Staphylococcal scalded skin syndrome
Staphylococcal scalded skin syndrome (SSSS), also known as Ritter’s disease or Ritter-Lyell syndrome, is marked by epidermal erythema, peeling, and necrosis that give the skin a scalded appearance. This severe skin disorder follows a consistent pattern of progression, and most patients recover fully. Mortality is 2% to 3%, with death usually resulting from complications of fluid and electrolyte loss, sepsis, and involvement of other body systems.
Causes and incidence
The causative organism in SSSS is group 2 Staphylococcus aureus, primarily phage type 71, which produces exotoxins that cause detachment of the epidermis. Predisposing factors may include impaired immunity and renal insufficiency — present to some extent in the normal neonate because of immature development of these systems.
SSSS is most prevalent in infants age 1 to 3 months but may develop in children. It’s uncommon in adults.
Signs and symptoms
SSSS can usually be traced to a prodromal upper respiratory tract infection, possibly with concomitant purulent conjunctivitis. Cutaneous changes progress through three stages:
❑ Erythema: Erythema, which may begin diffusely or as a scarlatiniform rash, usually becomes visible around the mouth and other orifices and may spread in widening circles over the entire body surface. The skin becomes tender; Nikolsky’s sign (sloughing of the skin when friction is applied) may appear.
❑ Exfoliation (24 to 48 hours later): In the more common, localized form of this disease, superficial erosions with a red, moist base and minimal crusting occur, generally around body orifices, and may spread to exposed areas of the skin. (See Identifying staphylococcal scalded skin syndrome.) In the more severe forms of this disease, large, flaccid bullae erupt and may spread to cover extensive areas of the body. These bullae eventually rupture, revealing sections of denuded skin; mucous membranes are spared.
❑ Desquamation: In this final stage, affected areas dry up, and powdery scales form. Normal skin replaces these scales in 5 to 7 days.
Diagnosis
Diagnosis requires careful observation of the three-stage progression of this disease. Results of exfoliative cytology and biopsy aid in differential diagnosis, ruling out erythema multiforme and drug-induced toxic epidermal necrolysis, both of which are similar to SSSS.
CONFIRMING DIAGNOSIS Isolation of group 2 S. aureus on cultures of skin lesions confirms the diagnosis. However, skin lesions sometimes appear sterile.
Treatment
Treatment includes systemic antibiotics, usually penicillinase-resistant penicillin. Severe cases require hospitalization and I.V. antibiotics. Oral antibiotics should be adequate for milder cases. Skin lubrication with a non–alcohol-based preparation is beneficial. Washing or bathing should be done sparingly. Replacement measures to maintain fluid and electrolyte balance are necessary.
PEDIATRIC TIP Admission is appropriate for neonates and young children with extensive sloughing.
Special considerations
❑ Carefully monitor intake and output to assess fluid and electrolyte balance. In severe cases, I.V. fluid replacement may be necessary.
❑ Check vital signs. Be especially alert for a sudden rise in temperature, indicating sepsis, which requires prompt, aggressive treatment.
❑ Maintain skin integrity. Use strict sterile technique to preclude secondary infection, especially during the exfoliative stage, because of open lesions. To prevent friction and sloughing of the skin, leave affected areas uncovered or loosely covered. Place cotton between severely affected fingers and toes to prevent webbing.
❑ Gently débride exfoliated areas, especially those that have become necrotic.
❑ Reassure the parents that complications are rare and residual scars are unlikely.
PEDIATRIC TIP Provide special care for the neonate, if required, including placement in a warming infant incubator to maintain body temperature and provide isolation. Pictures
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2008 Williams & Wilkins.
More About Causes of Flaking skin
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