[Staphylococcal scalded skin syndrome in a pediatric patient with atopic dermatitis]. / Síndrome de la piel escaldada estafilocócica en un paciente pediátrico con dermatitis atópica.

The staphylococcal scalded skin syndrome is a rare dermatological entity that in early stages may be confused with a flare-up of a rush of atopic dermatitis. We present the case of an 8-year-old boy with a history of atopic dermatitis and egg allergy that went to the Emergency Department for erythematous-bullous lesions on the skin. Symptoms began as an erythema in areas of flexures, which associated conjunctivitis and bilateral eyelid erythema. After 24 hours, a generalized erythema appeared with flaccid blisters predominating in flexural areas, accompanied by hyperkeratosis and perioral xerosis. Due to the clinical suspicion of staphylococcal scalded skin syndrome, empirical treatment with cloxacillin with favorable response was initiated. This case highlights the importance of making a correct differential diagnosis of skin lesions of patients with atopic dermatitis in order to apply the most appropriate treatment.

El síndrome de la escaldadura estafilocócica es una entidad dermatológica poco frecuente que, en fases iniciales, puede ser confundida con una reagudización de un brote de dermatitis atópica. Se presenta el caso de un niño de 8 años, con antecedentes de dermatitis atópica y alergia al huevo, que acudió al Servicio de Urgencias por lesiones eritematoampollosas en la piel. La sintomatología comenzó como un eritema en áreas de flexuras, que asociaba conjuntivitis y eritema palpebral bilateral. A las 24 horas, apareció un eritema generalizado con ampollas flácidas de predominio en áreas flexurales, acompañado de hiperqueratosis y xerosis perioral. Ante la sospecha clínica de síndrome de escaldadura estafilocócica, se inició el tratamiento empírico con cloxacilina, con respuesta favorable. Con este caso, se destaca la importancia de realizar un correcto diagnóstico diferencial de las lesiones cutáneas de los pacientes con dermatitis atópica, con el objetivo de aplicar el tratamiento más adecuado.

A young girl with a painful rash.

The speed with which this rash spread and the fact that the patient's skin sloughed off when pressure was applied made the diagnosis clear.

Epidermal exfoliation of over 95% after a burn in an 18-month-old boy: Case report and review of the literature.

This report concerns an 18-month-old boy who presented with a 6% total body surface area scald. The subject of this report is unique in that he developed the largest exfoliation described in literature. After 3 days an epidermal exfoliation with the appearance of a deliberately inflicted scald developed. As the exfoliation progressed to over 95% total body surface area the suspicion of child abuse or neglect could be abandoned. The diagnosis Staphylococcal scalded skin syndrome was set, due to the finding of Staphylococcus aureus on swabs, the lack of mucosal engagement, and the patient's age. The boy's skin healed within 3 weeks. The few reports published are all case reports and most frequently described visually infected burns with smaller epidermal exfoliations, and clinically based exfoliation diagnosis. S. aureus often cause burn wound infections that can lead to complications caused by cross-infection. It is important for burn surgeons and intensive care specialists to be aware of the increased possibility of Staphylococcal scalded skin syndrome occurring in patients who have a reduced barrier to infection such as burn patients and also, that the diagnosis can be difficult to make.

Varicella infection in a neonate with subsequent staphylococcal scalded skin syndrome and fatal shock.

A male term neonate, at day 23 of life, presented with vesicular lesions over the trunk, which spread to allover the body on the next day. Five days later, he started developing blistering of the skin over the trunk and extremities, which subsequently ruptured, leaving erythematous, tender raw areas with peeling of the skin. The mother had vesicular eruptions, which started on the second day of delivery and progressed over the next 3 days. Subsequently, similar eruptions were noticed in two of the siblings before affecting the neonate. On the basis of the exposure history and clinical picture, a diagnosis was made of varicella infection with staphylococcal scalded skin syndrome (SSSS). The blood culture and the wound surface culture grew Staphylococcus aureus. Treatment included intravenous fluid, antibiotics, acyclovir and wound care. However, after 72 h of hospitalisation, the neonate first developed shock, refractory to fluid boluses, vasopressors and catecholamine along with other supports; and he then succumbed. In all neonates, staphylococcal infection with varicella can be fatal due to SSSS, the toxic shock syndrome or septicaemia.

