • PMHx: asthma • SocHx: No drugs, non-smoker, rare Etoh Meningococcemia Meningococcemia • Etiology: Neisseria meningitidis • Pathophysiology: Gram – intracellular diplococci causing fulminate sepsis • Who: close quarters, adolescents, peds<4 • Clues: ill appearing, fever, shocky, mental status changes, maculopapular rash begins on extremities and spreads, evolves into palpable petechia • Diagnosis: LP, pleocytosis Glucose, Protein • Treatment: Ceftriaxone DOC, Dexamethasone 0.15/kg before ABX if possible • Prevention: vaccine • Prophylaxis: Cipro PO adults, Ceftriaxone IM peds • Mortality: 3-50% depending on treatment • Morbidity: DIC, ARDS, ARF 2 2/3/2014 Case #3 • 60 y.o. male complaining of weakness and weight loss with fatigue • PMHx: HTN, DM, ESRD • Vitals: HR 140, RR 26, BP 98/60 T 101.5° • PE: Appears toxic, JVD, coarse Breath sounds, 3/6 systolic murmur, generalized palpable petechial rash Bacterial Endocarditis • Symptoms: Fever, weakness, fatigue, weight loss • Signs: Murmur, Janeways, Oslers nodes, splinter hemorrhages, Roth spots, petechial rash (most common finding) • Clues: Valvular disorders, IVDU, Ports, Dialysis poor dental hygiene • Etiology: Streptococcus viridans, coag negative staphylococci Case #4 • 35 y.o. female complaining of fever and rash to her legs • PMHx: neg • SocHx: no IVDU • Vitals: BP 100/50, RR 24, HR 120, T 101.0° • PE: non-palpable Petechiae/Purpura Bacterial Endocarditis • Diagnosis: Blood Cultures X 3 and TEE • Treatment: Broad, but cover MRSA with vanco, or linezolid, gentamicin • Mortality: Staph 25-40% Strep 19% • Morbidity: high incidence of valvular surgery Thrombotic Thrombocytopenic Purpura • Symptoms: Fever, neuro changes, rash • Diagnosis: Thrombocytopenia, hemolytic anemia, LDH, renal failure • Etiology: Deficiency of ADAMTS13, platelet aggregation  thrombi • Treatment: Plasmapheresis • Causes: HIV,SLE, pregnancy, malignancy, transplant, Quinine, Cyclosporine, Clopidogrel 3 2/3/2014 TTP • Clues: PT, PTT, & fibrin are often normal, will see helmet cells and schistocytes on smear • Treatment: Immediate Heme consult, need plasmapheresis, if not available give FFP and transfer. Steroids and admit ICU • Mortality: >90% if not treated,  10% with exchange transfusion • AVOID: giving platelets- fuels the fire, causes more thrombi “Fifth Disease” Erythema Infectiosum • Etiology: Parvovirus B19, respiratory spread • Symptoms: fever, headache, runny nose, arthralgias • Signs: “slapped cheek” erythematous cheeks • Diagnosis: Clinical ( serum to check for immunity only if necessary) • Treatment: supportive • Why a deadly derm? Isolation-protect Purpura Fulminans/DIC • Signs: Hemorrhagic bullae, Multi organ failure • Etiology: Sepsis, Gram neg, & meningococcus - Massive trauma, hepatic failure, end stage malignancy, snake envenomations, transfusion reactions, pregnancy • Stimulus causes endogenous over activation of fibrinolytic system, resulting in bleeding, vessel occlusion and end organ damage Case#5 • • • • 4 y.o. male with rash and fever, rhinorrhea PMHx: neg Immun: UTD Vitals: BP 90/62, HR 105, RR 22, T 100.6° Case #6 • 49 y.o. female via EMS, lethargic, bleeding gums, hypotensive and petechial rash with widespread bullae • PMHx: HTN, COPD, known lung CA with mets • Vitals: BP 70/35, HR 134, RR 30, T 102° • PE: toxic, dry MM, Purpura Fulminans/DIC • Diagnosis: Thrombocytopenia, schistocytes from hemolysis • fibrinogen, PT,PTT, D-DIMER, FDP’s • Clue: the lower the fibrinogen the worse the prognosis • Treatment: ICU, Immediate Heme consult, ABX, FFP, Vit K, platelets, cryoppt, trans rbc’s • AND HEPARIN! • M&M: varies 4 2/3/2014 Case #7 Pemphigus Vulgaris • 58 y.o. female via EMS complaining of painful swallowing and rash • PMHx: HTN, myasthenia gravis • Vitals: BP 100/70 HR 110, RR 20, T 99.4° • PE: Toxic, mucocutaneous blistering • NOT Bullous pemphigoid because of the oral lesions. BP occurs in older patients and has a better prognosis than PV Pemphigus Vulgaris Case #8 • Clue:+Nikolsky’s, Bullae can coalesce resulting in sloughing • Diagnosis: Clinical and biopsy • Etiology: chronic autoimmune disorder, antibodies directed against keratinocytes causing the separation of the epidermis, triggers- stress, drugs (captopril, rifampin) • • • • 40 y.o. male with