Difficulties in diagnosis and management of the staphylococcal scalded skin syndrome.
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Biomedical subjects
Publications and source records attributed to S Ladhani.
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Staphylococcal scalded skin syndrome is the term used for a spectrum of primarily neonatal blistering skin diseases caused by the exfoliative toxins, ETA and ETB, of Staphylococcus aureus. Despite 25 y of research, the toxins' mechanism of action is still poorly understood, although evidence suggests that they may act as serine proteases. In this study, 0.1 mg purified ETA isolated from a baby with pemphigus neonatorum was incubated with A431 cells (a human squamous cell line) at 37 degrees C for 8, 24 and 48 h and the supernatant tested for protease activity using azocasein as a non-specific substrate. Phosphate-buffered saline was also incubated as negative control. Incubation of ETA with A431 cells for 48 h resulted in a four-fold increase in supernatant azocaseinolytic activity compared with buffer and cells, ETA alone and buffer alone (p < 0.001). Furthermore, this proteolytic activity was inhibited by PMSF (p < 0.001), a specific serine protease inhibitor. These results provide further evidence for the role of the exfoliative toxins as serine proteases. Furthermore, the A431 cell assay provides a simpler, quicker, cheaper and more acceptable alternative to neonatal mouse epidermis to study the mechanism of action of the exfoliative toxins.
The exfoliative (epidermolytic) toxins of Staphylococcus aureus are the causative agents of the staphylococcal scalded-skin syndrome (SSSS), a blistering skin disorder that predominantly affects children. Clinical features of SSSS vary along a spectrum, ranging from a few localized blisters to generalized exfoliation covering almost the entire body. The toxins act specifically at the zona granulosa of the epidermis to produce the characteristic exfoliation, although the mechanism by which this is achieved is still poorly understood. Despite the availability of antibiotics, SSSS carries a significant mortality rate, particularly among neonates with secondary complications of epidermal loss and among adults with underlying diseases. The aim of this article is to provide a comprehensive review of the literature spanning more than a century and to cover all aspects of the disease. The epidemiology, clinical features, potential complications, risk factors, susceptibility, diagnosis, differential diagnoses, investigations currently available, treatment options, and preventive measures are all discussed in detail. Recent crystallographic data on the toxins has provided us with a clearer and more defined approach to studying the disease. Understanding their mode of action has important implications in future treatment and prevention of SSSS and other diseases, and knowledge of their specific site of action may provide a useful tool for physiologists, dermatologists, and pharmacologists.
A case of neonatal Pseudomonas putida sepsis presenting as staphylococcal scalded skin syndrome is described. Staphylococcal scalded skin syndrome is a clinical term used for a spectrum of primarily neonatal blistering skin disorders caused by the exfoliative toxins of Staphylococcus aureus. The disease typically begins with general erythema and fever, followed by the formation of large fluid-filled bullae that coalesce and rupture on slightest pressure to leave extensive areas of denuded skin. The 9-day-old male infant described presented with a generalised non-tender, macular, erythematous rash that later developed into large, flaccid, clear fluid-filled bullae to leave extensive erythematous, weeping, and denuded areas covering over 90% of the total body surface. Despite aggressive antibiotic and symptomatic treatment, he died 11 days after admission. While Pseudomonas infections may present with vesico-bullous eruptions, this is believed to be the first case of neonatal Pseudomonas putida sepsis presenting as staphylococcal scalded skin syndrome.
In the face of an exponential increase in published biomedical studies, dermatologists frequently turn to review articles in order to keep abreast of important developments in the treatment of skin diseases. Traditional review articles have recently been criticized on the basis of their incompleteness and susceptibility to bias. Such biases can be minimized by employing a systematic approach to gathering, combining and interpreting the evidence of treatment efficacy. Using eight predetermined quality criteria, we compared the quality of 10 traditional review with one systematic review of treatments for postherpetic neuralgia, which were identified from the Medline database for 1992-96. None of the 10 traditional review articles satisfied all eight criteria: one satisfied five, two satisfied four and the rest satisfied two or fewer criteria. There was a wide variation in the recommendations of the authors for the treatment of postherpetic neuralgia, often based on anecdotal evidence and clinical experience. On the other hand, the systematic review fulfilled seven of the eight quality criteria, failing only to discuss future directives. Furthermore, treatment recommendations were made solely on the basis of randomized controlled trials, which are considered to be the gold standard for measuring the benefits of any intervention. The variation in quality and treatment recommendations of traditional reviews is worrying. Systematic reviews should be encouraged in dermatology because they provide a summary of evidence of the effects of dermatological treatments, which has been derived using explicit methods widely accepted within science.
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As part of the curriculum, medical students at the United Medical and Dental Schools of Guy's and St Thomas's Hospitals (UMDS), London, are encouraged to spend an elective period of 8 weeks in their final year anywhere in the world, studying any field of medicine they are interested in. Having lived in Tanzania for 10 years, I have had contact with people suffering from leprosy and my interest in leprosy continued after I moved to Europe to continue my education. I therefore decided to use my elective to gain hands-on experience with the disease so that I could understand and appreciate the impact of leprosy in developing countries such as Pakistan.
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Sweet's syndrome (acute febrile neutrophilic dermatitis) is characterised by classical skin lesions accompanied by fever and malaise. Systemic involvement may be present and lung involvement in Sweet's syndrome has been reported in the form of bilateral pulmonary infiltrates, bronchiolitis obliterans organising pneumonia and pleural effusion. There are dense papillary neutrophilic infiltrates on histopathology. We present a case of Sweets' syndrome with left lower lobe consolidation and persistent fever which was non-responsive to antibiotics but showed clinical improvement with clearing of radiological opacities on oral steroid therapy.