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1/7. Staphylococcal scalded-skin syndrome in a very low birth weight premature infant.

    Exfoliative skin diseases are rare in neonates. When caused by coagulase-positive staphylococcus aureus, scalded-skin diseases such as staphylococcal scalded-skin syndrome (SSSS), bullous impetigo, and staphylococcal scarlet fever may develop. These diseases might cause significant complications and mortality. SSSS is caused by staphylococcal exfoliative toxins A or B, which split the granular layer of the skin, induce proteolysis, and might exhibit superantigen activities, such as epidermolysis and lymphocyte mitogenicity. We describe a 1378-g premature male infant who was born at 29 weeks' gestation and developed SSSS on day 3 of life, with no clinical signs of neonatal sepsis. After cultures from the lesion and bloodstream were obtained, intravenous cloxacillin therapy was started. infection control measures were implemented instantly and included isolation of the infected infant, personnel handwashing with hexachlorophene, and placement of exposed neonates into a cohort. The initial lesion expanded and additional lesions appeared, but 12 hours after initiation of antibacterial therapy, the lesions ceased to proliferate. Cultures from scalded-skin lesions grew coagulase-positive staphylococcus aureus, whereas the bloodstream culture was sterile. The lesions resolved completely within 6 days, and the infant's subsequent course was uneventful. No similar skin lesions were noticed in other infants in the neonatal intensive care unit. We discuss recent advances in understanding the pathogenesis of neonatal SSSS, highlight the importance of early diagnosis and treatment, and stress the need for new adjunctive therapies for this disease.
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keywords = bullous
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2/7. Recurring staphylococcal scalded skin syndrome-like bullous mastocytosis: the utility of cytodiagnosis and the rapid regression with steroids.

    We report a male infant with onset of an extensive bullous eruption at the age of 45 days. staphylococcal scalded skin syndrome (SSSS) was suspected. Bullous mastocytosis was diagnosed by cytodiagnosis and confirmed by histologic examination. Three serious relapses were noted in a 2-year follow-up, and SSSS was again suspected because of high fever and leukocytosis with neutrophilia in an infectious context. cytodiagnosis revealed the presence of mast cells and permitted rapid diagnosis of recurrences of bullous mastocytosis. Systemic corticotherapy dramatically improved the cutaneous lesions and general symptoms. This case report emphasizes the utility of cytodiagnosis in extensive blistering diseases in infancy and the possibility of obtaining rapid healing by using steroids.
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ranking = 754.25257851165
keywords = mastocytosis, bullous
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3/7. 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.
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ranking = 3
keywords = bullous
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4/7. Diffuse cutaneous mastocytosis mimicking staphylococcal scalded-skin syndrome: report of three cases.

    Three cases of diffuse cutaneous mastocytosis (DCM) were at first incorrectly diagnosed as staphylococcal scalded-skin syndrome. In the first patient, at age 1 day the disease was recognized promptly by simple techniques such as Darier's sign and Tzanck smear. Much delay in making the diagnosis occurred in the other two patients, however: almost 1 year and 15 years, respectively. Bullous manifestations in mastocytosis occur only in the first two or three years. In the first months the disease can be dangerous and life threatening. To distinguish mastocytosis from vesicular and bullous neonatal disorders, one should perform Darier's sign and a Tzanck smear. The diagnosis is confirmed by histopathologic studies. Treatment of the bullous manifestations is symptomatic, with zinc oxide paste and oral antihistamines, which may provide some relief. In addition, cimetidine and sodium cromoglycate may be beneficial. At a later age psoralen plus ultraviolet A therapy may also relieve the symptoms. Particular foods and medicines can liberate histamine and should be restricted as much as possible in extremely affected patients. Special care should be taken when these patients are to undergo anesthesia. The risk of complications during and after anesthesia is also present in other forms of mastocytosis.
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ranking = 999.6701046822
keywords = mastocytosis, bullous
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5/7. Nikolsky's sign: is it 'dry' or is it 'wet'?

    Nikolsky's signs refers to the ability to induce peripheral extension of a blister as a consequence of applying lateral pressure to the border of an intact blister. Although initially used in reference to the pemphigus group of blistering dermatoses, a positive Nikolsky's sign can be seen in other bullous diseases such as toxic epidermal necrolysis and staphylococcus scalded skin syndrome. Appreciating whether the blister is 'wet' or 'dry' at the site of a positive Nikolsky's signs may have both diagnostic and prognostic significance which I illustrate with several clinical cases. Lastly, I review the significance of a positive Nikolsky's sign.
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keywords = bullous
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6/7. Recurrent staphylococcal scalded skin syndrome in children: report of two cases.

    A localised "scalded skin syndrome" occurred in two male siblings aged five and ten years old, in Morogoro, tanzania. This condition recurred in the same children within a period of about 12 months. A haemolytic ampicillin resistant staphylococcus aureus was isolated from the bullous material, suggesting that the condition was staphylococcal scalded skin syndrome (SSSS). This implies that the bacterial isolate was an exfoliative toxin (ET) producing S. aureus. This infection was, however, striking because SSSS occurs mostly in newborns, and it is rarely recurrent. It is possible that a new strain with particular adaptability, or an inherent susceptibility to the S. aureus of the affected children was the cause of this recurrency.
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keywords = bullous
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7/7. Neonatal pseudomonas putida infection presenting as staphylococcal scalded skin syndrome.

    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.
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keywords = bullous
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