Overview
Epidemiology
- Primarily a disease of children
- Incidence (in the United States):
- 8 cases per 1 million children
- 1 case per 1 million adults
- Demographics:
- Most patients are < 2 years of age.
- Rare in adults
- Boys > girls
- Mortality:
- 1%–5% in children
- 50%–60% in adults
Etiology
- Causative organism: exfoliative toxin-producing strains of
Staphylococcus
Staphylococcus
Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora.
Staphylococcus aureus:
- Gram-positive cocci in clusters
- Coagulase-positive
- Exfoliative toxin is produced in approximately 5% of S. aureus strains.
- Most common strains: types 55 and 71
- Transmission: contact with an asymptomatic or colonized carrier
- Common sites of outbreaks:
- ICUs
- Nurseries
- Risk factors:
- Children are more vulnerable because of:
- Lack of immunity
- Immature renal clearance (exotoxin cleared through the kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys)
- Adults:
- Immunodeficiency
- Renal failure
- Malignancy
- Diabetes
- Children are more vulnerable because of:
Microscopic image of Staphylococcus Staphylococcus Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora. Staphylococcus aureus
Image: “ Staphylococcus Staphylococcus Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora. Staphylococcus aureus Gram” by Y Tambe. License: CC BY-SA 3.0Pathophysiology
- Starts as a localized, staphylococcal infection:
- Skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin wounds
- Conjunctivitis Conjunctivitis Conjunctivitis is a common inflammation of the bulbar and/or palpebral conjunctiva. It can be classified into infectious (mostly viral) and noninfectious conjunctivitis, which includes allergic causes. Patients commonly present with red eyes, increased tearing, burning, foreign body sensation, and photophobia. Conjunctivitis
- Pharyngitis Pharyngitis Pharyngitis is an inflammation of the back of the throat (pharynx). Pharyngitis is usually caused by an upper respiratory tract infection, which is viral in most cases. It typically results in a sore throat and fever. Other symptoms may include a runny nose, cough, headache, and hoarseness. Pharyngitis
- Pneumonia Pneumonia Pneumonia or pulmonary inflammation is an acute or chronic inflammation of lung tissue. Causes include infection with bacteria, viruses, or fungi. In more rare cases, pneumonia can also be caused through toxic triggers through inhalation of toxic substances, immunological processes, or in the course of radiotherapy. Pneumonia
- The primary site of infection is not always evident.
-
Staphylococcus
Staphylococcus
Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora.
Staphylococcus produces exotoxin → spread hematogenously
- Two types of exotoxins:
- Exfoliative toxin A
- Exfoliative toxin B
- Exotoxin function:
- Cleaves desmoglein (Dsg) 1 complex in the stratum granulosum
- Disrupts keratinocyte-to-keratinocyte adhesion
- Causes separation and detachment of the superficial epidermis
- Two types of exotoxins:
Pathophysiology of staphylococcal scalded skin syndrome:
Exfoliative toxin cleaves desmoglein (Dsg) 1, disrupting the cell-to-cell adhesion of the stratum granulosum. This detachment of the superficial epidermis causes bullae formation and desquamation.
Clinical Presentation
- Incubation period: 1–10 days
- Prodromal symptoms:
- Fever
- Irritability
- Malaise
- Poor feeding
- Sites of primary infection:
- Infants: umbilical stump or diaper region
- Older children: face
- Frequently not evident
- Cutaneous findings:
- Early manifestations:
- Erythematous macules on the face and flexural surfaces (e.g., axilla Axilla The axilla is a pyramid-shaped space located between the upper thorax and the arm. The axilla has a base, an apex, and 4 walls (anterior, medial, lateral, posterior). The base of the pyramid is made up of the axillary skin. The apex is the axillary inlet, located between the 1st rib, superior border of the scapula, and clavicle. Axilla and Brachial Plexus, inguinal folds, gluteal cleft)
- Skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain
- Erythema spreads diffusely within 24–48 hours.
- Resembles an acute burn
- Flaccid blisters develop, creating a wrinkled appearance.
- Skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin peeling and erosions in areas of friction with red, moist skin underneath
- Positive Nikolsky’s sign: extension of skin blistering or sloughing by applying pressure
- Fissures and crusting around the mouth, eyes, and nose Nose The nose is the human body's primary organ of smell and functions as part of the upper respiratory system. The nose may be best known for inhaling oxygen and exhaling carbon dioxide, but it also contributes to other important functions, such as tasting. The anatomy of the nose can be divided into the external nose and the nasal cavity. Anatomy of the Nose (called “SSSS sad face”)
- Spares the mucosa
- Widespread desquamation may take place within 36–72 hours.
- Healing occurs within 2 weeks.
