Stevens-Johnson Syndrome

Stevens-Johnson syndrome (SJS) is a cutaneous, immune-mediated hypersensitivity reaction that is commonly triggered by medications, including antiepileptics and antibiotics. The condition runs on a spectrum with toxic epidermal necrolysis (TEN) based on the amount of body surface area (BSA) involved. Stevens-Johnson syndrome 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 on the face, thorax, and mucous membranes. Stevens-Johnson syndrome is considered a medical emergency, and management is largely supportive. Withdrawal of the causative agent is required. Monitoring for, and treating, superinfection is essential due to the high risk of associated death in these patients.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Table of Contents

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Overview

Classification

  • Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions. 
  • Considered a continuum of the same disease
  • Classified based on the percentage of body surface affected by blisters, erosions, and 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 detachment:
Subtype Involved BSA

Stevens-Johnson syndrome

10% of BSA

Overlapping SJS/TEN

10%‒30% of BSA

Toxic epidermal necrolysis

30% of BSA

BSA: body surface area
SJS: Stevens-Johnson syndrome
TEN: toxic epidermal necrolysis

Epidemiology

  • Incidence: 
    • 1–7 cases per million people per year
    • Higher in patients with HIV and active cancer
    • SJS is more common.
  • Mortality rate: 
    • SJS: 10%
    • TEN: 50%
  • More common in women than men, with a ratio of 2:1
  • Can occur at any age

Etiology

  • Medications 
    • Approximately 70% of cases
    • Occurs within 8 weeks of medication onset
  • Infections 
    • 2nd-most common trigger
    • Frequent cause in children
  • Idiopathic
    • > 1/3 of cases
  • Genetics Genetics Genetics is the study of genes and their functions and behaviors. Basic Terms of Genetics
    • Some HLA types are associated with an increased risk.
  • Other risk factors 
    • Malignancy 
    • Lupus 
    • Ultraviolet light stimulus 
    • Radiation therapy

The following table lists the major common medication and infectious causes of SJS/TEN:

Types Examples

Medications

Antiepileptics

Lamotrigine, phenobarbital, carbamazepine, valproate, phenytoin

Sulfa

Cotrimoxazole, sulfasalazine

Other antibiotics

Aminopenicillins, fluoroquinolones Fluoroquinolones Fluoroquinolones are a group of broad-spectrum, bactericidal antibiotics inhibiting bacterial DNA replication. Fluoroquinolones cover gram-negative, anaerobic, and atypical organisms, as well as some gram-positive and multidrug-resistant (MDR) organisms. Fluoroquinolones, cephalosporins Cephalosporins Cephalosporins are a group of bactericidal beta-lactam antibiotics (similar to penicillins) that exert their effects by preventing bacteria from producing their cell walls, ultimately leading to cell death. Cephalosporins are categorized by generation and all drug names begin with "cef-" or "ceph-." Cephalosporins

NSAIDs

Meloxicam, piroxicam

Antiretrovirals

Nevirapine

Miscellaneous

Allopurinol, chlormezanone

Infectious

Viral

Herpes simplex virus Virus Viruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology: Overview, HIV, coxsackievirus Coxsackievirus Coxsackievirus is a member of a family of viruses called Picornaviridae and the genus Enterovirus. Coxsackieviruses are single-stranded, positive-sense RNA viruses, and are divided into coxsackie group A and B viruses. Both groups of viruses cause upper respiratory infections, rashes, aseptic meningitis, or encephalitis. Coxsackievirus, hepatitis, influenza Influenza Influenza viruses are members of the Orthomyxoviridae family and the causative organisms of influenza, a highly contagious febrile respiratory disease. There are 3 primary influenza viruses (A, B, and C) and various subtypes, which are classified based on their virulent surface antigens, hemagglutinin (HA) and neuraminidase (NA). Influenza typically presents with a fever, myalgia, headache, and symptoms of an upper respiratory infection. Influenza Viruses/Influenza, mumps Mumps Mumps is caused by a single-stranded, linear, negative-sense RNA virus of the family Paramyxoviridae. Mumps is typically a disease of childhood, which manifests initially with fever, muscle pain, headache, poor appetite, and a general feeling of malaise, and is classically followed by parotitis. Mumps Virus/Mumps, Epstein-Barr virus Epstein-Barr Virus Epstein-Barr virus (EBV) is a linear, double-stranded DNA virus belonging to the Herpesviridae family. This highly prevalent virus is mostly transmitted through contact with oropharyngeal secretions from an infected individual. The virus can infect epithelial cells and B lymphocytes, where it can undergo lytic replication or latency. Epstein-Barr Virus, enteroviruses

