GOOD MORNING! AUGUST 5, 2014

Transcription

1 GOOD MORNING! AUGUST 5, 2014

2 PREP QUESTION During the health supervision visit of a term newborn boy, his mother relates that a cousins child died at age 4 months from sudden infant death syndrome. She asks what she can do to prevent such an occurrence in her son. Of the following, the single most important preventative measure is to: A. Avoid use of a pacifier B. Co-sleep in parental bed for close observation C. Ensure side-sleeping position to prevent aspiration D. Ensure supine sleep positioning E. Use home apnea and bradycardia monitoring

3 LET S HEAR FROM AND SEE THE PATIENT. WHAT ARE YOU THINKING?

4 WHAT S GOING ON?! Phototoxic Eruptions Bullous Impetigo Secondary Syphilis Stevens Johnson Syndrome Kawasaki Toxic Shock Syndrome Toxin Mediated Perineal Erythema Erythema Multiforme Serum Sickness Erythematous Drug Eruption Toxic Epidermal Necrolysis Urticarial Vasculitis Viral Exanthems Annular Urticarial Burns Pemphigus Vulgaris Herpetic Gingivostomatitis Aphthous Stomatitis Staph Scalded Skin Syndrome Linear Iga Bullous Dermatosis

5 A BLISTERING FAST RASH REVIEW (SAY THAT THREE TIMES FAST!)

6 WHAT S GOING ON?! Phototoxic Eruptions Bullous Impetigo Secondary Syphilis Stevens Johnson Syndrome Kawasaki Toxic Shock Syndrome Toxin Mediated Perineal Erythema Erythema Multiforme Serum Sickness Erythematous Drug Eruption Toxic Epidermal Necrolysis Urticarial Vasculitis Viral Exanthems Annular Urticarial Burns Pemphigus Vulgaris Herpetic Gingivostomatitis Aphthous Stomatitis Staph Scalded Skin Syndrome Linear Iga Bullous Dermatosis

7 STEVENS JOHNSON SYNDROME Immunologically mediated mucocutaneous reaction involving targetoid lesions and erosive mucositis Circulating immune complexes and pro-apoptotic factors Most commonly associated with medications: NSAIDs, PCN-related antibiotics, Allopurinol, Sulfonamides, Anticonvulsants (Phenobarbital, Phenytoin, Carbamazepine) Drug exposure precedes symptom onset by 1-3 weeks Re-exposure to meds may result in symptoms within 48 hours 2 commonly associated infections: Mycoplasma and HSV

8 STEVENS JOHNSON SYNDROME Lesion: atypical and targetoid Start as erythematous macules that develop bullous centers Central area often violaceous or blistered, surrounded by donut-shaped elevated area of pallor and peripheral rim of erythema May concentrate on torso + Nikolsky sign < 20% BSA

9 STEVENS JOHNSON SYNDROME Mucositis: Oropharyngeal, Conjunctival, Urethral, Rectal Includes 2 or more sites These areas are at risk for: scarring, adhesions, strictures, and blindness if conjunctival involvement Other symptoms may precede skin findings (usually by 1-3 days) Fever Influenza-like symptoms Skin tenderness Photophobia and conjunctival itching or burning

10 If you get labs: STEVENS JOHNSON SYNDROME Increased WBC, ESR Transaminitis Urogenital involvement Hematuria Proteinuria Pyuria Skin biopsy: Full-thickness epidermal necrosis and detachment with only slightly altered underlying dermis (aka at the dermalepidermal junction) Perivascular leukocytic infiltrates

11 STEVENS JOHNSON SYNDROME Diagnosis is CLINICAL BUT biopsy may help Treatment: Stop offending agent or treat etiology (ie Mycoplasma if PNA) Hospitalization for Fluid & Electrolyte management and pain control Diligent wound care and topical antibiotics (bacitracin, silver sulfadiazine) Consultation of subspecialists for any mucous membrane involvement IVIG?

