Steven Johnson Syndrome (SJS), Toxic Epidermal Necrolysis (TEN), Toxic Shock Syndrome (TSS), and Staphylococcal Scalded Skin Syndrome (SSSS) are severe cutaneous reactions characterized by skin detachment and multi-organ involvement. SJS/TEN are differentiated based on skin detachment percentage, with SJS involving <10%, TEN >30%, and overlap between 10-30%. Drugs are a common cause. TSS is caused by bacterial toxins leading to shock. SSSS results from Staphylococcal toxins causing blistering resembling burns. Treatment involves stopping the offending agent, supportive care, and antibiotics targeting the underlying infection.

1. Difference between Steven Johnson
Syndrome , Toxic Epidermal
Necrolysis , Toxic Shock Syndrome
and Staphylococcus Scalded Skin
Syndrome

2. Steven Johnson Syndrome ( SJS ) &
Toxic Epidermal Necrolysis ( TEN)
• Definition : severe mucocutaneous reactions
characterized by extensive necrosis and
detachment of epidermis .
• In US , the estimated incidences of SJS, SJS/TEN and
TEN among children between 2009 – 2012 were
5.3 , 0.8 and 0.4 cases per million children.
• Ration male to female is 1: 2

3. • SJS and TEN are differed based on total body
surface area affected.
• SJS involves < 10 % of total body surface area
• TEN involves if > 30% of total body surface area
• SJS/TEN overlap involves between 10 – 30 % total
body surface area

4. Wallace Rules of 9

5. Etiologies
1. Drugs – limited up to 8 weeks of exposure
2. Infection e.g mycoplasma pneumonia , CMV ,
URTI , Herpes
3. Others
- Vaccinations
- Herbs
- Foods
- Radiotherapy
- Chemical exposure

6. Drugs
• Anticonvulsant ( carbamazepine , phenytoin
,phenobarbitone , phenytoin, lamotrigine )
• Antibiotics – cephalosporin , quinolones , aminopenicillin ,
trimethoprim /sulfamethoxazole
• NSAIDs
• Analgesic – Paracetamol
• Allopurinol
• Anti malarial
• Tranxenamic acid
• Anti cancer drugs – busulfan
• Corticosteroids
• Retinoids

7. Risk Factors
• HIV
• Malignancy
• Genetic factors – HLA ( human leukocytes antigen )
• High doses and rapid introduction of medications
• SLE

8. Pathogenesis
• There are probably two major pathways involved:
• Fas-Fas ligand pathway of apoptosis has been
considered a pivotal step in the pathogenesis of TEN.
The Fas ligand (FasL), a form of tumour necrosis factor,
is secreted by blood lymphocytes and can bind to the
Fas ‘death’ receptor expressed by keratinocytes.
• Granule-mediated exocytosis via perforin and granzyme
B resulting in cytotoxicity (cell death). Perforin and
granzyme B can be detected in early blister fluid and it
has been suggested that levels may be associated with
disease severity.

9. Clinical Presentation
• Early signs/symptoms:
• Fever > 39 C
• Sore throat, difficulty swallowing
• Runny nose and cough
• Sore red eyes, conjunctivitis
• General aches and pains.

10. Later signs/symptoms:
• Marked erythema of skin leading to papules,
vesicles and necrosis started on the face neck and
anterior trunk and may extend over the entire
surface of the skin. May presented as
blisters/macules/ flat atypical lesions , diffuse
erythema
• Mucosal involvement, including ocular, GI, GU,
genital and upper and the epithelial cells of the lower
respiratory tree.

11. • Nicolsky’s sign : epidermal layer easily sloughs off
when pressure applied to affected area

13. • Eyes (conjunctivitis, less often corneal
ulceration, anterior uveitis) — red, sore,
sticky, photosensitive eyes
• Lips/mouth (cheilitis, stomatitis) — red crusted lips,
painful mouth ulcers
• Pharynx, oesophagus — causing difficulty eating
• Genital area and urinary tract — erosions, ulcers,
urethritis , urinary retention
• Upper respiratory tract (trachea and bronchi) — cough
and respiratory distress
• Gastrointestinal tract — diarrhoea.

14. Patient Evaluation and Diagnosis
• There are no universally accepted diagnostic
criteria for SJS/TEN
• Diagnosis would be appropriate with the history
and clinical findings.