Bacterial

Group A beta-hemolytic streptococci, brucellosis Brucellosis Brucellosis (also known as undulant fever, Mediterranean fever, or Malta fever) is a zoonotic infection that spreads predominantly through ingestion of unpasteurized dairy products or direct contact with infected animal products. Clinical manifestations include fever, arthralgias, malaise, lymphadenopathy, and hepatosplenomegaly. Brucella/Brucellosis, mycobacteria Mycobacteria Mycobacterium is a genus of the family Mycobacteriaceae in the phylum Actinobacteria. Mycobacteria comprise more than 150 species of facultative intracellular bacilli that are mostly obligate aerobes. Mycobacteria are responsible for multiple human infections including serious diseases, such as tuberculosis (M. tuberculosis), leprosy (M. leprae), and M. avium complex infections. Mycobacterium, Mycoplasma Mycoplasma Mycoplasma is a species of pleomorphic bacteria that lack a cell wall, which makes them difficult to target with conventional antibiotics and causes them to not gram stain well. Mycoplasma bacteria commonly target the respiratory and urogenital epithelium. Mycoplasma pneumoniae (M. pneumoniae), the causative agent of atypical or "walking" pneumonia. Mycoplasma pneumoniae, rickettsia Rickettsia Rickettsiae are a diverse collection of obligate intracellular, gram-negative bacteria that have a tropism for vascular endothelial cells. The vectors for transmission vary by species but include ticks, fleas, mites, and lice. Rickettsia, tularemia
NSAIDs: Nonsteroidal anti-inflammatory drugs

Pathogenesis

The exact mechanism is unknown, but there are several theories:

  1. Drug, or infectious, antigen in the 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 tissue → stimulates cytotoxic T cells T cells T cells, also called T lymphocytes, are important components of the adaptive immune system. Production starts from the hematopoietic stem cells in the bone marrow, from which T-cell progenitor cells arise. These cells migrate to the thymus for further maturation. T Cells, natural killer T cells T cells T cells, also called T lymphocytes, are important components of the adaptive immune system. Production starts from the hematopoietic stem cells in the bone marrow, from which T-cell progenitor cells arise. These cells migrate to the thymus for further maturation. T Cells (NKT), and natural killer (NK) cells → granulysin release → keratinocyte death
  2. Apoptosis of keratinocytes → epidermal separation from the dermis → characteristic 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 detachment of SJS/TEN
  3. Dying cells and necrosis → ↑ antigen load → triggers T cells T cells T cells, also called T lymphocytes, are important components of the adaptive immune system. Production starts from the hematopoietic stem cells in the bone marrow, from which T-cell progenitor cells arise. These cells migrate to the thymus for further maturation. T Cells to continue the inflammatory response → development of fluid-filled blisters 

This impaired 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 integrity can lead to:

  • Water loss
  • Secondary bacterial infections and 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
Pathophysiology in stevens-johnson syndrome and toxic epidermal necrolysis

Schematic showing how a peptide antigen (in this case, from a drug) presented on keratinocytes can lead to a cytotoxic inflammatory response resulting in granulysin release, keratinocyte apoptosis and necrosis, detachment of the epidermis, and blister formation in SJS and TEN.

Image by Lecturio.

