Dermatology Book

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Staphylococcal Scalded Skin Syndrome

Aka: Staphylococcal Scalded Skin Syndrome, Staphylococcus Scalded Skin Syndrome, Ritters Disease
  1. Epidemiology
    1. Typically affects age <5 years old (and especially <2 years)
    2. Incidence: <0.56 per Million (rare)
  2. Pathophysiology
    1. Group 2 Coagulase Positive Staphylococcus aureus strains
      1. Strains that carry exfoliative toxins A and B (only 5% of S. aureus strains)
      2. Breaks down desmoglein-1 resulting in epidermolysis or Acantholysis (breakage of cell to cell adhesions)
      3. Similar to Pemphigus Vulgaris (which in contrast is immune mediated)
    2. Sources
      1. Initial localized infection
        1. Skin sites (e.g. Diaper Dermatitis, ubilicus, face)
        2. Nn-skin sites (e.g. Otitis Media, Upper Respiratory Infection
      2. Later, hematologic dissemination of Staphylococcus aureus from an infectious source
        1. Contrast with Impetigo which spreads contiguously
  3. Symptoms
    1. Prodrome (initial Upper Respiratory Infection)
      1. Otitis Media
      2. Pharyngitis
      3. Conjunctivitis
    2. Acute phase (initial, prior to Desquamation)
      1. Fever
      2. Malaise
      3. Irritability
      4. Red, painful skin
  4. Signs
    1. Red, scarlatiniform, sandpaper-like rash
      1. Paper-thin skin that desquamates (Exfoliative Dermatitis)
      2. Distribution especially in the flexor creases, perioral area, neck and groin
    2. Large, flaccid Blisters
      1. Positive Nikolsky Sign (on affected skin)
    3. No mucous membrane involvement
  5. Labs
    1. Blood Cultures
      1. Bacteremia is often present in Staphylococcal Scalded Skin Syndrome
    2. Skin biopsy
    3. Exotoxin assay
    4. Avoid skin lesion cultures
      1. Lesions are sterile and will be non-diagnostic
  6. Management
    1. Admit patients with diffuse involvement to ICU or burn unit
    2. Intravenous antibiotics covering Staphylococcus aureus (consider MRSA coverage depending on risk)
      1. Methicillin Sensitive Staphylococcus aureus infections account for 95% of cases (as of 2018)
    3. Systemic Corticosteroids may be used in non-toxic appearing patients
    4. Adjunctive measures in severe cases
      1. Intravenous Immunoglobulin
      2. Plasmapheresis
  7. Course
    1. Resolution in 5-7 days after antibiotics are initiated
  8. Differential Diagnosis
    1. See Bullous Conditions
    2. Steven Johnson Syndrome
    3. Toxic Epidermal Necrolysis
    4. Multisystem Inflammatory Syndrome in Children (MIS-C, Kawasaki Disease)
    5. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS Syndrome)
  9. References
    1. Long (2016) Crit Dec Emerg Med 30(7):3-10
    2. Stewart (2022) Crit Dec Emerg Med 36(1): 16-7
    3. Rare Disease Database, accessed 1/13/2022
      1. https://rarediseases.org/rare-diseases/staphylococcal-scalded-skin-syndrome/

Staphylococcal Scalded Skin Syndrome (C0038165)