Síndrome de la piel escaldada estafilocóccica / Staphylococcal scalded skin syndrome

Lactante de 10 meses de edad, de sexo femenino, procedente del área periurbana de Encarnación, ingresa con lesiones cutáneas de 7 días de evolución tipo ampollosas al inicio, que luego se rompieron dejando un exudado seroso y con costras, simulando una quemadura, rápidamente extensivas a todo el cuerpo. Examen físico: Paciente grave, hipoactiva e irritable, con lesiones descamativas en todo el cuerpo, con pocas lesiones ampollosas, signo de Nikolsky (+), áreas de piel agrietada, otras denudadas, con costras melicéricas. Signos de deshidratación severa y edema de extremidades. Tª 38ºC, Peso 8650 grs., Talla: 69,5 cm, FR 40, FC 150. Se instala vía central, se realizan expansiones con solución fisiológica y se inicia cefotaxima más oxacilina. Estudios auxiliares: Hemograma; GB 22400 por mm3, N 59%, L 41%, Hg 10,7 gr/dl, Hcto. 33 %, Glucosa: 103 mg/dl, Urea: 65mg/dl, Calcio: 5,4 mg/dl, PCR positivo, VDRL no reactivo, HIV negativo. Plaquetas 360000 por mm3, TP 35% de actividad, Albúmina: 2,1g/dl. Orina simple normal. Electrolitos; Sodio146, Potasio: 5,59. Rx de tórax normal. Al 3º día de internación presenta Hg7 gr/dl, Hcto 22 %, requirió transfusión de GRC, albúmina, vitamina K, corrección de Calcio por hipocalcemia y convulsiones. Recibió leche Kass1000 por SNG y se le realizó lavados de piel con solución fisiológica. Paciente muy grave y edematizada los 3 primeros días y luego con mejoría progresiva, fue dada de alta a los 14 días con nivel de albúmina de 3,6 g/dl, sin edemas y con la piel cicatrizada con indicación de emolientes cutáneos y orientación nutricional. El síndrome de la Piel Escaldada es una entidad grave cuando se presenta en la forma generalizada; en este caso la consulta tardía agravó la presentación clínica, ingresando la paciente en estado séptico y con complicaciones severas. El manejo de estos pacientes requiere de una UCIP, nuestra niña fue atendida en una sala de terapia intermedia con los recursos básicos para su tratamiento exitoso.

Introduction: Staphylococcal Scalded Skin Syndrome, or Ritter’s disease, is a severe skin condition caused by the systemic impact of the exfoliative toxin produced by Staphylococcus aureus phage-group II, which causes intradermal separation in the granular layer. The diagnosis is clinical and supported by corroborative skin biopsy that excludes the differential diagnoses of epidermolysis bullosa or Stevens-Johnson syndrome. Case Presentation: Infant, 10 months old, female, from the peri-urban area of Encarnacion, was admitted with skin lesions of 7 days duration, initially of bullous type, which then broke, leaving a serous exudate and crusting, similar to the results of a burn, which quickly extended all over her body. Physical exam: Severely ill patient, underactive and irritable, with scaly lesions all over her body and a few bullous lesions, Nikolsky sign positive, areas of cracked skin, other areas bare with melicerous crusts, signs of severe dehydration, and swelling of limbs. Temp 38º C, weight 8650 grams, height: 69.5 cm, respiratory rate 40, heart rate 150. Installed central venous catheter; skin expansions done with saline, and initiated cefotaxime plus oxacillin. Ancillary studies: CBC: WBC 22400 mm3, neutrophils 59%, lymphocytes 41%, Hgb 10.7 g/dL, HCT 33%, glucose 103 mg/dL, urea 65 mg/dL, calcium 5.4 mg/dL, positive PCR, negative VDRL negative, HIV negative. Platelets 360 000 mm3, prothrombin time (PT) 35% of activity, albumin: 2.1 g/dL. Urinalysis: Electrolytes, Sodium 146, Potassium: 5.59, CXR normal. On the 3rd day of hospitalization HG 7 g/dL, HCT 22%, required transfusion of packed red blood cells PRBC, albumin, vitamin K, Calcium correction due to hypocalcemia and convulsions. Administered “Leche Kas 1000" (hydrolyzed sodium casein formula) administered by NGT and skin washed with saline. Patient was critically ill and edematous for the first 3 days then gradually improved...

Cleavage isn't everything: potential novel mechanisms of exfoliative toxin-mediated blistering.

This Commentary describes breakthroughs in understanding the interactions between desmoglein 1 and plakogloben in staphylococcal-mediated blistering skin diseases.

Bullous congenital ichthyosiform erythroderma: a sporadic case produced by a new KRT10 gene mutation.

Bullous congenital ichthyosiform erythroderma is an unusual type of inherited ichthyosis by mutations in the genes that encode K1 and K10. We report the case of a girl with typical clinical and histopathologic findings of bullous congenital ichthyosiform erythroderma, who was found to have a new mutation in KRT10 gene, Glu445Lys at position 445, affecting the 2B region of the KRT10 protein, the end of the rod domain, where many other keratin mutations associated with hereditary skin disease have been reported. This new mutation contributes to add to the catalog of bullous congenital ichthyosiform erythroderma mutations known.

Staphylococcal scalded skin syndrome and toxic shock syndrome after tooth extraction.

