11/46. Differential pathomechanisms of epidermal necrolytic blistering diseases. staphylococcal scalded skin syndrome (SSSS) results from the effect of exfoliative-toxins produced by staphylococcal strains. The disease affects predominantly children, and is rare in adults. We report two cases of the adult type of SSSS. Corticotherapy, chronic alcohol abuse and epilepsy-related immune changes might have been predisposing factors in these patients. The immunopathological characteristics of the inflammatory cell infiltrate in adults SSSS have not been thoroughly explored so far in the literature. Biopsies from 2 patients with bullous SSSS skin were studied by means of immunochemistry using a panel of 10 antibodies directed to FXIIIa, CD15, CD31, CD45R0, CD50, CD54, CD62E, CD95, CD106, and L1-protein, respectively. Cutaneous biopsies from related blistering diseases were compared. They included drug-induced toxic epidermal necrolysis (TEN), bullous impetigo and superficial pemphigus. A dense cell infiltrate composed of granulocytes (CD15 ), macrophages (L1 protein ) and memory T cells (CD45R0 ) and a strong expression of ICAM-3 (CD50) were present in the epidermis. CD95 keratinocytes were lining the intraepidermal blisters. Type I dermal dendrocytes (factor xiiia ) were numerous and plump in the dermis. Bullous impetigo exhibited the same pattern of inflammatory cells, but with a lower density in type I dermal dendrocytes. TEN differed from SSSS by both the absence of CD15 granulocytes and a stronger expression of the pro-apoptotic CD95 antigen in the epidermis. In superficial pemphigus, CD95 antigen was not expressed, and CD15 granulocytes, CD45R0 lymphocytes and L1 protein monocytes were much less numerous. It is concluded that the specific binding of SSSS-induced exotoxins to the desmosomes alters the keratinocyte metabolism leading to an inflammatory reaction followed by focal apoptosis. Our findings are in line with the concept that SSSS exotoxins might be superantigens. A common pathomechanism leading to epidermal destruction is likely operative in SSSS and bullous impetigo. The inflammatory cell composition in TEN and superficial pemphigus markedly differs from that in SSSS. ( info) |
12/46. staphylococcal scalded skin syndrome related to an exfoliative toxin A- and B-producing strain in preterm infants. A previously well, spontaneously breathing premature infant (gestational age 25 weeks, birth weight 364 g, age 74 days) developed staphylococcal scalded skin syndrome (SSSS). A methicillin-sensitive strain of staphylococcus aureus producing exfoliative toxins A and B (ETA, ETB) was isolated from a gastric aspirate and a pharyngeal swab. The disease recurred with a milder clinical picture 4 weeks later in the same patient while under steroid treatment. Cultures obtained from conjunctiva and pharynx were again positive for S. aureus. A second premature infant in an adjacent ward developed SSSS 2 weeks after the recurrence in the first patient. No other cases were observed thereafter. A total of 25 individuals who had contact with the first patient were screened for staphylococcal colonisation. S.aureus was isolated from the posterior part of the nasal cavity in 8 of the 25 contacts. These strains and the strain of the first patient were evaluated by PCR for the presence of genes encoding ETA and ETB. Expression of toxins was confirmed by gel electrophoresis and Western blot analysis. Purified toxins were injected into newborn mice to confirm toxin activity. Besides the strain isolated from the first patient, only one isolate from the medical staff was positive for the genes encoding ETA and ETB. CONCLUSION: the carrier of this strain had contact with both patients, suggesting that this individual was the vector between the two patients but not necessarily the source of the original infection. Strict infection control measures were implemented and no further spread of the disease occurred. ( info) |
| staphylococcal scalded skin syndrome (SSSS) is a disease primarily of young children, characterized by exfoliative dermatitis caused by exfoliative toxin producing staphylococcus aureus. We had three cases of SSSS with varied dermatological manifestations-diffuse/scarlitiniform erythema, generalized exfoliation, sand paper skin texture, flaccid bullae, erosions, seborrheic dermatitis like scaling and cracking in skin creases which can be confused with other skin conditions. Hence, a high index of suspicion, early diagnosis and prompt treatment is imperative. ( info) |
| staphylococcal scalded skin syndrome (SSSS) may cause significant morbidity in children. It is common practice for adhesive occlusive dressings to be used to apply topical local anaesthetic prior to venepuncture. We report two cases in which removal of these dressings from children with SSSS caused injury and discomfort in areas previously free from blistering. We recommend that an alternative method of topical anaesthetic application is used to minimize skin trauma in these patients. ( info) |
| staphylococcal scalded skin syndrome is a toxin mediated Staphylococcal infection, the toxin produced by staphylococcus aureus type 2 phage types (55,71,3A,3B,3C). There is a generalized tender erythema which commences on the head and neck, accompanied by fever, irritability, continuous cry and miserable look. The erythema is followed by cleavage of the upper epidermis in a large sheets mainly in the head, neck and the flexures, with formation of bullae (Nikolsky sign). It is most common in infants and children under 5 years. Most cases respond to antibiotics with other supportive measures. The prognosis is good, and the skin lesions disappear without a residual scar. ( info) |