Clinical Presentation

Clinical manifestations

Prodrome: 

  • Fever (often > 39°C)
  • Myalgia and arthralgia
  • Keratoconjunctivitis ( inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation of the cornea and conjunctiva)
  • Sore throat
  • Headache
  • Malaise

Acute phase: 

  • 1‒3 days after the prodrome
  • Lasts 8‒12 days
  • Cutaneous lesions
    • Begin as ill-defined, coalescing macules with purpuric centers or diffuse erythema
      • Start on the face and thorax, then spread symmetrically
      • Typically spare scalp, palms, and soles
      • Tender to touch
    • Vesicles and bullae form as the disease progresses
    • Skin begins to slough within days
    • Nikolsky’s sign: extension of 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 sloughing by applying pressure
  • Mucosal lesions 
    • Occur in approximately 90% of cases
    • Includes oral, pharyngeal, ocular, and urogenital erosions

Complications

  • Significant fluid loss 
    • Severe dehydration Dehydration Volume status is a balance between water and solutes, the majority of which is Na. Volume depletion refers to a loss of both water and Na, whereas dehydration refers only to a loss of water. Dehydration is primarily caused by decreased water intake and presents with increased thirst and can progress to altered mental status and low blood pressure if severe. Volume Depletion and Dehydration 
    • Hypovolemic shock Shock Shock is a life-threatening condition associated with impaired circulation that results in tissue hypoxia. The different types of shock are based on the underlying cause: distributive (↑ cardiac output (CO), ↓ systemic vascular resistance (SVR)), cardiogenic (↓ CO, ↑ SVR), hypovolemic (↓ CO, ↑ SVR), obstructive (↓ CO), and mixed. Types of Shock 
    • Renal failure
  • Bacterial infection 
    • Sepsis and septic shock Sepsis and Septic shock 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. Septic shock is diagnosed during treatment when vasopressors are necessary to control hypotension. Sepsis and septic shock are medical emergencies and antibiotics are given within an hour of diagnosis. Sepsis and Septic Shock are the main cause of death in SJS/TEN patients.
    • Most often 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 aureus and Pseudomonas Pseudomonas Pseudomonas is a non-lactose-fermenting, gram-negative bacillus that produces pyocyanin, which gives it a characteristic blue-green color. Pseudomonas is found ubiquitously in the environment, as well as in moist reservoirs, such as hospital sinks and respiratory equipment. Pseudomonas aeruginosa
  • Tracheobronchial epithelial involvement  
    • Pneumonia 
    • Interstitial pneumonitis 
    • Acute respiratory distress syndrome Acute Respiratory Distress Syndrome Acute respiratory distress syndrome is characterized by the sudden onset of hypoxemia and bilateral pulmonary edema without cardiac failure. Sepsis is the most common cause of ARDS. The underlying mechanism and histologic correlate is diffuse alveolar damage (DAD). Acute Respiratory Distress Syndrome 
  • Protein loss: edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema
  • Electrolyte imbalances
  • Epithelial necrosis of the gastrointestinal tract  
    • Diarrhea Diarrhea Diarrhea is defined as ≥ 3 watery or loose stools in a 24-hour period. There are a multitude of etiologies, which can be classified based on the underlying mechanism of disease. The duration of symptoms (acute or chronic) and characteristics of the stools (e.g., watery, bloody, steatorrheic, mucoid) can help guide further diagnostic evaluation. Diarrhea 
    • Melena 
    • Colonic perforation 
    • Small bowel intussusception Intussusception Intussusception occurs when a part of the intestine (intussusceptum) telescopes into another part (intussuscipiens) of the intestine. The condition can cause obstruction and, if untreated, progress to bowel ischemia. Intussusception is most common in the pediatric population, but is occasionally encountered in adults. Intussusception

Diagnosis

The diagnosis is clinical, based on history and physical exam findings.

  • Skin biopsy 
    • Not required for diagnosis, but can confirm and rule out other conditions
    • Keratinocyte necrosis is the hallmark finding.
      • Can be partial or full-thickness
      • Apoptotic keratinocytes are scattered in the basal layer of the epidermis in early lesions.
      • Full-thickness epidermal necrosis and subepidermal bullae later in the disease
    • Lymphocytic inflammatory infiltration
    • Direct immunofluorescence is negative.
  • Supporting workup 
    • Aids AIDS Chronic HIV infection and depletion of CD4 cells eventually results in acquired immunodeficiency syndrome (AIDS), which can be diagnosed by the presence of certain opportunistic diseases called AIDS-defining conditions. These conditions include a wide spectrum of bacterial, viral, fungal, and parasitic infections as well as several malignancies and generalized conditions. HIV Infection and AIDS in monitoring treatment and complications
    • Basic metabolic panel → evaluate for electrolyte imbalance and renal failure
    • Complete blood count → significant leukocytosis may signal an infection
    • Bacterial and fungal cultures → bacterial superinfection and 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
    • Chest radiograph → 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 or interstitial pneumonitis
Curcumin in stevens-johnsons syndrome culprit or bystander1

