Staphylococcal scalded skin syndrome: diagnosis and management in children and adults

Staphylococcal scalded skin syndrome is a potentially life‐threatening disorder caused most often by a phage group II Staphylococcus aureus infection. Staphylococcal scalded skin syndrome is more common in newborns than in adults. Staphylococcal scalded skin syndrome tends to appear abruptly with diffuse erythema and fever. The diagnosis can be confirmed by a skin biopsy specimen, which can be expedited by frozen section processing, as staphylococcal scalded skin syndrome should be distinguished from life threatening toxic epidermal necrolysis. Histologically, the superficial epidermis is detached, the separation level being at the granular layer. The diffuse skin loss is due to a circulating bacterial exotoxin. The aetiological exfoliating toxin is a serine protease that splits only desmoglein 1. The exfoliative toxins are spread haematogenously from a localized source of infection, causing widespread epidermal damage at distant sites. Sepsis and pneumonia are the most feared complications. The purpose of this review is to summarize advances in understanding of this serious disorder and provide therapeutic options for both paediatric and adult patients. Recent epidemiological studies have demonstrated that paediatric patients have an increased incidence of Staphylococcal scalded skin syndrome during the summer and autumn. Mortality is less than 10% in children, but is between 40% and 63% in adults, despite antibacterial therapy. Previously, intravenous immunoglobulin had been recommended to combat Staphylococcal scalded skin syndrome, but a recent study associates its use with prolonged hospitalization.     

Staphylococcal scalded skin syndrome in an adult

We report a case of a 77-year-old man with staphylococcal scalded skin syndrome (SSSS). The patient, who had no physical abnormalities other than herpes zoster, showed intact humoral and cellular immunity. A 3- and 1-phage group strain of Staphylococcus aureus was isolated from an unruptured pustule of the herpes zoster lesion, but not from the throat. Inoculation of the isolated bacterium into newborn mice produced a characteristic Nikolsky sign. Histological diagnosis of SSSS was established by findings of intraepithelial cleavage through the stratum granulosum and a lack of inflammatory cells in both dermis and epidermis. After a ten-day course of oral cefaclor (250 mg every 8 hours) and topical triamcinolone acetonide containing fradiomycin and gramycidin J, the patient recovered completely. The patient left the hospital without any scars other than those of herpes zoster.     

Staphylococcal scalded skin syndrome in an adult.

The staphylococcal form of toxic epidermal necrolysis or staphylococcal scalded skin syndrome (SSSS) is exceptionally seen in an adult patient. We report a case of SSSS in a 21-year-old male who suffered from a fulminant pneumopathia due to a phage group II Staphylococcus aureus. The onset of that clinical picture in an adult patient is unusual and could be due to a deficient immunity, as previous case reports have emphasized. Unfortunately, the sudden death of our patient did not allow us to investigate his immunological defences.     

Staphylococcal scalded skin syndrome with prosthetic valve endocarditis

We report staphylococcal scalded skin syndrome (SSSS) in a 67-year-old man. He showed diffuse erythema with erosion on his face and erythema with giant desquamation on his neck, axilla, genitalia, chest and abdomen 39 days after a coronary artery bypass graft and aortic valve replacement. He died of cardiac rupture caused by myocardial necrosis, and autopsy findings demonstrated prosthetic valve endocarditis due to a strain of exfoliative toxin-B producing methicillin-resistant Staphylococcus aureus. To the best of our knowledge, this is the first case of SSSS caused by prosthetic valve endocarditis.     

Staphylococcal scalded skin syndrome in a healthy adult

We report a case of staphylococcal scalded skin syndrome (SSSS) in a 65-year-old healthy woman. Fever, purulent conjunctivitis, and exfoliation of the skin in the gluteal region were noted. A scarlatiniform rash was observed on the body, and this erythema was followed by generalized desquamation. Staphylococcus aureus was isolated from her eye discharge, posterior nasopharynx, and the erosive surface of the skin. All the investigated strains produced exfoliative toxin B, but none produced toxic shock toxin-1 (TSST-1) or enterotoxin. The patient was treated with antibiotics and fluid supplementation, resulting in subsidence. This case is thought to have been caused by an abortive form of SSSS or a scarlatiniform variant, which is very rare in healthy adults.     

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.     

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.     

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 in an HIV-1 seropositive man

Summary The staphylococcal scalded skin syndrome (SSSS) is very rare in adults. Renal impairment and immunocompromise are predisposing causes. We report a 38-year-old HIV-1 seropositive intravenous drug abuser who developed SSSS due to staphylococcal pneumonia. An exfoliating toxin releasing Staphylococcus aureus, phage type II type 3C, was isolated from the sputum and from blood cultures. This is the third case of adult SSSS to be reported in the context of HIV disease.     

Staphylococcal scalded skin syndrome in an adult with Hodgkin's disease.

An elderly man with Hodgkin's disease who was receiving multiple drug chemotherapy became septic and a wide spread bullous eruption developed. Intraepidermal cleavage on skin biopsy supported a diagnosis of the staphylococcal scalded skin syndrome (SSSS) type of toxic epidermal necrolysis. Blood cultures confirmed a staphylococcal septicemia. Occurrence of this syndrome in an adult is unusual. A review of the literature on SSSS indicates an increased mortality when adults are compared with children with this syndrome.     

Staphylococcal scalded skin syndrome in 2 adults with acute kidney failure

We report on two cases of staphylococcal scalded skin syndrome (SSSS) in adults. In contrast to the previously reported cases our patients were neither immunosuppressed nor haemodialysed, nor did they have cancer. In both cases, acute renal failure in the presence of a rather banal staphylococcal infection preceded the SSSS. The pathogenic role of acute renal failure in the development of SSSS is discussed.