16/46. adult staphylococcus scalded skin syndrome in a peritoneal dialysis patient. staphylococcal scalded skin syndrome (SSSS), a generalized exfoliative dermatitis complicating infections by exfoliative toxin-producing strains of Staphylococcus aureus, is rarely observed in adults, especially in those with chronic renal failure. In contrast to the mortality in infants, the mortality in adults is usually high. A case of generalized SSSS in a peritoneal dialysis patient is reported. A-62-year old Japanese man in whom peritoneal dialysis had been carried out was admitted to our hospital with acute peritonitis. It was intractable peritonitis in terms of resistance to various antibiotics; however, improvement of the peritonitis was shown after the injection of vancomycin. Nine days after the completion of this medication, erythema appeared in the eyes and mouth, and around the nostrils, with an exothermic reaction at 39.0 degrees C. Radial cracks formed in the face within a few days, and the erythema rapidly expanded to the neck, axilla, the whole body. The blood pressure was also lowered, and this led to a state of shock. culture of skin biopsy specimens yielded identical strains of S. aureus. A presumptive diagnosis of SSSS was made. The patient was treated with antibiotics that were effective against the organisms and with both fluid supplementation and dopamine, resulting in subsidence of the signs and symptoms. The exothermic reaction and skin symptoms were improved promptly, with improvement in the general condition, including the state of shock. This appears to be the first reported case of SSSS caused by S. aureusin an adult patient with peritoneal dialysis who was treated successfully. It is very important that SSSS be differentiated from toxic epidermal necrosis, as the treatment is different. ( info) |
| Two premature infants with very low birth weight were diagnosed with staphylococcal scalded skin syndrome (SSSS) during hospitalization in the neonatal intensive care unit. This syndrome which is rare in premature infants, is characterized by blistering and superficial desquamation of the skin and is caused by two epidermolytic toxins (ETA and ETB) produced by staphylococcus aureus. staphylococcal scalded skin syndrome usually occurs in young children probably because of inefficient clearance of the epidermolytic toxins from the bloodstream, which causes dysfunction of cell adhesion in the superficial epidermis. early diagnosis and early treatment with parenterally administered beta-lactamase resistant penicillins are important to prevent life threatening complications of this syndrome. ( info) |
18/46. Streptococcal toxic shock syndrome and sepsis manifesting in a patient with chronic rheumatoid arthritis. Streptococcal-toxic-shock syndrome is caused by virulent strains of exotoxin-producing streptococcus, almost always group-A organisms such as streptococcus pyogenes. It has often been described in the setting of surgical wounds, burns, childbirth, diabetics, elderly, neonates, and immunocompromised hosts, where the portal of entry is the skin. Our patient was on steroids and nonsteroidal anti-inflammatory drugs for chronic rheumatoid arthritis and developed this deadly infection after a fall. ( info) |
19/46. netherton syndrome: report of two Taiwanese siblings with staphylococcal scalded skin syndrome and mutation of SPINK5. netherton syndrome (NS) is a severe autosomal recessive ichthyosis. It is characterized by congenital ichthyosiform erythroderma, trichorrhexis invaginata, ichthyosis linearis circumflexa, atopic diathesis and frequent bacterial infections. Pathogenic mutations in SPINK5 have recently been identified in NS. SPINK5 encodes lymphoepithelial Kazal-type-related inhibitor (LEKTI), a new type of serine protease inhibitor involved in the regulation of skin barrier formation and immunity. We report two Taiwanese brothers with NS. The patients had typical manifestations of NS with an atopic diathesis and recurrent staphylococcal infections, including staphylococcal scalded skin syndrome (SSSS) since birth. Horny layers were obtained by skin surface biopsy for electron microscopy from lesional skin of both patients and from normal controls. All 33 exons and flanking intron boundaries of SPINK5 were amplified for direct sequencing. The ultrastructure of the stratum corneum (SC) was characterized by premature degradation of corneodesmosomes (CDs) with separation of corneocytes. A homozygous 2260A --> T (K754X) mutation of SPINK5 was found in both patients. Staphylococcal exfoliative toxin A (ETA) is a serine protease capable of cleaving desmoglein 1, an important adhesive molecule of CDs, and can cause separation of the SC, resulting in SSSS. The premature degradation of CDs found in our patients may be attributable to insufficient LEKTI, and possibly also to colonization/infection of ETA-producing staphylococcus aureus. Mechanisms involved in the pathogenesis of the skin barrier defect in NS are proposed. Further study is needed to prove this hypothesis. ( info) |
20/46. Staphyloccocal scalded skin syndrome in an adult. We report a case of staphyloccocal scalded skin syndrome due to an oxacillin-susceptible staphylococcus aureus in an 81-year-old woman. The patient was admitted to the emergency room with arthritis of the left shoulder, ten days after an intra-articular injection of corticosteroids. The shoulder's puncture showed a purulent liquid and gram positive cocci in cluster suggesting the presence of Staphylococcus sp. on the Gram-stain. The culture confirmed the identification of an oxacillin-susceptible staphylococcus aureus. Before administration of any dose of oxacillin, blisters appeared on the skin, that quickly ruptured, particulary in areas of friction. The exfoliated areas were extensive and resolution of all the lesions was reached after 3 weeks. The skin biopsy showed superficial epidermolysis confirming the diagnosis. Staphyloccocal scalded skin syndrome is usually described in neonates and young children, often in outbreaks. Few cases have been reported in adults, most often associated with severe underlying diseases. The mortality rate is low in children but can reach almost 60% in adults. The most important diagnosis to exclude is Lyell's syndrome which can be done by the skin biopsy. ( info) |