Staphylococcus aureus may cause cutaneous and systemic infections such as staphylococcal scalded skin syndrome (SSSS) and toxic shock syndrome (TSS). Although exfoliative toxins A and B, which cause SSSS, and TSS toxin-1 may be produced by different strains of S aureus, the two syndromes rarely occur simultaneously. We describe a patient admitted to the intensive care department with an exfoliative generalized erythroderma, signs of shock, and biopsy specimen findings consistent with SSSS. This patient presented after a tooth extraction. Symptoms recurred after dismissal, and he was readmitted. Subsequent test results were positive for exfoliative toxin B and TSS toxin-1, suggesting concurrent SSSS and TSS. For patients with acute, exfoliative, generalized erythroderma, TSS and SSSS should be considered. Although rare, it is possible to see both syndromes present concurrently.

Molluscum contagiosum prevents progression of staphylococcal scalded skin syndrome.

Prevention of progression of staphylococcal scalded skin syndrome by molluscum contagiosum indicated a possible interference by viral anti-cytokine molecule such as interleukin-18 binding protein.

Treatment of bullous impetigo and the staphylococcal scalded skin syndrome in infants.

Impetigo is a common, superficial, bacterial infection of the skin characterized by an inflamed and infected epidermis. The rarer variant, bullous impetigo, is characterized by fragile fluid-filled vesicles and flaccid blisters and is invariably caused by pathogenic strains of Staphylococcus aureus. Bullous impetigo is at the mild end of a spectrum of blistering skin diseases caused by a staphylococcal exfoliative toxin that, at the other extreme, is represented by widespread painful blistering and superficial denudation (the staphylococcal scalded skin syndrome). In bullous impetigo, the exfoliative toxins are restricted to the area of infection, and bacteria can be cultured from the blister contents. In staphylococcal scalded skin syndrome the exfoliative toxins are spread hematogenously from a localized source causing widespread epidermal damage at distant sites. Both occur more commonly in children under 5 years of age and particularly in neonates. It is important to swab the skin for bacteriological confirmation and antibiotic sensitivities and, in the case of staphylococcal scalded skin syndrome, to identify the primary focus of infection. Topical therapy should constitute either fusidic acid (Fucidin, Leo Pharma Ltd) as a first-line treatment, or mupirocin (Bactroban, GlaxoSmithKline) in proven cases of bacterial resistance. First-line systemic therapy is oral or intravenous flucloxacillin (Floxapen, GlaxoSmithKline). Nasal swabs from the patient and immediate relatives should be performed to identify asymptomatic nasal carriers of Staphylococcus aureus. In the case of outbreaks on wards and in nurseries, healthcare professionals should also be swabbed.

Neonatal Staphylococcal scalded skin syndrome complicating ileal atresia.

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.

Staphylococcal scalded skin syndrome complicating acute generalized pustular psoriasis.

A 60-year-old woman with psoriasis vulgaris treated with oral cyclosporin and acitretin developed an acute generalized pustular eruption with erythema and associated fever consistent with acute generalized pustular psoriasis. She was admitted to hospital and, despite intravenous fluid replacement, developed acute renal failure. In addition, she developed staphylococcal septicaemia. After transfer to the intensive care unit because of deteriorating renal function, a sudden onset of widespread flaccid blistering (Nikolsky sign positive) and superficial erosions was noted. Histology of a biopsied blister revealed subcorneal splitting of the epidermis consistent with staphylococcal scalded skin syndrome. The patient was treated with intravenous dicloxacillin and the blistering gradually improved over 10 days.

Staphylococcal scalded skin syndrome developing during burn treatment.

We report a case of staphylococcal scalded skin syndrome (SSSS) in a 5-year-old girl who had been hospitalized for burn treatment. When she developed an upper respiratory tract infection, she manifested extensive erythema and exfoliation. There was a purulent discharge from the ulcer caused by the burn, and exfoliation was observed in the surrounding area. Based on clinical symptoms and laboratory data, SSSS was diagnosed and treated with antibiotics, resulting in subsidence. Staphylococcus aureus was isolated from the posterior nasopharynx and the skin erosion and was proved to produce exfoliative toxin A (ETA). Infection from an asymptomatic carrier of an ET-producing strain was suspected, but we failed to identify the origin, in spite of a thorough inspection of the mother, nurses, and physician. SSSS is occasionally reported as a hospital-acquired infection. We should study the frequency of asymptomatic carriers of ET-producing strains so that we can formulate strategies to prevent such infections.

Lesiones cutáneas en pacientes con infecciones sistémicas severas agudas o subagudas / Skin lesions in patients with acute or subacute severe sistemic infections

El diagnóstico y tratamiento temprano son determinantes en las infecciones que ponen en riesgo la vida del paciente. Las lesiones cutáneas, orientan a determinar la causa de la infección y son el resultado de diferentes procesos. Este artículo describe las manifestaciones cutáneas de las infecciones sistémicas severas que permiten en numerosas oportunidades, por sus características típicas, llegar al diagnóstico etiológico de las mismas