LIFE-THREATENING DERMATOSES DERMATOSES Patricia Treadwell, M.D. - - PowerPoint PPT Presentation

LIFE-THREATENING DERMATOSES DERMATOSES

Patricia Treadwell, M.D. Professor of Pediatrics Professor of Pediatrics IU School of Medicine UK H lth C UK Health Care

Faculty Disclosure Faculty Disclosure

Novartis PI for research study Novartis- PI for research study Eli Lilly & Co- spouse has stocks I do intend to discuss an

unapproved/investigative use of FDA approved products in my presentation. pp p y p

Practice Gap Practice Gap

Cutaneous findings are sometimes the first Cutaneous findings are sometimes the first

clue to a life-threatening disorder. Practitioners are not always cognizant of Practitioners are not always cognizant of the diseases associated with the cutaneous findings and proper diagnosis may be findings and proper diagnosis may be delayed.

Objective Objective

This presentation will highlight the This presentation will highlight the

cutaneous findings in staphylococcal scalded skin syndrome toxic shock scalded skin syndrome, toxic shock syndrome, meningococcemia, RMSF, Steven’s Johnson Syndrome and Kawasaki Steven s Johnson Syndrome and Kawasaki

• disease. Recognition of the findings will

allow for prompt diagnosis allow for prompt diagnosis.

Expected Outcome Expected Outcome

The attendees should be able to recognize The attendees should be able to recognize

skin changes in these disorders and appropriately recommend further work up appropriately recommend further work-up and treatment following this session. Patient outcomes will be improved through Patient outcomes will be improved through the acquisition of this knowledge.

STAPHYLOCOCCAL SCALDED SKIN SYNDROME SCALDED SKIN SYNDROME

Exfoliatin toxin Exfoliatin toxin Colonization with S.aureus usually phage

II type II

Primarily children under 5 years of age Renal disease contributes to poor clearance

• f the toxin

SSSS CLINICAL FINDINGS CLINICAL FINDINGS

Generalized erythema with flexural Generalized erythema with flexural

accentuation Ski d

Skin tenderness Flaccid bullae in the intertriginous areas Exfoliation Positive Nikolsky’s sign Positive Nikolsky s sign Later desquamation

SSSS THERAPY THERAPY

Maintain fluid status Maintain fluid status Prevent secondary infection Systemic anti-staphylococcal antibiotic

REFERENCES REFERENCES

Blyth M et al: Severe Stapylococcal Blyth M, et al: Severe Stapylococcal

Scalded Skin Syndrome in Children. Burns 2008;34:98 103 2008;34:98-103.

Chang P, et al: Picture of the Month.

S h l l S ld d Ski S d Staphylococcal Scalded Skin Syndrome. Arch Pediatr Adolesc Med 2008;162:1189- 1190 1190.

REFERENCES REFERENCES

Norbury WB et al: Neonate Twin with Norbury WB, et al: Neonate Twin with

Staphylococcal Scalded Skin Syndrome from a Renal Source Pediatr Crit Care from a Renal Source. Pediatr Crit Care Med 2010;11:e20-23. P l NN l S h l l S ld d

Patel NN, et al: Staphylococcal Scalded

Skin Syndrome. Am J Med 2010;123:505- 507 507.

TOXIC SHOCK SYNDROME TOXIC SHOCK SYNDROME

Toxic shock syndrome toxin TSST 1 Toxic shock syndrome toxin TSST-1 Staphylococcal enterotoxins Streptococcal toxin Other toxins

TOXIC SHOCK SYNDROME CASE DEFINITION CASE DEFINITION

Fever Fever Erythema Desquamation, 1-2 weeks after the onset of

the illness, particularly of the palms and soles

Hypotension (systolic BP <90 for adults

yp ( y and <5th percentile for age for children <16 years of age, or orthostatic syncope) y g , y p )

TOXIC SHOCK SYNDROME- CASE DEFINITION (cont) CASE DEFINITION (cont)

Involvement of 3 or more of the following: Involvement of 3 or more of the following:

Gastrointestinal (vomiting or diarrhea M l ( l i hi h CK) Muscular (severe myalgia or high CK) Mucous membrane hyperemia Renal (sterile pyuria , high BUN or CR) Hepatic (high bili, SGOT< or SGPT) Hematologic (low platelets) CNS (disorientation)

TOXIC SHOCK SYNDROME TOXIC SHOCK SYNDROME

Cutaneous findings Cutaneous findings

• erythema

conjunctival injection

• conjunctival injection
• necrolysis

multiple pustules

• multiple pustules
• desquamation

TOXIC SHOCK SYNDROME- TREATMENT TREATMENT

Supportive therapy including maintaining Supportive therapy-including maintaining

fluid status and use of vasoactive agents as necessary necessary

Adequate drainage of suppurative sites Anti-staphylococcal antibiotics

REFERENCES REFERENCES

Alwattar BJ et al: Streptococcal Toxic Alwattar BJ, et al: Streptococcal Toxic

Shock Syndrome Presenting as Septic Knee Arthritis in a 5 year old Child J Pediatr Arthritis in a 5-year-old Child. J Pediatr Orthop 2008;28:124-127. B k DR l MRSA S h l l

Berk DR, et al: MRSA, Staphylococcal

Skin Syndrome, and Other Cutaneous B i l E i P di A Bacterial Emergencies. Pediatr Ann 2010;39:627-633.

REFERENCES REFERENCES

Chan KH et al: Toxic Shock Syndrome and Chan KH, et al: Toxic Shock Syndrome and

Rhinosinusitis in Children. Arch Otolaryngol Head Neck Surg Otolaryngol Head Neck Surg 2009;135:538-542. T dd JK T i Sh k S d E l i

Todd JK: Toxic Shock Syndrome-Evolution

• f an Emerging Disease. Adv Exp Med Biol

2011 697 175 181 2011;697:175-181.

MENINGOCCEMIA MENINGOCCEMIA

Patients present with fever myalgias Patients present with fever, myalgias

and malaise

Sometimes may see meningismus Skin lesions - macules, petechiae, and

purpuric lesions with jagged edges

Profound hypotension and shock can

• ccur with overwhelming infections
• ccur with overwhelming infections

MENINGOCCEMIA MENINGOCCEMIA

DIC may develop DIC may develop Complications of DIC include Complications of DIC include

thromboses or gangrene

MENINGOCCEMIA - TREATMENT TREATMENT

Isolation Isolation Supportive therapy including fluids and

vasoactive agents as necessary

Systemic penicillin Systemic penicillin Cefotaxime and ceftriaxone are alternatives If patient has anaphylactoid-type penicillin

reaction may use chloramphenicol reaction, may use chloramphenicol

MENINGOCCEMIA MENINGOCCEMIA

Evaluate need for treatment of household Evaluate need for treatment of household

members and close contacts

REFERENCES REFERENCES

Agarwal MP et al: Clinical Images: Purpura Agarwal MP,et al: Clinical Images: Purpura

Fulminans caused by Meningococcemia. CMAJ 2010;182:E18 CMAJ 2010;182:E18.

Klinkhammer MD, et al: Pediatric Myth:

F d P hi CJEM 2008 10 479 Fever and Petechiae. CJEM 2008;10:479- 482.

ROCKY MOUNTAIN SPOTTED FEVER SPOTTED FEVER

Ca sed b Ri k tt i

i k tt ii

Caused by Rickettsia rickettsii Typically history of tick exposure Incubation 2-14 days

ROCKY MOUNTAIN SPOTTED FEVER SPOTTED FEVER

Fever Fever Severe headache Confusion Nausea and vomiting Photophobia

ROCKY MOUNTAIN SPOTTED FEVER exanthem SPOTTED FEVER - exanthem

Exanthem present in 90 % patients Exanthem present in 90 % patients Erythematous macules and papules initially Later, petechial or purpuric lesions Lesions occur initially on the palms and

y p soles, then spread centrally

ROCKY MOUNTAIN SPOTTED FEVER SPOTTED FEVER

Supportive therapy may be necessary Supportive therapy may be necessary Doxycycline Chloramphenicol

ROCKY MOUNTAIN SPOTTED FEVER References FEVER-References

Davis RF et al: Recognition and Davis RF, et al: Recognition and

Management of Common Ectoparasitic Diseases in Travelers Am J Clin Dermatol Diseases in Travelers. Am J Clin Dermatol 2009;10:1-8. Mi i TD l M i R k

Miniear TD, et al: Managing Rocky

Mountain Spotted Fever. Expert Rev Anti I f Th 2009 7 1131 1137 Infect Ther 2009;7:1131-1137.

