Dermatology

Staphylococcal Scalded Skin Syndrome (Ritter’s disease)

Staphylococcal Scalded Skin Syndrome (Ritter’s Disease, ICD-9 code 695.81)

Are You Confident of the Diagnosis?

The diagnosis of staphylococcal scalded skin syndrome (SSSS) can be made with confidence in the setting of a compatible clinical appearance with supporting histopathology, and established underlying Staphylococcal infection.

What you should be alert for in the history

A history of malaise, irritability and fever may be elicited in patients with SSSS. A preceding or concurrent focus of Staphylococcus aureus, such as omphalitis, otitis media, purulent conjunctivitis, nasopharyngeal infection or abscess may be identified.

Characteristic findings on physical examination

On physical examination, SSSS starts as generalized erythema, possibly accentuated in flexural surfaces and periorificial locations. Cutaneous tenderness is typical. Progression to blister formation and exfoliation usually occurs. A positive Nikolsky sign may be present. Although SSSS spares the mucous membranes, periorificial crusting may be noted.

Skin biopsy for routine histopathology, or by expedited frozen section, shows cleavage within and below the granular layer, and not full-thickness necrosis (Figure 1).

Figure 1.

Staph scalded skin syndrome. Superficial roof of the blister at the granular cell layer. (Courtesy of Bryan Anderson, MD)

Expected results of diagnostic studies

Cultures are typically negative from the eruption, but S aureus can be cultured from sites such as conjunctiva, nasopharynx, ears or abscess, depending on the focus of infection. Although rare in children, blood cultures may be positive in adults. Polymerase chain testing for staphylococcal toxins is available, but not generally necessary.

Although imaging studies are not necessary for the diagnosis of SSSS, they may be indicated if the focus of underlying staphylococcal infection is not apparent.

Diagnosis confirmation

The differential diagnosis of SSSS includes the following entities (1) Toxic Epidermal Necrolysis (can be distinguished by prominent mucous membrane involvement and full-thickness epidermal necrosis histologically); (2) Toxic Shock Syndrome (also due to underlying bacterial infection, due to Staphylococcus or Streptococcus, but is typically a generalized nonbullous eruption, and is usually associated with hypotension and a more profoundly ill patient with multisystem disease); (3) Kawasaki Disease (although usually also most common in young children, Kawasaki disease has concurrent prolonged fever, prominent mucous membrane involvement, extremity changes [edema and late acral desquamation], cervical lymphadenopathy and a polymorphous eruption); and (4) Scarlet fever (usually diffuse “sandpaper-like” eruption but nonbullous, associated with bacterial pharyngitis, “strawberry tongue,” and Pastia’s lines [linear petechia] in flexural surfaces).

Who is at Risk for Developing this Disease?

SSSS primarily occurs in neonates and young children, and can occur in neonatal nurseries and day-care settings. When SSSS rarely occurs in adults, patients typically have impaired renal function or underlying immunocompromise. Males are more frequently reported, and African-American children are less susceptible than Caucasians.

What is the Cause of the Disease?

Etiology

Pathophysiology

SSSS is caused by underlying infection with toxin-producing strains of S aureus, usually belonging to phage group 2, types 3A, 3B, 3C, 55 or 71. Exfoliative toxins ET-A and ET-B, produced by S aureus, are serine proteases that target desmoglein-1, leading to epidermal cleavage and bulla formation. At-risk groups, such as neonates and renally-impaired adults, are unable to clear the toxin adequately, leading to clinical disease. Additionally, neonates and immunocompromised patients of all ages may lack an adequate humoral antibody response, allowing bacterial proliferation and development of SSSS.

Systemic Implications and Complications

Childhood SSSS generally has a good prognosis. The pediatric mortality rate of 4% is associated with more severe infection, such as sepsis or pneumonia. The much higher mortality rate (>60%) encountered in adult patients may be due to more frequent underlying renal compromise or immunosuppression, widespread fluid and electrolyte imbalances, or progression of infection to pneumonia or sepsis.

Treatment Options

Treatment optons are summarized in Table I.

Table I.

