LIFE-THREATENING DERMATOSES
Patricia Treadwell, M.D. Professor of Pediatrics IU School of Medicine UK Hea lth Care Faculty Disclosure
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 m y p resentation. Practice Gap
Cutaneous findings are sometimes the first clue to a life-threatening disorder. Practitioners are not always cognizant of the diseases associated with the cutaneous findings and proper diagnosis may be delayed. Objective
This presentation will highlight the cutaneous findings in staphylococcal scalded skin syndrome , toxic shock syndrome, meningococcemia, RMSF, Steven’s Johnson Syndrome and Kawasaki disease. Recognition of the findings will allow for prompt diagnosis . Expected Outcome
The attendees should be able to recognize skin changes in these disorders and appropriately recommend further work-up and treatment following this session. Patient outcomes will be improved through the acquisition of this knowledge. STAPHYLOCOCCAL SCALDED SKIN SYNDROME
Exfoliatin toxin Colonization with S.aureus usually phage type II Primarily children under 5 years of age Renal disease contributes to poor clearance of the toxin SSSS CLINICAL FINDINGS
Generalized erythema with flexural accentuation Skin tend erness Flaccid bullae in the intertriginous areas Exfoliation Positive Nikolsky ’ sssign sign Later desquamation SSSS THERAPY
Maintain fluid status Prevent secondary infection Systemic anti-staphylococcal antibiotic REFERENCES
Blyth M , et al: Severe Stapylococcal Scalded Skin Syndrome in Children. Burns 2008;34:98-103. Chang P, et al: Picture of the Month. ShlStaphylococcal lSlddSkiSd Scalded Skin Syndrome. Arch Pediatr Adolesc Med 2008;162:1189- 1190. REFERENCES
Norbury WB, et al: Neonate Twin with Staphylococcal Scalded Skin Syndrome from a Renal Source . Pediatr Crit Care Med 2010;11:e20-23. PlNNlShlPatel NN, et al: Staphylococca lSlddl Scalded Skin Syndrome. Am J Med 2010;123:505- 507. TOXIC SHOCK SYNDROME
Toxic shock syndrome toxin TSST-1 Staphylococcal enterotoxins Streptococcal toxin Other toxins TOXIC SHOCK SYNDROME CASE DEFINITION
Fever Erythema Desquamation, 1-2 weeks after the onset of the illness, particularly of the palms and soles Hypotension (s ystolic BP <90 for adults and <5th percentile for age for children <16 yyg,yp)ears of age, or orthostatic syncope) TOXIC SHOCK SYNDROME- CASE DEFINITION (cont)
Involvement of 3 or more of the following : Gastrointestinal (vomiting or diarrhea MlMuscular (severe myalilgiaor 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
Cutaneous findings -erythema -conjunctival injection -necrolysis -multiple pustules -desquamation TOXIC SHOCK SYNDROME- TREATMENT
Supportive therapy -including maintaining fluid status and use of vasoactive agents as necessary Adequate drainage of suppurative sites Anti-staphylococcal antibiotics REFERENCES
Alwattar BJ , et al: Streptococcal Toxic Shock Syndrome Presenting as Septic Knee Arthritis in a 5 -year-old Child. J Pediatr Orthop 2008;28:124-127. BkDRBerk DR, et al lMRSAS: MRSA, Stap hlhylococca l Skin Syndrome, and Other Cutaneous BilEiBacterial Emergencies. PdiPediatr A nn 2010;39:627-633. REFERENCES
Chan KH, et al: Toxic Shock Syndrome and Rhinosinusitis in Children. Arch Otolaryngol Head Neck Surg 2009;135:538-542. TddJKTTodd JK: Tox ic Sh ShkSock Syn drome-EliEvolution of an Emerging Disease. Adv Exp Med Biol 2011; 697: 175-181. MENINGOCCEMIA
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 occur with overwhelming infections MENINGOCCEMIA
DIC may develop
Complications of DIC include thromboses or gangrene MENINGOCCEMIA - TREATMENT
Isolation
Supportive therapy including fluids and vasoactive agents as necessary
Systemic penicillin
Cefotaxime and ceftriaxone are alternatives
If patient has anaphylactoid-type penicillin reaction, may use chloramphenicol MENINGOCCEMIA
Evaluate need for treatment of household members and close contacts REFERENCES
Agarwal MP, et al: Clinical Images: Purpura Fulminans caused by Meningococcemia. CMAJ 2010;182:E18. Klinkhammer MD, et al: Pediatric Myth: FdPhiFever and Petechiae. CJEM 2008; 10: 479- 482. ROCKY MOUNTAIN SPOTTED FEVER
Cau sed b y Rick ett si a ri ck ett sii Typically history of tick exposure Incubation 2-14 days ROCKY MOUNTAIN SPOTTED FEVER
Fever Severe headache Confusion Nausea and vomiting Photophobia ROCKY MOUNTAIN SPOTTED FEVER - exanthem
Exanthem present in 90 % patients Erythematous macules and papules initially Later, petechial or purpuric lesions Lesions occur initiallyyp on the palms and soles, then spread centrally ROCKY MOUNTAIN SPOTTED FEVER
Supportive therapy may be necessary Doxycycline Chloramphenicol ROCKY MOUNTAIN SPOTTED FEVER-References