REFERENCES REFERENCES

Usatine RP et al: Dermatologic Usatine RP, et al: Dermatologic

• Emergencies. Am Fam Physician

2010;82:773 480 2010;82:773-480.

HENOCH-SCHONLEIN PURPURA PURPURA

A hypersensitivity reaction that occurs A hypersensitivity reaction that occurs

typically following an infection

Infection most often streptococcal or viral

HENOCH-SCHONLEIN PURPURA PURPURA

Palpable purpura Palpable purpura Petechial lesions Acral distribution Vesicular or bullous lesions Infants tend to have more involvement

• f the face and scalp with accompanying

edema

HENOCH-SCHONLEIN PURPURA PURPURA

Gastrointestinal abnormalities Gastrointestinal abnormalities –

including abdominal pain, vomiting and bloody stools and bloody stools

Arthralgias and/or arthritis Arthralgias and/or arthritis Renal abnormalities

HENOCH-SCHONLEIN PURPURA PURPURA

Immune complex formation Immune complex formation Histology shows leukocytoclastic Histology shows leukocytoclastic

vasculitis with extravasated RBC’s

Immunofluorescence shows IGA

HENOCH-SCHONLEIN PURPURA PURPURA

Treatment Treatment

Elevation Anti inflammatory medication Anti-inflammatory medication Corticosteroid controversy

Renal status should be monitored

if h i id f l di if there is evidence of renal disease in the acute stages

REFERENCES REFERENCES

Nobile S et al: Herpes Zoster Infection Nobile S, et al: Herpes Zoster Infection

Followed by Henoch-Schonlein Purpura in a Girl Receiving Inflixamab for Ulcerative a Girl Receiving Inflixamab for Ulcerative

• Colitis. J Clin Rheumatol 2009;15:101.

P kh T l V li i I A

Pankhurst T, et al: Vasculitic IgA

Nephropathy : Prognosis and Outcome. N h Cli P 2009 112 16 24 Nephron Clin Pract 2009;112:c16-24.

REFERENCES REFERENCES

Rashtak S et al: Skin Involvement in Rashtak S, et al: Skin Involvement in

Systemic Autoimmune Diseases. Curr Dir Autoimmun 2008;10:344 358 Autoimmun 2008;10:344-358.

Zhang Y, et al: Sibling Cases of Henoch-

S h l i P i T F ili d Schonlein Purpura in Two Families and Review of Literature. Pediatr Dermatol 2008 25 393 395 2008;25:393-395.

STEVENS JOHNSON SYNDROME SYNDROME

Stevens Johnson Syndrome and Toxic Stevens-Johnson Syndrome and Toxic

Epidermal Necrolysis (TEN) considered part of a spectrum part of a spectrum

Hypersensitivity (allergic) reaction to

di i i f i medications or infectious agent

Toxic injury to keratinocytes and mucosal

epithelial cells

STEVENS-JOHNSON SYNDROME ETIOLOGY SYNDROME-ETIOLOGY

Sulfa drugs Sulfa drugs Anti-convulsants Non steroidal anti-inflammatory agents Other drugs

g

Mycoplasma

STEVENS-JOHNSON SYNDROME SYNDROME

 Clinical findings  Clinical findings

Rapid onset of symptoms Mucous membrane erosions

• Oral
• Ocular
• Genital

Subepidermal bullous formation ik l k i Nikolsky’s sign Can see atypical target lesions

