Staphylococcal scalded skin syndrome (SSSS) is a

dermatological Dermatology is the branch of medicine dealing with the skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medical ...

condition caused by ''

Staphylococcus aureus ''Staphylococcus aureus'' is a Gram-positive spherically shaped bacterium, a member of the Bacillota, and is a usual member of the microbiota of the body, frequently found in the upper respiratory tract and on the skin. It is often positive ...

''.

Signs and symptoms

The disease presents with the widespread formation of fluid-filled blisters that are thin walled and easily ruptured, and the patient can be positive for

Nikolsky's sign Nikolsky's sign is a clinical dermatological sign, named after Pyotr Nikolsky (1858–1940), a Russian physician who trained and worked in the Russian Empire. The sign is present when slight rubbing of the skin results in exfoliation of the outermo ...

. Ritter's disease of the newborn is the most severe form of SSSS, with similar signs and symptoms. SSSS often includes a widespread painful

erythroderma Erythroderma is an inflammatory skin disease with redness and scaling that affects nearly the entire cutaneous surface.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. p. 436. . This term applie ...

, often involving the face, diaper, and other

intertriginous In medicine, an intertriginous area is where two skin areas may touch or rub together. Examples of intertriginous areas are the axilla of the arm, the anogenital region, skin folds of the breasts and between digits. Intertriginous areas are known t ...

areas. Extensive areas of

desquamation Desquamation occurs when the outermost layer of a tissue, such as the skin, is shed. The term is . Physiologic desquamation Keratinocytes are the predominant cells of the epidermis, the outermost layer of the skin. Living keratinocytes reside in ...

might be present. Perioral crusting and fissuring are seen early in the course. Unlike

toxic epidermal necrolysis Toxic epidermal necrolysis (TEN) is a type of severe skin reaction. Together with Stevens–Johnson syndrome (SJS) it forms a spectrum of disease, with TEN being more severe. Early symptoms include fever and flu-like symptoms. A few days later th ...

, SSSS spares the mucous membranes. It is most common in children under 6 years, but can be seen in adults who are

immunosuppressed Immunosuppression is a reduction of the activation or efficacy of the immune system. Some portions of the immune system itself have immunosuppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse reacti ...

or have

kidney failure Kidney failure, also known as end-stage kidney disease, is a medical condition in which the kidneys can no longer adequately filter waste products from the blood, functioning at less than 15% of normal levels. Kidney failure is classified as eit ...

Pathophysiology

The

syndrome A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek σύνδρομον, meaning "concurrence". When a syndrome is paired ...

is induced by epidermolytic

exotoxin An exotoxin is a toxin secreted by bacteria. An exotoxin can cause damage to the host by destroying cells or disrupting normal cellular metabolism. They are highly potent and can cause major damage to the host. Exotoxins may be secreted, or, simi ...

s (

exfoliatin Exfoliatin is a '' Staphylococcus aureus'' exotoxin that causes a blistering of the skin known as staphylococcal scalded skin syndrome, usually in infants. Exfoliatins are glutamate-specific serine proteases highly specific to desmoglein I, a ...

) A and B, which are released by ''S. aureus'' and cause detachment within the epidermal layer, by breaking down the

desmosome A desmosome (; "binding body"), also known as a macula adherens (plural: maculae adherentes) (Latin for ''adhering spot''), is a cell structure specialized for cell-to-cell adhesion. A type of junctional complex, they are localized spot-like adh ...

s. One of the exotoxins is encoded on the bacterial chromosome, while the other is encoded on a

plasmid A plasmid is a small, extrachromosomal DNA molecule within a cell that is physically separated from chromosomal DNA and can replicate independently. They are most commonly found as small circular, double-stranded DNA molecules in bacteria; how ...

. These exotoxins are proteases that cleave

desmoglein-1 Desmoglein-1 is a protein that in humans is encoded by the ''DSG1'' gene. Desmoglein-1 is expressed everywhere in the skin epidermis, but mainly it is expressed in the superficial upper layers of the skin epidermis. Function Desmosomes are cell ...

, which normally holds the granulosum and spinosum layers together, similar to the pathophysiology of the autoimmune skin disease,

pemphigus vulgaris Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. Pemphigus was derived from the Greek word ''pemphix'', meaning blister. It is classified as a type II hypersensitivity reaction in which antibodies ...

