Neonate twin with staphylococcal scalded skin syndrome from a renal source.

OBJECTIVE: To understand the underlying mechanism of exfoliative toxins causing staphylococcal scalded skin syndrome or Ritter's Disease that predominantly affects newborns and infants, although it is sometimes found in adults. Staphylococcal scalded skin syndrome is typically diagnosed by the characteristic fluid-filled bullae together with superficial skin loss. A histopathological diagnosis may be made by looking for subcorneal acantholytic cleavage with minimal inflammation on biopsy, although this is not normally required. Exfoliative toxin A and B are both responsible for the "acantholytic" infection of Staphylococcus aureus as they target desmoglein-1 leading to loss of cell-to-cell cohesion and subsequent spread of infection. Other factors produced by S. aureus can cause a myriad of other problems including neutralization of antimicrobial peptides, inactivation of neutrophils, proteolysis, T-cell anergy, and immunosuppression. DESIGN: Individual care report. SETTING: Pediatric intensive care unit. PATIENT: We describe a normal male infant who was born at term and developed 100% total body surface area staphylococcal scalded skin syndrome on the 14 day of life with associated renal sepsis. INTERVENTIONS: After cultures from the lesions, bloodstream, and urine were obtained, intravenous Vancomycin and Ceftriaxone were commenced. The initial lesions increased in size over a 36-hr period to cover the entire body surface; this was associated with a decline in hemodynamic status. MEASUREMENTS AND MAIN RESULTS: Cultures from the urine and blood grew coagulase-positive S. aureus. An ultrasound scan revealed bilateral pyonephroses, which necessitated the placement of percutaneous nephrostomies with subsequent decompression of the collecting system. CONCLUSIONS: After the decompression hemodynamic status stabilized and over the ensuing 10 days, the patient made a full recovery with no scarring. No similar lesions were noticed on the infant's twin brother. We discuss the recent developments in understanding the underlying mechanism of exfoliative toxins causing staphylococcal scalded skin syndrome, review current treatment guidelines, and outline the need for new therapeutic options.

[Plastic bronchitis and mucoid impaction--uncommon disease syndromes with expectoration mucus plugs]. / Plastyczne zapalenie oskrzeli i zaczopowanie sluzem--rzadkie zespoly chorobowe przebiegajace z wykrztuszaniem odlewów oskrzelowych.

Mucoid impaction and plastic bronchitis are relatively rare disorders caused by the formation of obstructive airway plugs. We observed from February 1999 to June 2000 seven patients with mucoid impaction and one with plastic bronchitis. In the group of mucoid impaction there were 4 patients with bronchial asthma and 3 without history of lung disease. At the admission to hospital all patients suffered from cough, chest pain and effort dyspnea. Two of them expectorated during cough "bronchial casts". The chest X-ray of 5 patients revealed atelectasis of one of the lung's lobes and diffuse opacities in 2 others. In 4 cases during bronchoscopy one bronchus and in another three--numerous bronchi were obstructed with mucoid casts. Removing of the casts caused both the improvement of the patients' condition and withdrawal of atelectasis in 4 cases. In 5 patients the final diagnosis was allergic bronchopulmonary aspergillosis and in two mucoid impaction in the course of asthma without aspergillosis. Plastic bronchitis was observed in 44 years old man, who expectorated white, branching, bronchial casts for three months. On admission he was in respiratory failure. The chest X-ray revealed diffuse alveolar infiltrates and HRCT glass-ground opacities in both lungs and bronchiectasis in the middle lobe. The bronchofiberoscopy disclosed diffuse tracheobronchitis with casts occluding the middle lobe bronchus. Microscopic examination of the removed casts showed aggregates of mucus, macrophages, neutrophils and cells of respiratory epithelium. Precipitins against Aspergillus fumigatus were not found. Staphyloccocus coagulase (-) was cultured from urine and sputum specimens. We administered Vancomycin with Netylmycin, acetylocysteine, oxygen therapy and humid inhalation and the patient recovered. HRCT made six months after admission revelated total withdrawal of glass-ground opacities. The pathogenesis of plastic bronchitis in this case was unclear.