- Early manifestations:
- The loss of the skin barrier predisposes patients to:
- Dehydration
- Electrolyte imbalances
- Sepsis Sepsis Organ dysfunction resulting from a dysregulated systemic host response to infection separates sepsis from uncomplicated infection. The etiology is mainly bacterial and pneumonia is the most common known source. Patients commonly present with fever, tachycardia, tachypnea, hypotension, and/or altered mentation. Sepsis and Septic Shock
- Hypothermia Hypothermia Hypothermia can be defined as a drop in the core body temperature below 35°C (95°F) and is classified into mild, moderate, severe, and profound forms based on the degree of temperature decrease. Hypothermia
Staphylococcal scalded skin syndrome in a newborn Newborn A neonate, or newborn, is defined as a child less than 28 days old. A thorough physical examination should be performed within the first 24 hours of life to identify abnormalities and improve outcomes by offering timely treatment. Physical Examination of the Newborn:
Image: “Staphylococcal Scalded Skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin Syndrome in Neonate” by Kouakou K, Dainguy ME, Kassi K. License: CC BY 3.0
This image shows diffuse erythema, bullae formation, and skin peeling.
Cutaneous manifestations of SSSS:
Image: “Staphylococcal scalded skin syndrome in a newborn Newborn A neonate, or newborn, is defined as a child less than 28 days old. A thorough physical examination should be performed within the first 24 hours of life to identify abnormalities and improve outcomes by offering timely treatment. Physical Examination of the Newborn” by Jeyakumari D, Gopal R, Eswaran M, Maheshkumar C. License: CC BY 2.0
Skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin peeling and erythema is noted over the thigh Thigh The thigh is the region of the lower limb found between the hip and the knee joint. There is a single bone in the thigh called the femur, which is surrounded by large muscles grouped into 3 fascial compartments. Thigh.
Cutaneous manifestations of SSSS:
Image: “Staphylococcal scalded skin syndrome in a newborn Newborn A neonate, or newborn, is defined as a child less than 28 days old. A thorough physical examination should be performed within the first 24 hours of life to identify abnormalities and improve outcomes by offering timely treatment. Physical Examination of the Newborn” by Jeyakumari D, Gopal R, Eswaran M, Maheshkumar C. License: CC BY 2.0
Erythema, skin peeling, and crusting on the face
Diagnosis and Management
Diagnosis
- Usually diagnosed clinically
- Cultures:
- Used for confirmation and antibiotic susceptibility data
- Should not be taken from bullae: Site will be sterile, since condition is caused by a toxin.
- Options:
- Possible areas of a primary infection
- Conjunctiva
- Nares
- Nasopharynx
- Blood
- Biopsy:
- Can be used for confirmation if the diagnosis is unclear.
- Findings:
- Superficial epidermal splitting
- Noninflammatory
- Supporting workup:
- Leukocytosis
- ↑ ESR
- Basic metabolic panel → assess for dehydration and electrolyte imbalances
- Chest radiography → rule out staphylococcal pneumonia as the originating source
Management
Management of SSSS includes aggressive treatment of primary infection and supportive care.
- Supportive measures:
- Hospital admission:
- Burn unit (if available) for severe cases
- Intensive care unit
- IV fluid hydration
- Monitor and replace electrolytes Electrolytes Electrolytes are mineral salts that dissolve in water and dissociate into charged particles called ions, which can be either be positively (cations) or negatively (anions) charged. Electrolytes are distributed in the extracellular and intracellular compartments in different concentrations. Electrolytes are essential for various basic life-sustaining functions. Electrolytes
- Gentle skin and wound care
- Analgesia
- Hospital admission:
- IV antibiotics should be initiated upon suspicion of the diagnosis:
- For coverage of methicillin-sensitive S. aureus (MSSA):
- Oxacillin
- Nafcillin
- Cefazolin
- For coverage of methicillin-resistant S. aureus (MRSA):
- Vancomycin
- Linezolid
- Clindamycin may be added in severe cases:
- May ↓ toxin production
- Evidence is lacking
- For coverage of methicillin-sensitive S. aureus (MSSA):
Differential Diagnosis
- Stevens–Johnson syndrome: a cutaneous, immune-mediated hypersensitivity reaction that is commonly triggered by medications: Stevens–Johnson syndrome runs on a spectrum with toxic epidermal necrolysis and is characterized by keratinocyte necrosis and separation of the epidermis from the dermis. Patients will present with a flu-like prodrome, followed by cutaneous bullae and sloughing. Unlike SSSS, the mucous membranes are involved. Management is largely supportive, and withdrawal of the causative agent is required.