Diagnosis

SSSS is a clinical diagnosis. This is sometimes confirmed by isolation of ''S. aureus'' from blood, mucous membranes, or skin biopsy; however, these are often negative. Skin biopsy may show separation of the superficial layer of the epidermis (intraepidermal separation), differentiating SSSS from TEN, wherein the separation occurs at the dermo-epidermal junction (subepidermal separation). SSSS may be difficult to distinguish from toxic epidermal necrolysis and pustular psoriasis.

Treatment

The mainstay of treatment for SSSS is supportive care along with eradication of the primary infection. Conservative measures include rehydration, antipyretics (e.g.,

ibuprofen Ibuprofen is a nonsteroidal anti-inflammatory drug (NSAID) that is used for treating pain, fever, and inflammation. This includes painful menstrual periods, migraines, and rheumatoid arthritis. It may also be used to close a patent ductus arte ...

paracetamol Paracetamol, also known as acetaminophen, is a medication used to treat fever and mild to moderate pain. Common brand names include Tylenol and Panadol. At a standard dose, paracetamol only slightly decreases body temperature; it is inferior ...

), management of thermal burns, and stabilization. Parenteral antibiotics to cover ''S. aureus'' should be administered. Most strains of ''S. aureus'' implicated in SSSS have

penicillinase Beta-lactamases, (β-lactamases) are enzymes () produced by bacteria that provide multi-resistance to beta-lactam antibiotics such as penicillins, cephalosporins, cephamycins, monobactams and carbapenems ( ertapenem), although carbapenems ...

s, so are penicillin resistant. Therefore, treatment with

nafcillin Nafcillin sodium is a narrow-spectrum beta-lactam antibiotic of the penicillin class. As a beta-lactamase-resistant penicillin, it is used to treat infections caused by Gram-positive bacteria, in particular, species of staphylococci that are res ...

oxacillin Oxacillin (trade name Bactocill) is a narrow-spectrum beta-lactam antibiotic of the penicillin class developed by Beecham. It was patented in 1960 and approved for medical use in 1962. Medical uses Oxacillin is a penicillinase-resistant β-la ...

, or

vancomycin Vancomycin is a glycopeptide antibiotic medication used to treat a number of bacterial infections. It is recommended intravenously as a treatment for complicated skin infections, bloodstream infections, endocarditis, bone and joint infections, ...

is typically indicated.

Clindamycin Clindamycin is an antibiotic medication used for the treatment of a number of bacterial infections, including osteomyelitis (bone) or joint infections, pelvic inflammatory disease, strep throat, pneumonia, acute otitis media (middle ear infec ...

is sometimes also used because of its inhibition of exotoxins.

Prognosis

The prognosis of SSSS in children is excellent, with complete resolution within 10 days of treatment, and without significant scarring. However, SSSS must be differentiated carefully from

, which carries a poor prognosis. The prognosis in adults is generally much worse, and depends upon various factors such as time to treatment, host immunity, and comorbidities.

History

The clinical features were first described in 1878 by Baron Gottfried Ritter von Rittershain, who observed 297 cases among children in a single Czechoslovakian children's home over a 10-year period. In 1885, Nil Filatow, and in 1894, Clement Dukes, described an exanthematous disease which they thought to be a form of rubella, but in 1900, Dukes identified it to be a separate entity which came to be known by the names

Dukes' disease Dukes' disease, named after Clement Dukes, also known as fourth disease or Filatov-Dukes' disease (after Nil Filatov), is an exanthem. It is distinguished from measles or forms of rubella, though it was considered as a form of viral rash. Altho ...

, Filatov's disease, or fourth disease. Although Dukes identified it as a separate entity, it is thought not to be different from

scarlet fever Scarlet fever, also known as Scarlatina, is an infectious disease caused by ''Streptococcus pyogenes'' a Group A streptococcus (GAS). The infection is a type of Group A streptococcal infection (Group A strep). It most commonly affects childr ...

caused by staphylococcal exotoxin after Keith Powell proposed equating it with the condition currently known as staphylococcal scalded skin syndrome in 1979.

References

External links

{{Cutaneous infections Bacterial diseases Bacterium-related cutaneous conditions Syndromes caused by microbes