leg pain, weakness, vomiting PMHx: HTN, IDDM, arthritis Vitals: BP 95/55, HR 120, RR 24, T 102° PE: Toxic, dry MM, hemorrhagic bullae • Treatment: wound care prevent infection and steroids, immunosuppressant Rx by Rheum • Mortality: 90%without steroids, 5-15% with Necrotizing Fasciitis • Etiology: Group A β-hemolytic Strep most common, however can be polymicrobial and need to cover anaerobes, Vibrio vulnificus • Risk patients: IVDA, DM, AIDS, Alcoholics, any immunocompromised • Clue: pain out of proportion for cellulitis, crepitance, rapidly spreading Necrotizing Fasciitis • Treatment: Surgical debridement, Broad spectrum ABX, possibly HBO • Mortality: depends on the organism and patient population, upwards of 75%, but improved with HBO used after surgery • Diagnosis: plain film may show SubQ air, blood cultures 5 2/3/2014 Case #9 • 22 y.o. male with skin peeling for 2 days and complaint of weakness • PMHx: recent cellulitis tx with TMP-SMX • SocHx: no travel • Vitals: BP 96/50 HR 120 RR 20 T 99.2° • PE: Toxic, dry MM, shedding skin TEN-Scorten Score Toxic Epidermal Necrolysis • Definition: >30% BSA detachment, extreme of Steven Johnson’s • Symptoms: fever, eye irritation, sore throat • Signs: mucosal lesions, +Nikolsky’s • Etiology: Drug reaction from Sulfa • Severity rated via Scorten score done on presentation and then 3 days later • RISK patients: HIV poly pharm Toxic Epidermal Necrolysis • Treatment: ICU-Burn unit, wound care • AVOID Sulfadiazine burn cream • Ophthalmology consult-adhesions • Careful Fluid/Electrolyte management • IVIG-not FDA approved • Controversy over steroids- successful in India Case #10 • • • • 65 y.o. male with oral lesions and rash PMHx: recent sinus infection MEDS: TMP-SMX Vitals: BP 115/79 HR 124 RR 24 T °101.7 Stevens Johnson Syndrome • Etiology Drug Reaction is most common-SULFA 50% idiopathic • Clue: Coalescing target lesions, mucosal lesions, <10% skin slough • Treatment: STOP the precipitant, and treat any other underlying cause, Fluid resuscitation and electrolyte monitoring, comfort measures 6 2/3/2014 Case #11 • 10 month old male with fever, decreased oral intake and peeling rash • PMHx: neg • Immun: UTD • Vitals: HR 130, RR 18 T 102.4° • PE: diffuse erythematous rash, which keeps peeling Case #12 • • • • • 25 y.o. female with fever and red rash PMHx: neg Meds: none LMP: currently Vitals: BP 90/60 HR 120 RR 20 T 101.4° Toxic Shock Syndrome • Treatment: Remove the source, and give Clindamycin plus another anti-staph ABX, fluid resuscitation, admit to ICU and possible IVIG • Morbidity: Multi-organ failure Staphylococcal Scalded Skin Syndrome • Children <5 yr • Symptoms: Starts as ‘scarlatiniform’ erythematous rash and proceeds to blister and sloughs + Nikolsky’s • Treatment: ABX against staph, wound care, fluid management • Mortality: <5% • Clue: no oral lesions Toxic Shock Syndrome • Classic association with tampons-staph infection • Etiology: Toxin mediated • Change in susceptibility antigenic shift in the population now most have developed resistance • Clue: Not always tampons, any source of staph or strep can do it Acknowledgement to Heather M. Murphy-Lavoie, MD • Petechiae and fever should get your attention • Palpable petechiae means vasculitis • Non-palpable petechiae most often due to thrombocytopenia • Hemorrhagic bullae are BAD! • Diffuse erythema with associated fever and +Nikolsky’s is ominous • Bullous rash and mucosal lesions steroids are likely indicated 7 2/3/2014 Image Challenge Image Challenge Q What is the diagnosis in this patient who had abdominal pain? : Answer: 2. Henoch-Schönlein purpura Q What is the diagnosis in this patient who had abdominal pain? : 1. Familial Mediterranean fever 2. Henoch-Schönlein purpura 3. Meningococcemia 4. Polyarteritis nodosa 5. Rocky Mountain spotted fever Skin biopsies revealed leukocytoclastic vasculitis with IgA deposition, which is consistent with IgA vasculitis (Henoch-Schönlein purpura). The patient was treated with high-dose glucocorticoids including pulse therapy, which were tapered successfully after cyclosporine was added to the treatment. Henoch-Schönlein purpura, a systemic smallvessel vasculitis, causes palpable purpura in the lower extremities, abdominal pain, arthralgia, and glomerulonephritis. 8