- Bullous impetigo Impetigo Impetigo is a highly contagious superficial bacterial infection typically caused by Staphylococcus aureus (most common) and Streptococcus pyogenes. Impetigo most commonly presents in children aged 2 to 5 years with lesions that evolve from papules to vesicles to pustules, which eventually break down to form characteristic "honey-colored" crusts. Impetigo: a superficial skin infection caused by Streptococcus Streptococcus Streptococcus is one of the two medically important genera of gram-positive cocci, the other being Staphylococcus. Streptococci are identified as different species on blood agar on the basis of their hemolytic pattern and sensitivity to optochin and bacitracin. There are many pathogenic species of streptococci, including S. pyogenes, S. agalactiae, S. pneumoniae, and the viridans streptococci. Streptococcus or Staphylococcus Staphylococcus Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora. Staphylococcus: Unlike SSSS, the exotoxin remains localized and does not spread. Bullous impetigo Impetigo Impetigo is a highly contagious superficial bacterial infection typically caused by Staphylococcus aureus (most common) and Streptococcus pyogenes. Impetigo most commonly presents in children aged 2 to 5 years with lesions that evolve from papules to vesicles to pustules, which eventually break down to form characteristic "honey-colored" crusts. Impetigo is commonly seen in children and manifests as clusters of vesicles that quickly enlarge and form bullae. After the bullae burst, the exposed bases become covered with a honey-colored crust. The diagnosis is clinical, but cultures may be obtained from the lesions if the patient does not respond to antibiotic therapy.
- Bullous pemphigoid Bullous pemphigoid Bullous pemphigoid and pemphigus vulgaris are two different blistering autoimmune diseases. In bullous pemphigoid, autoantibodies attack the hemidesmosomes, which connect epidermal keratinocytes to the basement membrane. This attack results in large, tense subepidermal blisters. Bullous Pemphigoid and Pemphigus Vulgaris: an autoimmune blistering disease caused by antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins against hemidesmosomes: Patients are usually elderly and present with pruritic, tense, bullous lesions and spared mucosal surfaces. Triggers include medications, trauma, skin conditions, and systemic disease. Biopsy with immunofluorescent staining is used for the diagnosis. Management includes steroids, immunosuppressants Immunosuppressants Immunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response. Immunosuppressants, and antiinflammatory medications.
- Pemphigus vulgaris Pemphigus vulgaris Bullous pemphigoid and pemphigus vulgaris are two different blistering autoimmune diseases. In pemphigus vulgaris, autoantibodies attack the desmosomal proteins, which connect the keratinocytes to one another. This attack results in a more severe, potentially fatal condition with fragile, flaccid blisters, usually with significant mucosal involvement. Bullous Pemphigoid and Pemphigus Vulgaris: an autoimmune disorder due to antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins against desmoglein 1 and 3, causing intraepidermal blistering and erosions of the skin and mucous membranes: Patients will have cutaneous bullae appearing on normal-appearing skin and painful mucocutaneous erosions. Diagnosis is by biopsy with immunofluorescent staining. Blistering occurs lower in the epidermis than occurs with SSSS. Management includes corticosteroids, immunosuppressants Immunosuppressants Immunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response. Immunosuppressants, and IV immunoglobulin.
- Exfoliative dermatitis: a generalized, symmetric, erythematous, scaling rash caused by an underlying cutaneous diseases ( psoriasis Psoriasis Psoriasis is a common T-cell-mediated inflammatory skin condition. The etiology is unknown, but is thought to be due to genetic inheritance and environmental triggers. There are 4 major subtypes, with the most common form being chronic plaque psoriasis. Psoriasis, atopic dermatitis Atopic Dermatitis Atopic dermatitis, also known as eczema, is a chronic, relapsing, pruritic, inflammatory skin disease that occurs more frequently in children, although adults can also be affected. The condition is often associated with elevated serum levels of IgE and a personal or family history of atopy. Skin dryness, erythema, oozing, crusting, and lichenification are present. Atopic Dermatitis (Eczema)), medications, and malignancy (lymphoma): Unlike SSSS, bullae are not typically seen and the Nikolsky sign is negative. Diagnosis is made clinically, and management focuses on treating the underlying cause, withdrawal of implicated medications, and supportive care.
- Toxic shock syndrome Toxic Shock Syndrome Toxic shock syndrome (TSS) is an acute, multi-systemic disease caused by the toxin-producing bacteria, Staphylococcus aureus and Streptococcus pyogenes. Staphylococcal TSS is more common and associated with tampons and nasal packing. Toxic Shock Syndrome: a systemic syndrome caused by Staphylococcus Staphylococcus Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora. Staphylococcus or Streptococcus Streptococcus Streptococcus is one of the two medically important genera of gram-positive cocci, the other being Staphylococcus. Streptococci are identified as different species on blood agar on the basis of their hemolytic pattern and sensitivity to optochin and bacitracin. There are many pathogenic species of streptococci, including S. pyogenes, S. agalactiae, S. pneumoniae, and the viridans streptococci. Streptococcus endotoxins: Patients present with fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever, shock, and multisystem organ dysfunction, which are not seen in SSSS. Cutaneous manifestations include a diffuse, erythematous rash and desquamation. Diagnosis is based on blood culture results, the clinical history, and the exam. Management includes hemodynamic support, fluid resuscitation, and antibiotics.
