Staphylococcal scalded skin syndrome, SSSS, also known as Pemphigus neonatorum or Ritter's disease, or Localized bullous impetigo is a dermatological

Dermatology

Dermatology is the branch of medicine dealing with the skin and its diseases, a unique specialty with both medical and surgical aspects. A dermatologist takes care of diseases, in the widest sense, and some cosmetic problems of the skin, scalp, hair, and nails....

 condition caused by Staphylococcus aureus

Staphylococcus aureus

Staphylococcus aureus is a facultative anaerobic Gram-positive coccal bacterium. It is frequently found as part of the normal skin flora on the skin and nasal passages. It is estimated that 20% of the human population are long-term carriers of S. aureus. S. aureus is the most common species of...

.

Pathophysiology

The syndrome

Syndrome

In medicine and psychology, a syndrome is the association of several clinically recognizable features, signs , symptoms , phenomena or characteristics that often occur together, so that the presence of one or more features alerts the physician to the possible presence of the others...

 is induced by epidermolytic exotoxin

Exotoxin

An exotoxin is a toxin excreted by a microorganism, like bacteria, fungi, algae, and protozoa. 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...

s (exfoliatin

Exfoliatin

Exfoliatin is a Staphylococcus aureus exotoxin that causes a blistering of the skin known as staphylococcal scalded skin syndrome.Exfoliatins are glutamate-specific serine proteases highly specific to the cadherin desmoglein I, an adhesion protein in the desmosomes of the stratum granulosum that...

) A and B, which are released by S. aureus and cause detachment within the epidermal layer. One of the exotoxins is produced by the bacterial chromosome, while the other is produced by a plasmid

Plasmid

In microbiology and genetics, a plasmid is a DNA molecule that is separate from, and can replicate independently of, the chromosomal DNA. They are double-stranded and, in many cases, circular...

. (Bacterial plasmids are pieces of self-replicating DNA that often code for secondary characteristics, such as antibiotic resistance, and toxin production.) These exotoxins are proteases that cleave desmoglein-1, which normally holds the granulosum

Stratum granulosum

The stratum granulosum is a thin layer of cells in the epidermis. Keratinocytes migrating from the underlying stratum spinosum become known as granular cells in this layer...

 and spinosum

Stratum spinosum

The stratum spinosum is a layer of the epidermis found between the stratum granulosum and stratum basale. This layer is also referred to as the "spinous" or "prickle-cell" layer. This appearance is due to desmosomal connections of adjacent cells. Keratinization begins in the stratum spinosum....

 layers together.

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

Nikolsky's sign is a clinical dermatological sign, named after the Russian physician Pyotr Nikolsky . The sign is positive when slight rubbing of the skin results in exfoliation of the outermost layer....

. Ritter's Disease of the Newborn is the most severe form of SSSS with similar signs and symptoms.

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.