12 WHAT S GOING ON?! Phototoxic Eruptions Bullous Impetigo Secondary Syphilis Stevens Johnson Syndrome Kawasaki Toxic Shock Syndrome Toxin Mediated Perineal Erythema Erythema Multiforme Serum Sickness Erythematous Drug Eruption Toxic Epidermal Necrolysis Urticarial Vasculitis Viral Exanthems Annular Urticarial Burns Pemphigus Vulgaris Herpetic Gingivostomatitis Aphthous Stomatitis Staph Scalded Skin Syndrome Linear Iga Bullous Dermatosis

13 STAPHYLOCOCCAL SCALED SKIN SYNDROME Bacterial toxin-mediated exfoliative eruption characterized by areas of red skin and superficial desquamation, resembling skin that has been scalded In children who lack neutralizing antibodies to staph toxins Seen in those <5 years of age, preterm, or older children and adults with renal impairment or immunosuppression Exfoliative toxins produced by staph act as proteases and bind to desmoglein-1 in epidermis to cause superficial separation

14 STAPHYLOCOCCAL SCALED SKIN SYNDROME Low grade fever, malaise, irritability precede rash Scarlatiniform rash: starts in intertriginous zones and perioral areas before generalizing Mild to moderately tender skin Nikolsky sign Denuded skin is moist red and resembles burns Involved areas desquamate without scarring days +/- conjuncitivis Hyperemic mucous membranes without oral lesions

15 STAPHYLOCOCCAL SCALED SKIN SYNDROME Identifying potential foci of infection is important! NP, conjunctivae, blood, urine, umbilicus, abnormal skin Toxin-induced blisters generally culture-negative Biopsy (if needed to differ from TEN): superficial split within granular layer compared to deeper, full-thickness epidermal involvement Supportive care Parenteral antibiotics for extensive skin involvement or systemic disease Oxacillin, Nafacillin, Clindamycin for 7-10 days Topical antibiotics and steroids are not indicated Excellent prognosis

16 WHAT S GOING ON?! Phototoxic Eruptions Bullous Impetigo Secondary Syphilis Stevens Johnson Syndrome Kawasaki Toxic Shock Syndrome Toxin Mediated Perineal Erythema Erythema Multiforme Serum Sickness Erythematous Drug Eruption Toxic Epidermal Necrolysis Urticarial Vasculitis Viral Exanthems Annular Urticarial Burns Pemphigus Vulgaris Herpetic Gingivostomatitis Aphthous Stomatitis Staph Scalded Skin Syndrome Linear Iga Bullous Dermatosis

17 TOXIC EPIDERMAL NECROLYSIS Life-threatening mucocutaneous reaction involving extensive skin sloughing, often in association with mucous membrane erosions. May evolve from pre-existing Stevens Johnson Syndrome Circulating immune complexes and elaboration of circulating propoptotic factors Most common drugs: PCN-related antibiotics, Sulfonamides, and Anticonvulsants (Phenobarbital, Phenytoin, Carbamazepine)

18 TOXIC EPIDERMAL NECROLYSIS Lesion: tender, red areas of skin often on dependent areas (lower back and butt) but can be anywhere May blister or simply slough and show + Nikolsky sign Widespread, involving >20% of BSA Mucositis may be as severe as SJS with similar sequelae Extensive full-thickness denudation of skin clinically resembles burned skin Predisposes patient to sepsis, fluid and electrolyte disturbances, and thermal instability

19 TOXIC EPIDERMAL NECROLYSIS Diagnosis: CLINICAL Biopsy with frozen section: full-thickness epidermal necrosis and subepidermal split Treatment: Stop offending agent or treat etiology Fluid and Electrolyte management in ICU or Burn Unit Diligent wound care and topical antibiotics (bacitracin, silver sulfadiazine) Systemic analgesia Consultation of subspecialists for mucous membrane involvement IVIG?

20 WHAT S GOING ON?! Phototoxic Eruptions Bullous Impetigo Secondary Syphilis Stevens Johnson Syndrome Kawasaki Toxic Shock Syndrome Toxin Mediated Perineal Erythema Erythema Multiforme Serum Sickness Erythematous Drug Eruption Toxic Epidermal Necrolysis Urticarial Vasculitis Viral Exanthems Annular Urticarial Burns Pemphigus Vulgaris Herpetic Gingivostomatitis Aphthous Stomatitis Staph Scalded Skin Syndrome Linear Iga Bullous Dermatosis

21 TOXIC SHOCK SYNDROME Acute streptococcal or staphylococcal exotoxin-mediated infection with perivascular infiltrates resulting in fever, diffuse erythroderma, hypotension and impairment of 3 or more organ systems Associations: foreign body (<50% due to tampons!), primary Staph infection, post-op wound infection, mucous membrane or skin disruption Streptococcal TSS is associated with Varicella, DM, HIV Exotoxins act as superantigens-> massive T cell activation, cytokine release Activation of coagulation cascade and thrombolytic enzymes-> hemolytic anemia and DIC

22 TOXIC SHOCK SYNDROME Fever, rash, hypotension, arthritis, with multi-organ involvement AND clinical course out of proportion to extent of local infection Initial erythroderma: diffuse, macular rash resembling sunburn which can include palms and soles; petechiae and bullae may develop 1-2 weeks after disease onset: pruritic, maculopapular rash, desquamation affects palms and soles Mucosal involvement: Hyperemia of the pharyngeal and GU mucosa, conjunctival-scleral hemorrhage