| Number | Other names for the disease | Etiology | Description |
|---|---|---|---|
| 1st disease |
|
Measles Measles Measles (also known as rubeola) is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae. It is highly contagious and spreads by respiratory droplets or direct-contact transmission from an infected person. Typically a disease of childhood, measles classically starts with cough, coryza, and conjunctivitis, followed by a maculopapular rash. Measles Virus morbillivirus |
|
| 2nd disease |
|
Streptococcus Streptococcus Streptococcus is one of the two medically important genera of gram-positive cocci, the other being Staphylococcus. Streptococci are identified as different species on blood agar on the basis of their hemolytic pattern and sensitivity to optochin and bacitracin. There are many pathogenic species of streptococci, including S. pyogenes, S. agalactiae, S. pneumoniae, and the viridans streptococci. Streptococcus pyogenes |
|
| 3rd disease |
|
Rubella virus Rubella Virus Rubella (also known as German measles or three-day measles) is caused by a single-stranded, positive-sense RNA virus of the Togaviridae family and the Rubivirus genus. Rubella only infects humans and spreads prenatally via vertical transmission or postnatally via droplet contact. Rubella Virus |
|
| 4th disease |
|
Due to Staphylococcus Staphylococcus Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora. Staphylococcus aureus strains that make epidermolytic (exfoliative) toxin |
|
| 5th disease | Erythema infectiosum Erythema infectiosum Erythema infectiosum is a rash illness caused by parvovirus B19. Erythema infectiosum is also known as fifth disease, being 5th in the historical list of rash-causing childhood infectious diseases: measles (1st), scarlet fever (2nd), rubella (3rd), Dukes' disease (4th), and roseola (6th). Erythema Infectiosum | Erythrovirus or parvovirus B19 Parvovirus B19 Primate erythroparvovirus 1 (generally referred to as parvovirus B19, B19 virus, or sometimes erythrovirus B19) ranks among the smallest DNA viruses. Parvovirus B19 is of the family Parvoviridae and genus Erythrovirus. In immunocompetent humans, parvovirus B19 classically results in erythema infectiosum (5th disease) or "slapped cheek syndrome." Parvovirus B19 (primate erythroparvovirus 1) |
|
| 6th disease |
|
HHV-6B or HHV-7 HHV-7 Human herpesvirus (HHV)-6 and HHV-7 are similar double-stranded DNA viruses belonging to the Herpesviridae family. Human herpesviruses are ubiquitous and infections are commonly contracted during childhood. Human Herpesvirus 6 & 7 |
|
References
- Bukowski M, Wladyka B, Dubin G. (2010). Exfoliative toxins of Staphylococcus Staphylococcus Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora. Staphylococcus aureus. Toxins 2(5):1148–1165. https://pubmed.ncbi.nlm.nih.gov/22069631/
- McMahon P. (2020). Staphylococcal scalded skin syndrome. UpToDate. Retrieved February 1, 2021, from: https://www.uptodate.com/contents/staphylococcal-scalded-skin-syndrome
- Arora P, et al. (2011). Staphylococcal scalded skin syndrome in a preterm newborn Newborn A neonate, or newborn, is defined as a child less than 28 days old. A thorough physical examination should be performed within the first 24 hours of life to identify abnormalities and improve outcomes by offering timely treatment. Physical Examination of the Newborn presenting within first 24 h of life. BMJ Case Rep. https://pubmed.ncbi.nlm.nih.gov/22670002/
- Neubauer HC, et al. (2018). Variation in diagnostic test use and associated outcomes in staphylococcal scalded skin syndrome at children’s hospitals. Hosp Pediatr 8(9), 530–537. https://pubmed.ncbi.nlm.nih.gov/30139766/
- Staiman A, Hsu DY, Silverberg JI. (2018). Epidemiology of staphylococcal scalded skin syndrome in U.S. children. Br J Dermatol 178(3):704–708. https://pubmed.ncbi.nlm.nih.gov/29077993/
- Dhar AD. (2019). Staphylococcal scalded skin syndrome. MSD Manual Professional Version. Retrieved February 8, 2021, from https://www.msdmanuals.com/professional/dermatologic-disorders/bacterial-skin-infections/staphylococcal-scalded-skin-syndrome
- King RW, Carone HL, de Saint Victor PR. (2019). Staphylococcal scalded skin syndrome (SSSS). In Taylor JP III (Ed.). Medscape. Retrieved February 8, 2021, from https://emedicine.medscape.com/article/788199-overview
