Staphylococcal scalded skin syndrome L00.-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Butcher's pemphigus; Dermatitis exfoliativa neonatorum; Dermatitis exfoliativa neonatorum staphylogenes; infant pemphigoid; Knight of Rittershain M.; neonatal bullous impetigo; Pemphigoid staphylogenes; pemphigus acutus febrilis gravis; pemphigus acutus neonatorum; pemphigus febrilis; Ritter von Rittershain disease; Scalded skin syndrome; SSSS; staphylodermia superficialis bullosa; Staphylodermia superficialis bullosa neonatorum et infantum; staphylodermia superficialis diffusa exfoliativa; Staphylogenic Lyell Syndrome

History
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Knight of Rittershain 1878; Lyell,1956

Definition
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Staphylococcus toxin induced, mostly severe (often intensive care) extensive dermatitis with extensive detachment of the skin. The blister formation is localized intraepithelially.

Pathogen
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Staphylococcus aureus, mostly phage group II, here especially type 3A, 3C, 55 and 71

Etiopathogenesis
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This is caused by the exfoliative toxins (exfoliatins A, B) forming staphylococci. As highly specific serine proteases, these circulating exfoliatins cause the splitting of intercellular connections of the epidermis(desmoglein I - is only formed within the epidermis) and thus lead to the dissolution of the keratinocyte network and an intraepidermal bladder. The high level of blister formation in the epidermis is explained by the fact that desmoglein is formed throughout the entire epidermis, but in the deeper parts desmoglein 3 prevents further acantholysis.

Manifestation
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Occurs in infants in the first 3 months of life, infants and immunologically weakened adults.

Clinical features
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Significantly reduced general condition with fever, skin tension, possibly purulent rhinitis, pharyngitis, otitis or conjunctivitis
Following the intial symptoms caused by the staphylogenic local infection, a large-area, blurred, flexural, scarlatiniform macular exanthema appears.

Within 1-2 days on trunk and extremities, formation of large, flabby, easily bursting blisters. The burst blister cover lies on the underlying red skin areas like a wet clapped on cloth. Positive Nikolski sign.

Drying of the blister cover, coarse lamellar, greasy scaling. In this case the skin shreds can be easily peeled off the underlay like a thin cellophane skin.

Histology
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Subcorneal bladder: Acantholytic cleavage within the stratum granulosum.

Diagnosis by frozen section: Carefully remove a piece of the bladder roof with scissors and tweezers, place in saline solution, frozen section in a histological laboratory using cryostat technique and staining with methylene blue. Determination of the level of blister formation in the epidermis.

Differential diagnosis
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General therapy
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Corresponding to the TEN; hospital admission; antibiotic therapy.

External therapy
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Detach necrotic skin areas, open blisters. Cover open skin areas with sulfadiazine-silver (e.g. flammazine) and bandage sterile.oral hygiene with astringent liquids. Several times a day eye hygiene with disinfecting and astringent eye drops (e.g. Solan eye drops), also apply a thick layer of eye ointment containing dexpanthenol, loosen adhesions with a swab.

Internal therapy
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Immediate high-dose antibiotic administration: Penicillinase-resistant penicillins i.v., e.g. oxacillin (InfectoStaph) 2-4 g/day in 2-4 ED i.v. Alternatively cephalosporins such as cefuroxime (e.g. zinacef) 750-1500 mg every 6 hours i.v. or cefotaxime (e.g. claforan) 2 g every 12 hours; forced diuresis.

Notice! Glucocorticoids are contraindicated!

In severe cases, balance fluid and parenteral nutrition. Immediate check of intensive care obligation!

Progression/forecast
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With timely therapy reepithelialization within one week. Otherwise severe course.

The"Recalcitrant erythematous desquamating disorder -RED-" is considered a TSS variant. In this clinical picture, the course of the disease is clearly prolonged with about 50 days and characterized by repeated relapses.

The"perineal recurrent toxin-mediated erythema" also belongs to the group of dermatitis caused by staphylococcal toxins.

Note(s)
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The"Recalcitrant erythematous desquamating disorder -RED-" is considered a TSS variant. In this clinical picture, the course of the disease is clearly prolonged with about 50 days and characterized by repeated relapses.

The"perineal recurrent toxin-mediated erythema" also belongs to the group of dermatitis caused by staphylococcal toxins.

Literature
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  1. Dobson CM et al (2003) Adult staphylococcal scalded skin syndrome: histological pitfalls and new diagnostic perspectives. Br J Dermatol 148: 1068-1069
  2. Hanakawa Y et al (2002) Molecular mechanisms of blister formation in bullous impetigo and staphylococcal scalded skin syndrome. J Clin Invest 110: 53-60
  3. Patel GK et al (2003) Staphylococcal scalded skin syndrome: diagnosis and management. At J Clin Dermatol 4: 165-175
  4. Pereira FA et al (2007) Toxic epidermal necrolysis. J Am Acad Dermatol 56: 181-200
  5. Petzelbauer P et al (1989) Staphylococcal scalded skin syndrome in two adults with acute renal failure. dermatologist 40: 90-93
  6. Prevost G et al (2003) Staphylococcal epidermolysins. Curr Opin Infect Dis 16: 71-76
  7. Suzuki R et al (2003) Adult staphylococcus scalded skin syndrome in a peritoneal dialysis patient. Clin Exp Nephrol 7: 77-80

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