STEVENS-JOHNSON SYNDROME OTHER FINDINGS SYNDROME –OTHER FINDINGS

Fever Fever Malaise Headache Respiratory distress

p y

GI Involvement-dysphagia, malnutrition,

abdominal pain diarrhea abdominal pain, diarrhea

STEVENS-JOHNSON SYNDROME DIAGNOSIS SYNDROME -DIAGNOSIS

A frozen section of the blister roof will A frozen section of the blister roof will

show several layers of cell

STEVENS-JOHNSON SYNDROME SYNDROME

May be a genetic predisposition May be a genetic predisposition Family history pertinent Avoid triggers

STEVENS-JOHNSON SYNDROME TREATMENT SYNDROME -TREATMENT

Discontinue medication Discontinue medication Supportive care ?Burn center Ophthalmology consult if eye involvement

p gy y

STEVENS-JOHNSON SYNDROME TREATMENT SYNDROME -TREATMENT

Corticosteroid controversy Corticosteroid controversy IVIG Cyclosporine

REFERENCES REFERENCES

Hazin R et al: Stevens Johnson Syndrome: Hazin R, et al: Stevens-Johnson Syndrome:

Pathogenesis, Diagnosis, and Management. Ann Med 2008;40:129 138 Ann Med 2008;40:129-138.

Mayes T, et al: Energy Requirements of

P di i P i i h S J h Pediatric Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. N Cli P 2008 23 547 550 Nutr Clin Pract 2008;23:547-550.

REFERENCES REFERENCES

Reese D et al: Cyclosporine for SJS/TEN: Reese D, et al: Cyclosporine for SJS/TEN:

A Case Series and Review of the Literature. Cutis 2011;87: 24 29 Cutis 2011;87: 24-29.

Treat J: Stevens-Johnson Syndrome and

T i E id l N l i P di A Toxic Epidermal Necrolysis. Pediatr Ann 2010;39:667-674.

KAWASAKI DISEASE KAWASAKI DISEASE

Searching many for etiologic infectious Searching many for etiologic infectious

agent R l f i

Role of superantigens

KAWASAKI DISEASE KAWASAKI DISEASE

Evidence for superantigens Evidence for superantigens

activation of T/B cells

• activation of T/B cells

induction of immunologic tolerance

• induction of immunologic tolerance

KAWASAKI DISEASE CASE DEFINITION CASE DEFINITION

Fever persisting 5 days or more Fever persisting 5 days or more Erythema and swelling of the palms and

l l d i soles- later desquamation

Polymorphous exanthema Conjunctival injection Erythema of the lips and oral pharynx Erythema of the lips and oral pharynx,

strawberry tongue

lymphadenopathy lymphadenopathy

KAWASAKI DISEASE KAWASAKI DISEASE

CUTANEOUS FINDINGS CUTANEOUS FINDINGS

Conjunctival injection Erythema of the lips and oral pharynx Erythema of the lips and oral pharynx Strawberry tongue Fissures of the lips Fissures of the lips Swelling of the hands and feet Perineal desquamation Perineal desquamation Desquamation of the hands and feet

KAWASAKI DISEASE KAWASAKI DISEASE

Cardiac consultation Cardiac consultation

Echocardiogram IVIG

IVIG

REFERENCES REFERENCES

Athappan G et al: Corticosteroid Therapy Athappan G, et al: Corticosteroid Therapy

for Primary Treatment of Kawasaki Disease Weight of Evidence: A Meta Disease-Weight of Evidence: A Meta- analysis and Systemic Review of the Literature Cardiovacs J Afr 2009;20:233

• Literature. Cardiovacs J Afr 2009;20:233-

236. H l K ki Di f

Hua w, et al: Kawasaki Disease after

• Vaccination. Pediatr Infect Dis J

2009 28 943 947 2009;28:943-947.

REFERENCES REFERENCES

Macias ES et al: Superantigens in Macias ES, et al: Superantigens in

• Dermatology. J Am Acad Dermatol

2011;64:455 472 2011;64:455-472.

Rowley AH, et al: Pathogenesis and

M f K ki Di E Management of Kawasaki Disease. Expert Rev Anti Infect Ther 2010;8:197-203.