Definition (NCI) A blistering skin disorder caused by exfoliative toxins produced by Staphylococcus aureus infection. The toxins cause the formation of bullae and diffuse skin desquamation. The lesions may be localized or generalized, far away from the initial site of infection.
Definition (MSH) A disease of infants due to group 2 phage type 17 staphylococci that produce an epidermolytic exotoxin. Superficial fine vesicles and bullae form and rupture easily, resulting in loss of large sheets of epidermis.
Concepts Disease or Syndrome (T047)
MSH D013206
ICD9 695.81
ICD10 L00
SnomedCT 87758007, 367537006, 200946001
English Disease, Ritter, Ritter Disease, Ritter's Disease, Ritters Disease, Scalded-Skin Syndrome, Staphylococcal, Scalded-Skin Syndromes, Staphylococcal, Staphylococcal Scalded Skin Syndrome, Staphylococcal Scalded-Skin Syndrome, Staphylococcal Scalded-Skin Syndromes, Syndrome, Staphylococcal Scalded-Skin, Syndromes, Staphylococcal Scalded-Skin, Disease, Ritter's, Scalded Skin Syndrome, Staphylococcal, SSSS-Staph scalded skin syndr, Staph scalded skin syndrome, RITTERS DIS, RITTER DIS, SCALDED SKIN SYNDROME STAPH, STAPH SCALDED SKIN SYNDROME, staphylococcal scalded skin syndrome (diagnosis), scalded skin syndrome, scalded skin syndrome (diagnosis), staphylococcal scalded skin syndrome, (Staphylococcal) Scalded skin syndrome, Ritter's-Lyells syndrome, Staphylococcal Scalded Skin Syndrome [Disease/Finding], ritter disease, ritters disease, scalding skin syndrome, scald skin syndrome, staph scalded skin syndrome, ritter's disease, skin scalded syndrome, Dermatitis exfoliativa neonatorum, Ritter's disease, Scalded skin syndrome, Staphylococcal scalded skin syndrome, Toxic epidermal necrolysis, subcorneal type, SSSS - Staphylococcal scalded skin syndrome, Staphylococcal scalded skin syndrome (disorder), Toxic epidermal necrolysis, subcorneal type (disorder), Ritter, SSSS, exfoliation; dermatitis, neonatorum, scalded skin; syndrome, syndrome; scalded skin, Toxic epidermal necrolysis, subcorneal type (disorder) [Ambiguous]
Italian Sindrome della cute ustionata da stafilococco, Malattia di Ritter, Sindrome di Ritter-Lyell, Necrolisi epidermica acuta, Necrolisi epidermica tossica stafilococcica
Dutch syndroom van Ritter's-Lyells, ziekte van Ritter, verschroeide huidsyndroom, exfoliatie; dermatitis, neonatorum, scalded skin; syndroom, syndroom; scalded skin, 'Scalded skin syndrome' door stafylokokken, stafylokokken verschroeide huidsyndroom, Ritter-ziekte, Scalded-skin syndroom, door stafylokokken, Stafylokken-scalded-skin-syndroom, Syndroom door stafylokokken, scalded-skin
French Syndrome de la peau ébouillantée, Épidermolyse staphylococcique aigüe, Syndrome de la peau ébouillantée staphylococcique, Dermatite exfoliatrice des nouveau-nés, Epidermolyse staphylococcique aiguë, Dermatite exfoliatrice Ritter von Rittersheim, Dermatite exfoliatrice du nouveau-né, Maladie de Ritter, Nécrolyse épidermique staphylococcique, Syndrome d'épidermolyse staphylococcique du nourrisson, Syndrome de Lyell staphylococcique, Épidermolyse staphylococcique aiguë, Épidermolyse superficielle staphylococcique
German Syndrom der verbruehten Haut, Ritter-Lyells-Syndrom, Ritter-Krankheit, Staphylococcal scalded skin syndrome [SSS-Syndrom], staphylogenes Syndrom der verbruehten Haut, Dermatitis exfoliativa neonatorum, SSS-Syndrom, Lyell-Syndrom, staphylogenes, Ritter-von-Rittershain-Krankheit, Staphylococcal Scalded Skin Syndrome, Staphylogenes Lyell-Syndrom
Portuguese Síndrome de Ritter-Lyell, Síndrome de pele escaldada, Síndrome da Pele Escaldada Estafilocócica do Recém-Nascido, Síndrome da Pele Escaldada por Staphylococcus, Síndrome da Pele Escaldada por Estafilococos, Síndrome estafilocócica de pele escaldada, Doença de Ritter, Síndrome da Pele Escaldada Estafilocócica, Síndrome Estafilocócica da Pele Escaldada
Spanish Síndrome de piel escaldada, Síndrome de Ritter-Lyells, síndrome estafilocóccico de la piel escaldada, necrólisis epidérmica tóxica, tipo subcorneana (trastorno), necrólisis epidérmica tóxica, tipo subcorneana, Síndrome de Escaldadura Estafilocócica de la Piel, Síndrome de la Piel Escaldada por Staphylococcus, Síndrome de la Piel Escaldada por Estafilococos, dermatitis exfoliativa neonatal, enfermedad de Ritter, necrólisis epidérmica tóxica, tipo subcorneal (trastorno), necrólisis epidérmica tóxica, tipo subcorneal, necrólisis epidérmica tóxica, tipo subcorneana (concepto no activo), síndrome de la piel escaldada, Síndrome de piel escaldada estafilocócica, síndrome estafilocóccico de la piel escaldada (trastorno), síndrome estafilocóccico de la piel escaldada-SSSS, síndrome estafilocócico de la piel escaldada, síndrome estafilocócico de la piel escaldada (trastorno), síndrome estafilocócico de la piel escaldada-SSSS, Enfermedad de Ritter, Síndrome de la Piel Escaldada Estafilocócica, Síndrome Estafilocócico de la Piel Escaldada
Japanese リッター・ライエル症候群, 熱傷様皮膚症候群, ﾌﾞﾄﾞｳｷｭｳｷﾝｾｲﾈｯｼｮｳﾖｳﾋﾌｼｮｳｺｳｸﾞﾝ, ﾘｯﾀｰﾗｲｴﾙｼｮｳｺｳｸﾞﾝ, ﾈｯｼｮｳﾖｳﾋﾌｼｮｳｺｳｸﾞﾝ, ﾘｯﾀｰﾋﾞｮｳ, ブドウ球菌性熱傷様皮膚症候群, 熱傷様皮膚症候群-ブドウ球菌性, ぶどう球菌性熱傷様皮膚症候群, ぶどう球菌性皮膚剥離症候群, リッター病, 葡萄球菌性熱傷様皮膚症候群
Swedish Stafylokockorsakad epidermal nekrolys
Czech stafylokoková toxická epidermální nekrolýza, Ritter-Lyellův syndrom, Stafylokokový syndrom opařené kůže, Syndrom opařené kůže, Ritterův syndrom
Finnish Vastasyntyneen kesivä ihottuma
Russian NEKROLIZ EPIDERMAL'NYI TOKSICHESKII STAFILOKOKKOVYI, STAFILOKOKKOVYI NEKROLIZ EPIDERMAL'NYI TOKSICHESKII, RITTERA BOLEZN', НЕКРОЛИЗ ЭПИДЕРМАЛЬНЫЙ ТОКСИЧЕСКИЙ СТАФИЛОКОККОВЫЙ, РИТТЕРА БОЛЕЗНЬ, СТАФИЛОКОККОВЫЙ НЕКРОЛИЗ ЭПИДЕРМАЛЬНЫЙ ТОКСИЧЕСКИЙ
Korean 포도알균 화상피부 증후군
Polish Choroba Rittera, Gronkowcowe złuszczające zapalenie skóry, Gronkowcowy zespół oparzonej skóry
Hungarian Leforrázott bőr syndroma, Ritter-betegség, Ritter-Lyell syndroma, Staphylococcus leforrázott bőr syndroma
Norwegian Ritters sykdom, Staphylococcal scalded skin syndrome
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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