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© 2003 American Society for Clinical Oncology
Diagnostic Challenges in Patients With TumorsCASE 2. STAPHYLOCOCCAL SCALDED-SKIN SYNDROME IN A PATIENT WITH EXTENDED SMALL-CELL LUNG CANCERAzienda Ospedaliera S. Filippo Neri, Rome, Italy
A 66-year-old white man affected by small-cell lung cancer with isolated brain metastasis was treated with chemotherapy and whole-brain irradiation. He was admitted to our center because of severe mucosal erosions and skin macules with fever. Hematologic studies revealed a normochromic-normocytic anemia (hemoglobin, 8.7 g/dL) with normal leukocyte and platelet counts. Chemical studies showed an increased alkaline phosphatase level (619 U/L; normal value [NV], < 300 U/L) and hypoalbuminemia (1,770 mg/dL; NV, 3,600 to 5,100 mg/dL). Serum and urine protein electrophoresis and quantitative immunoglobulin levels were normal. Hemocoagulation tests demonstrated high levels of D dimers (3.05 µg/mL; NV, 0.0 to 0.5 µg/mL), fibrinogen degradation products (20 µg/mL; NV, 0.0 to 5.0 µg/mL), and fibrinogen (638 mg/dL; NV, 150 to 450 mg/dL) with normal levels of antithrombin III and International Normalized Ratio. Velocity of erythro-sedimentation was 130 mm/h. Objective examination showed diffuse mucosal erosions with widespread erythematous, cutaneous macules, partially confluent, and flaccid bullae with exfoliation, involving more than 30% of the total body skin area (Fig 1
Staphylococcal scalded-skin syndrome (SSSS) is caused by the exfoliative toxins A and B produced by certain strains of S aureus.1,2 It primarily affects infants and children3,4 but occasionally has been reported in adults.5 On the basis of case registries and observational studies, its incidence is estimated to be 1 to 1.4 cases per million inhabitants per year.6,7 SSSS is characterized by erythema, fever, and large, fragile, superficial blisters that rupture, leaving extensive areas of denuded skin.1,2 Disease severity ranges from a few localized blisters to generalized exfoliation affecting the entire body surface.8 In SSSS, epidermal detachment involves greater than 30% of the total-body skin area; in Stevens-Johnson syndrome (SJS), epidermal detachment involves less than 10% of the total-body skin area, whereas transitional SJS-SSSS is defined by a detachment between 10% and 30%.6 Mortality rates are 5% with SJS, 30% to 50% with SSSS, and 10% to 15% with transitional forms.6 Superinfection, thermoregulation impairment, excessive energy expenditure, alteration of immunologic response, and electrolytes and hematologic abnormalities are common systemic complications. The tracheobronchial and gastrointestinal epithelium can be involved, causing high morbidity and mortality. Age, percentage of denuded skin, neutropenia, serum urea nitrogen level, and visceral involvement are prognostic factors.6 There are different scoring systems for vital prognosis estimation (simplified acute physiology score and simplified acute physiology score 11), which are not specific. A more specific score (SCORTEN) has been recently elaborated and validated.9 The management of patients with SSSS must be prompt. First, intravenous fluid replacement must be initiated using macromolecules or saline solutions. Symptomatic treatments are the same as for burns: temperature control, careful and aseptic handling, continuous parenteral nutrition, prophylactic antibiotics, anticoagulation, prevention of stress ulcer, and analgesic drugs. The use of corticosteroids is controversial. Under clinical investigation are intravenous immunoglobulin, plasmapheresis, cyclophosphamide, N-acetylcysteine, infliximab, and thalidomide. After healing, altered pigmentation and corneal lesions are the main long-term complications.6 AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicate no potential conflicts of interest.
REFERENCES
1. Ladhani S, Robbie S, Garratt RC, et al: Development and evaluation of detection systems for Staphylococcal exfoliative toxin A responsible for scalded-skin syndrome. J Clin Microbiol 39:2050–2054, 2001 (suppl 6)
2. Ladhani S, Joannou CL, Lochrie DP, et al: Clinical microbial and biochemical aspects of the exfoliative toxins causing staphylococcal scalded-skin syndrome. Clin Microbiol Rev 12:224–242, 1999 3. Ladhani S, Joannou CL: Difficulties in the diagnosis and management of staphylococcal scalded-skin syndrome. Pediatr Infect Dis J 19:819–821, 2000[CrossRef][Medline] 4. Ladhani S, Newson T: A familial outbreak of staphylococcal scalded-skin syndrome. Pediatr Infect Dis J 19:578–579, 2000[CrossRef][Medline] 5. Cribier B, Piemont Y, Grosshans E: Staphylococcal scalded-skin syndrome in adults: A clinical review illustrated with a case. J Am Acad Dermatol 30:319–324, 1994[Medline] 6. Ghislain PD, Roujeau JC: Treatment of severe drug reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis and hypersensitivity syndrome. Dermatol Online J 8:5–14, 2002[Medline] 7. Rzany B, Mockenhaupt M, Baur S, et al: Epidemiology of erythema exsudativum multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis in Germany (1990–92): Structure and results of a population-based registry. J Clin Epidemiol 49:769–773, 1996[CrossRef][Medline]
8. Ladhani S, Evans RW: Staphylococcal scalded-skin syndrome. Arch Dis Child 78:85–88, 1998 9. Bastun-Garin S, Fouchard N, Bertocchi M, et al: SCORTEN: A severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 115:149–153, 2000 (suppl 2)[CrossRef][Medline]
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Copyright © 2003 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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