Treatment options for staphylococcal scalded skin syndrome
MEDICAL SURGICAL PHYSICAL
Antistaphylococcalantibiotics
Fluid and electrolyte support
Mupirocin ointment NotApplicable Physical Therapy
Intravenous immunoglobulin
Fresh frozen plasma

Optimal Therapeutic Approach for this Disease

Antibiotic therapy should be initiated intravenously, with a possible change to oral therapy as disease improves over several days. Dosages of antibiotics should be titrated according to the age and weight of the patient.

When susceptibility of organism is not yet known, antibiotic therapy should be initiated with a penicillamine-resistant penicillin such as cloxicillin or nafcillin, with a first-generation cephalosporin as an alternative In penicillin-allergic patients, clindamycin, macrolides, aminoglycosides and trimethoprim/sulfamethoxazole are possible alternatives. Tetracycline antibiotics may be considered in children older than 8 years, or in adults. If the isolate is proven or suspected to be methicillin-resistant S aureus (MRSA), vancomycin may be necessary. Fluid and electrolyte support are important, as imbalances may be present due to exfoliation.

A thin layer of mupirocin ointment applied three to five times daily for 5-14 days to the site of infection, such as umbilicus or nares, can be helpful. Strict hand-washing and infection prevention are paramount in health care workers attending to these patients to prevent nosocomial spread.

For refractory cases, especially in the setting of toxemia, intravenous immunoglobulin therapy or fresh frozen plasma (which is likely to contain antibodies to exotoxins) may be considered. Physical therapy to encourage mobilization, especially of affected limbs, may be utilized.

Patient Management

Patients should be monitored for clinical improvement, resolution of underlying infection, and fluid/electrolyte status. Worsening or progression of illness should lead to concern for a resistant organism, or concurrent underlying serious infection such as pneumonia or sepsis. Appropriate cultures and imaging studies, as well as directed changes in antibiotic therapy, may be necessary.

Families should be counseled as to the generally favorable prognosis, and that antibiotic therapy and fluid/electrolyte management is typically well-tolerated.

Adult patients, due to usual co-morbid conditions such as renal compromise, have a higher mortality rate. Renal toxicity of medications, as well as adequate hydration, are important concerns in this group.

Unusual Clinical Scenarios to Consider in Patient Management

Failure to improve with appropriate antibiotic therapy raises concern for an antibiotic-resistant strain of S aureus such as MRSA, with adjustment of antibiotics accordingly.

Awareness of the potential for nosocomial spread, especially in a nursery setting, is important. Proper hand-washing, appropriate control measures such as barrier protocols and possible intranasal mupirocin ointment for treating health care workers may all be necessary

What is the Evidence?

Patel, GK, Finlay, AY. "Staphylococcal scalded skin sydrome: diagnosis and management". Am J Clin Dermatol. vol. 4. 2003. pp. 165-75.

(An excellent summary of the clinical features, pathogenesis and treatment of SSSS.)

Stanley, JR, Amagai, M. "Pemphigus, bullous impetigo and the staphylococcal scalded skin syndrome". N Engl J Med. vol. 355. 2006. pp. 1800-10.

(A thorough discussion of the pathophysiology of antibodies and molecular targets involved in epidermal cleavage in these diseases.)

Nishifuji, K, Sugai, M, Amagai, M. "Staphylococcal exfoliative toxins: “molecular scissors” of bacteria that attack the cutaneous defense barrier in mammals". J Dermatol Sci. vol. 49. 2008. pp. 21-31.

(A complete discussion of the pathomechanism involved in toxins leading to clincal disease.)

Murray, RJ. "Recognition and management of Staphylococcus aureus toxin-mediated disease". Int Med J. vol. 35. 2005. pp. S106-S119.

(A summary of the epidemiologic and clinical features of staphylococcal toxin-mediated disease.)

El Helali, N, Carbonne, A, Naas, T, Kerneis, S, Fresco, O, Giovangrandi, Y. "Nosocomial outbreak of Staphylococcal scalded skin syndrome in neonates: epidemiological investigation and control". J Hosp Infect. vol. 61. 2005. pp. 130-8.

(Details a nosocomial outbreak in a maternity unit, and discusses appropriate precautions to avoid further outbreaks.)

Kapoor, V, Travadi, J, Braye, S. "Staphylococcal scalded skin syndrome in an extremely premature neonate: a case report with a brief review of literature". J Paediatr Child Health. vol. 44. 2008. pp. 374-6.

(Discusses the use of intravenous immunoglobulin in the treatment of a pre-term infant with SSSS.)
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