Histopathology of a 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 biopsy taken from a patient with SJS/TEN showing characteristic epidermal detachment and lymphocyte infiltration in the dermis

Image: “Curcumin in stevens-johnsons syndrome: culprit or bystander?” by Irani C, Haddad F, Maalouly G, Nemnoum R. License: CC BY 2.0

Treatment

  • Immediate hospital admission
    • Patients typically require intensive care or a burn unit.
    • SCORTEN score (score of toxic epidermal necrolysis) is calculated to determine severity, prognosis, and appropriate setting for management (see table below).
  • Withdrawal of causative agent is required.
  • Supportive care:
    • Wound care 
      • Debridement 
      • Moisturizers 
      • Antibacterial ointments
    • Fluid and electrolyte management
    • Nutritional support 
      • Parenteral nutrition for those with dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming "stuck." Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia and odynophagia 
      • Transition to oral feeds when tolerated
    • Ocular care 
      • Ophthalmology consultation
      • Corticosteroid and antibiotic eye drops 
      • Artificial tears for lubrication 
    • Temperature management
    • Pain control 
      • Acetaminophen Acetaminophen Acetaminophen is an over-the-counter nonopioid analgesic and antipyretic medication and the most commonly used analgesic worldwide. Despite the widespread use of acetaminophen, its mechanism of action is not entirely understood. Acetaminophen and ibuprofen for mild 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 
      • Opioids Opioids Opiates are drugs that are derived from the sap of the opium poppy. Opiates have been used since antiquity for the relief of acute severe pain. Opioids are synthetic opiates with properties that are substantially similar to those of opiates. Opioid Analgesics for severe 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
    • Monitoring and treatment of superinfections 
      • Bacterial and fungal cultures of blood, wounds, and mucosal lesions
      • Appropriate antibiotics and antifungals, as necessary
    • Education on future drug avoidance, including closely related agents
  • Controversial treatments:
    • Cyclosporine
    • Systemic corticosteroids
    • Plasmapharesis
    • Intravenous immunoglobulin (IVIG)
Table: SCORTEN score
Prognostic factorsScore

Age ≥ 40 years

1

Malignancy present

1

Body surface area detached ≥ 10%

1

Tachycardia ≥ 120/min

1

Serum urea > 10 mmol/L

1

Serum glucose > 14 mmol/L

1

Serum bicarbonate < 20 mmol/L

1

The SCORTEN score is used to help determine the severity, prognosis, and appropriate setting for management in SJS/TEN patients.

  • Score 0‒1: 94% survival, may be treated in non-specialized wards
  • Score 2: 87% survival, should be transferred to an intensive care unit, burn unit, or specialized dermatology unit
  • Score 3: 53% survival, should be transferred to an intensive care unit, burn unit, or specialized dermatology unit
  • Score 4: 25% survival, should be transferred to an intensive care unit, burn unit, or specialized dermatology unit
  • Score 5‒7: 17% survival, should be transferred to an intensive care unit, burn unit, or specialized dermatology unit