Davis RF , et al: Recognition and Management of Common Ectoparasitic Diseases in Travelers . Am J Clin Dermatol 2009;10:1-8. Min iear TD , et al : M anagi ing R ocky k Mountain Spotted Fever. Expert Rev Anti IfInfect Th er 2009; 7: 1131-1137. REFERENCES
Usatine RP , et al: Dermatologic Emergencies. Am Fam Physician 2010;82:773-480. HENOCH-SCHONLEIN PURPURA
A hypersensitivity reaction that occurs typically following an infection
Infection most often streptococcal or viral HENOCH-SCHONLEIN PURPURA
Palpable purpura
Petechial lesions
Acral distribution
Vesicular or bullous lesions
Infants tend to have more involvement of the face and scalp with accompanying edema HENOCH-SCHONLEIN PURPURA
Gastrointestinal abnormalities – including abdominal pain, vomiting and bloody stools
Arthralgias and/or arthritis
Renal abnormalities HENOCH-SCHONLEIN PURPURA
Immune complex formation
Histology shows leukocytoclastic vasculitis with extravasated RBC’s
Immunofluorescence shows IGA HENOCH-SCHONLEIN PURPURA
Treatment Elevation Anti-inflammatory medication Corticosteroid controversy
Renal status should be monitored if th ere i s evid ence of renal di sease in the acute stages REFERENCES
Nobile S , et al: Herpes Zoster Infection Followed by Henoch-Schonlein Purpura in a Girl Receiving Inflixamab for Ulcerative Colitis. J Clin Rheumatol 2009;15:101. PkhTPankhurst T, et a lVl: Vascu liiIAlitic IgA Nephropathy : Prognosis and Outcome. NhNephron CliPClin Pract 2009; 112:c 16-24. REFERENCES
Rashtak S , et al: Skin Involvement in Systemic Autoimmune Diseases. Curr Dir Autoimmun 2008;10:344-358. Zhang Y, et al: Sibling Cases of Henoch- Sc hon le in P urpura in T wo F amili es and Review of Literature. Pediatr Dermatol 2008; 25: 393-395. STEVENS JOHNSON SYNDROME
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis (TEN) considered part of a spectrum Hypersensitivity (allergic) reaction to medicati ons or i nf ecti ous agent Toxic injury to keratinocytes and mucosal epithelial cells STEVENS-JOHNSON SYNDROME-ETIOLOGY
Sulfa drugs Anti-convulsants Non steroidal anti-inflammatory agents Other drugs Mycoplasma STEVENS-JOHNSON SYNDROME
Clinical findings Rapid onset of symptoms Mucous membrane erosions -Oral -Ocular -Genital Subepidermal bullous formation Nikikol sk y’s si gn Can see atypical target lesions STEVENS-JOHNSON SYNDROME –OTHER FINDINGS
Fever Malaise Headache Resppyiratory distress GI Involvement-dysphagia, malnutrition, abdominal pain, diarrhea STEVENS-JOHNSON SYNDROME -DIAGNOSIS
A frozen section of the blister roof will show several layers of cell STEVENS-JOHNSON SYNDROME
May be a genetic predisposition Family history pertinent Avoid triggers STEVENS-JOHNSON SYNDROME -TREATMENT
Discontinue medication Supportive care ?Burn center Oppgyyhthalmology consult if eye involvement STEVENS-JOHNSON SYNDROME -TREATMENT
Corticosteroid controversy IVIG Cyclosporine REFERENCES
Hazin R , et al: Stevens -Johnson Syndrome: Pathogenesis, Diagnosis, and Management. Ann Med 2008;40:129-138. Mayes T, et al: Energy Requirements of PdiPediatr ic P at ients wi ihSth Stevens-JhJohnson Syndrome and Toxic Epidermal Necrolysis. NCliPNutr Clin Pract 2008; 23: 547-550. REFERENCES
Reese D, et al: Cyclosporine for SJS/TEN: A Case Series and Review of the Literature. Cutis 2011;87: 24 -29. Treat J: Stevens-Johnson Syndrome and TiEidlNliToxic Epidermal Necrolysis. PdiPediatr A nn 2010;39:667-674. KAWASAKI DISEASE
Searching many for etiologic infectious agent RlRole of superantig iens KAWASAKI DISEASE
Evidence for superantigens
-activation of T/B cells
-induction of immunologic tolerance KAWASAKI DISEASE CASE DEFINITION
Fever persisting 5 days or more Erythema and swelling of the palms and soles- ldlater desquamati on Polymorphous exanthema Conjunctival injection Erythema of the lips and oral pharynx , strawberry tongue lymphadenopathy KAWASAKI DISEASE
CUTANEOUS FINDINGS Conjunctival injection Erythema of the lips and oral pharynx Strawberry tongue Fissures of the lips Swelling of the hands and feet Perineal desquamation Desquamation of the hands and feet KAWASAKI DISEASE
Cardiac consultation Echocardiogram IVIG REFERENCES
Athappan G, et al: Corticosteroid Therapy for Primary Treatment of Kawasaki Disease-Weight of Evidence: A Meta - analysis and Systemic Review of the Literature. Cardiovacs J Afr 2009;20:233- 236. HlKkiDifHua w, et al: Kawasaki Disease after Vaccination. Pediatr Infect Dis J 2009; 28: 943-947. REFERENCES
Macias ES , et al: Superantigens in Dermatology. J Am Acad Dermatol 2011;64:455-472. Rowley AH, et al: Pathogenesis and MfKkiDiManagement of Kawasaki Disease. Expert Rev Anti Infect Ther 2010;8:197-203.