23 TOXIC SHOCK SYNDROME

24 Systemic symptoms: TOXIC SHOCK SYNDROME Fever (>102), hypotension Non-pitting edema of face and hands (secondary to capillary leak) Diarrhea and vomiting Myalgia Mental status changes Multi-organ failure/dic Lab findings: Blood, throat, CSF cultures typically negative Positive blood culture (more common with GAS) +/- Leukocytosis with a pronounced bandemia Increased CPK, Myoglobinuria/hemoglobinuria

25 TOXIC SHOCK SYNDROME

26 TOXIC SHOCK SYNDROME Treatment Anticipate shock and multisystem organ failure Remove or drain any loculated source of infection Empiric abx- beta-lactamase resistant anti-staph plus a protein-synthesis inhibitor (clindamycin). Strep pyogenes- PCN + Clindamycin Staph aureus- appropriate beta-lactam ABx based on susceptibility + Clindamycin Total course days IVIG? Patients with inaccessible focus or those with continued deterioration (400mg/kg once) Early admin may predispose to recurrence by blunting initial immune response

27 WHAT S GOING ON?! Phototoxic Eruptions Bullous Impetigo Secondary Syphilis Stevens Johnson Syndrome Kawasaki Toxic Shock Syndrome Toxin Mediated Perineal Erythema Erythema Multiforme Serum Sickness Erythematous Drug Eruption Toxic Epidermal Necrolysis Urticarial Vasculitis Viral Exanthems Annular Urticarial Burns Pemphigus Vulgaris Herpetic Gingivostomatitis Aphthous Stomatitis Staph Scalded Skin Syndrome Linear Iga Bullous Dermatosis

28 ERYTHEMA MULTIFORME (MINOR) Immunologically mediated mucocutaneous eruption characterized by targetoid lesions. Oral mucous membranes are commonly affected Older children and young adults Reactive phenomenon in response to multiple etiologies: infectious, medications, inflammatory conditions, environmental agents Infections > Medications Recurrent EM: strongly associated with HSV May occur a few days to a few weeks following an infection

29 ERYTHEMA MULTIFORME (MINOR) Lesion: targetoid and demonstrates triple color phenomenon of a central area that is violaceous and dusky, a surrounding pale ring of edema, and an erythematous peripheral rim Distribution: symmetric, acral predilection (spreads in centripetal manner) involving the arms and legs, palms and soles, with fewer lesions on the torso Typically asymptomatic HSV-associated lesions appear 2-17 days after outbreak

30 ERYTHEMA MULTIFORME (MINOR) TYPICALLY INVOLVES ONLY ONE MUCOUS MEMBRANE ORAL 70% CAN EXTEND TO PHARYNX AND URT GENITAL 25% OCULAR 17%

31 ERYTHEMA MULTIFORME (MINOR) If you get labs: Non-specific: Elevated ESR, WBC Transaminitis If any suspicion: Skin biopsy? HSV (DFA, Viral Cx, PCR) M. Pneumoniae (serology) If diagnosis is in question Epidermal cell apoptosis, basal cell vacuolar degeneration, lymphocytic exocytosis, and dermal lymphocytic infiltrate

32 ERYTHEMA MULTIFORME (MINOR) Diagnosis is CLINICAL Self-limited Lesions appear over 3-5 days and disappear over ~2 weeks Usually no scarring, but may leave area of post-inflammatory hyperpigmentation that can remain for months Requires supportive care Poor oral intake from mucositis may require IV rehydration Antihistamines for pruritus Ophtho consult if eye involvement

33 WHAT WOULD YOU LIKE TO DO? WHAT WE DID Skin biopsy: extensive full thickness epidermal necrosis, very mild inflammation in dermis FINAL DIAGNOSIS? Stevens Johnson Syndrome

34 BOARD SPECS AT A GLANCE Recognize that the spectrum of severity of erythema multiforme ranges from targetoid lesions to stevens-johnson syndrome Recognize the major clinical syndromes of staphylococcus aureus: scalded skin syndrome, toxic shock syndrome Recognize the appearance of staphylococcal scalded skin syndrome, know it is mediated by a toxin Understand the management of a patient with staphylococcal toxic shock syndrome Know that Group A Streptococcus can cause a toxic shock syndrome

35 NOON CONFERENCE: DR HESCOCK INTRO TO QI PROJECTS (STUDENTS OFF)