Differential Diagnosis

  • Erythema multiforme Erythema multiforme Erythema multiforme (EM) is an acute hypersensitivity reaction characterized by targetoid skin lesions with multiple rings and dusky centers. Lesions may be accompanied by systemic symptoms (e.g., fever) and mucosal lesions (e.g., bullae). Erythema Multiforme: an acute, immune-mediated 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 eruption with typical targetoid lesions; may be accompanied by systemic symptoms and mucosal involvement. Etiology is usually due to infection from the herpes simplex virus Virus Viruses are infectious, obligate intracellular parasites composed of a nucleic acid core surrounded by a protein capsid. Viruses can be either naked (non-enveloped) or enveloped. The classification of viruses is complex and based on many factors, including type and structure of the nucleoid and capsid, the presence of an envelope, the replication cycle, and the host range. Virology: Overview, unlike SJS/TEN, which is usually caused by a medication. Diagnosis is clinical and will differentiate this condition from SJS. Treatment includes supportive care.
  • Staphylococcal scalded skin syndrome Staphylococcal Scalded Skin Syndrome Staphylococcal scalded skin syndrome (SSSS), also known as Ritter disease and staphylococcal epidermal necrolysis, is a toxin-mediated condition caused by Staphylococcus aureus. The exfoliative toxin produced disseminates and cleaves desmoglein 1 in the epidermis, causing separation and detachment of the skin. Staphylococcal Scalded Skin Syndrome (SSSS) ( SSSS SSSS Staphylococcal scalded skin syndrome (SSSS), also known as Ritter disease and staphylococcal epidermal necrolysis, is a toxin-mediated condition caused by Staphylococcus aureus. The exfoliative toxin produced disseminates and cleaves desmoglein 1 in the epidermis, causing separation and detachment of the skin. Staphylococcal Scalded Skin Syndrome (SSSS)): presents with a painful, desquamative 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 rash, especially around the 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, mouth, and anus. The condition results from a staphylococcal toxin, and is usually seen in children. Unlike SJS/TEN, there is no mucosal involvement. Diagnosis is clinical and confirmed with bacterial cultures. A biopsy will show noninflammatory, superficial splitting of the epidermis. Treatment includes antibiotics, wound care, and supportive care.
  • Exfoliative dermatitis: a generalized, symmetric, erythematous rash caused by an underlying cutaneous disease ( 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). The condition can mimic the early stages of SJS; however, there is no mucosal involvement (which will differentiate the condition from SJS). Diagnosis is made clinically, and treatment 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 or 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 Shock Shock is a life-threatening condition associated with impaired circulation that results in tissue hypoxia. The different types of shock are based on the underlying cause: distributive (↑ cardiac output (CO), ↓ systemic vascular resistance (SVR)), cardiogenic (↓ CO, ↑ SVR), hypovolemic (↓ CO, ↑ SVR), obstructive (↓ CO), and mixed. Types of Shock, and multisystem organ dysfunction. Cutaneous manifestations include a diffuse, erythematous rash and desquamation. Diagnosis is based on blood culture results, clinical history, and exam, which will differentiate this condition from SJS. Treatment includes hemodynamic support, fluid resuscitation, and antibiotics.
  • 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 causing intraepidermal blistering and erosions of the 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 and mucous membranes. Patients will have cutaneous bullae appearing on normal-appearing 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 and painful mucocutaneous erosions. Diagnosis involves a biopsy with immunofluorescence testing showing immunoglobulin G (IgG) 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 keratinocytes, differentiating this condition from SJS. Treatment 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 IVIG.
  • 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 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 disorder causing pruritus, erythematous plaques, and tense, bullous lesions. Mucous membrane involvement is rare. Triggers include medications, trauma, 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 conditions, and systemic disease. Biopsy with immunofluorescence shows IgG and complement deposits along the basement membrane, which differentiates the condition from SJS. Treatment 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 anti-inflammatory medications.

References

  1. High, W.A. (2019). Stevens-Johsnon syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis. In Corona, R. (Ed.), Uptodate. Retrieved November 7, 2020, from https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-pathogenesis-clinical-manifestations-and-diagnosis
  2. High, W.A. (2020). Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae. In Corona, R. (Ed.), Uptodate. Retrieved November 7, 2020, from https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-management-prognosis-and-long-term-sequelae
  3. Benedetti, J. (2020). Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). MSD Manual Professional Edition. Retrieved November 7, 2020, from https://www.msdmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-inflammatory-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten
  4. Foster, C.S, Ba-Abbad, R., Letko, E., and Parillo, S.J. (2019). Stevens-Johnson syndrome. In Dahl, A.A. (Ed.), Medscape. Retrieved November 7, 2020, from https://emedicine.medscape.com/article/1197450-overview

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