15. Differential Diagnosis
• Staphylococcal scalded skin syndrome and toxic
shock syndrome
• Erythema multiforme,
particularly erythema multiforme major
(with mucosal involvement)
• Mycoplasma infections
• Bullous systemic lupus erythematosus

16. Investigations :
• 1. skin biopsy
Result shows keratinocyte apoptosis with detachment of
the epidermal layer of the skin from the dermal layer
2. Blood CnS – negative
3. FBC
4. RP
5. LFT
6. Blood gases

17. Course of Illness
• The acute phase of SJS/TEN lasts 8–12 days.
• Repithelialisation of denuded areas takes several
weeks, and is accompanied by peeling of the less
severely affected skin

18. Treatment algorithm

19. • Occupational and Physiotherapy

20. Complications
• Dehydration and acute malnutrition
• Infection of skin, mucous membranes, lungs
(pneumonia), septicaemia (blood poisoning)
• Acute respiratory distress syndrome
• Gastrointestinal ulceration, perforation and
intussusception
• Shock and multiple organ failure including kidney
failure
• Thromboembolism and disseminated intravascular
coagulopathy.

21. Long-term sequelae include:
• Pigment change — patchwork of increased and
decreased pigmentation
• Skin scarring, especially at sites of pressure or infection
• Loss of nails with permanent scarring (pterygium) and
failure to regrow
• Scarred genitalia — phimosis (constricted foreskin
which cannot retract) and vaginal adhesions
(occluded vagina)
• Joint contractures
• Lung disease — bronchiolitis, bronchiectasis,
obstructive disorders.

23. Prognosis
• SCORTEN is an illness severity score that has been
developed to predict mortality in SJS and TEN cases.
• One point is scored for each of seven criteria present at
the time of admission. The SCORTEN criteria are:
Age > 40 years
Presence of a malignancy (cancer)
Heart rate > 120
Initial percentage of epidermal detachment > 10%
Serum urea level > 10 mmol/L
Serum glucose level > 14 mmol/L
Serum bicarbonate level < 20 mmol/L.

24. • The risk of dying from SJS/TEN depends on the
score.
• SCORTEN 0-1 > 3.2%
• SCORTEN 2 > 12.1%
• SCORTEN 3 > 35.3%
• SCORTEN 4 > 58.3%
• SCORTEN 5 or more > 90%

25. Prognosis is best when:
• Patients are <50 years of age
• The total body surface area (TBSA) involved is low
• Patients are transferred to a burn centre
• Patients do not have sepsis
• Patients do not require antibiotics.

26. Toxic Shock Syndrome

27. Definition :
• Streptococcal TSS is defined as an invasive infection
secondary to group A streptococcus ( Streptococcus
pyogenes ) associated with shock and multi-organ
system failure occurring early in the course of the
disease
• Organisms commonly responsible include group A
streptococcus ( Streptococcus pyogenes ), or
methicillin-sensitive (MSSA) or methicillin-resistant
(MRSA) Staphylococcus aureus .

28. Can be divided to :
• Menstrual cause
• Non menstrual

29. Pathophysiology
• Toxic shock syndrome starts from a localised staphylococcal infection which
produces the causative exotoxins.
• When tampons are used, bacteria can gain entery into the uterus via the cervix.
They can also cause cuts in the vagina and permit access of bacteria into the
tissues.
• It may occur as a complication of other localised or systemic infections such as
pneumonia, osteomyelitis, sinusitis, and skin wounds (surgical, traumatic or
burns).
• Streptococcal TSS is mediated by streptococcal pyrogenic exotoxins
(superantigens) and virulence factors (M strains with M proteins 1 and 3) that
activate the immune system to release inflammatory cytokines.
• The cytokines (TNF-alpha, interleukin [IL]-1, and IL-6) result in shock and multi-
organ failure

30. Clinical presentation
• CDC case definition for toxic shock syndrome requires presence of the
following 5 clinical criteria:
1. Temperature = > 38.9 c
2. Low blood pressure (including fainting or dizziness on standing) :
systolic BP ≤90 mmHg for adults or less than fifth percentile by age
for children aged less than 16 yea
3. Widespread red flat rash
4. Shedding of skin, especially on palms and soles, 1–2 weeks after
onset of illness
5. Abnormalities in 3 or more of the following organ systems:
a) Gastrointestinal: vomiting or diarrhoea
b) Muscular: severe muscle pain
c) Hepatic: decreased liver function
d) Renal: raised urea or creatinine levels
e) Hematologic: bruising due to low blood plateletcount

31. • Toxic shock syndrome diagnosis is confirmed if all 5
CDC clinical criteria are fulfilled.
• A probable case fulfils 4 of the 5 criteria.

34. • Microscopy and culture on normally sterile sites
(blood, CSF, pleural or peritoneal fluid, tissue, or
throat) may be positive for group A streptococcus
or Staphylococcus aureus . However, of patients
with streptococcal TSS, 60% have positive blood
cultures, and of patients with staphylococcal TSS,

35. Investigations
• FBC and differential show leukocytosis, anaemia, and thrombocytopenia
• Renal function: elevated urea, creatinine, and haemoglobinuria are signs of renal failure.
• Liver function: elevation of bilirubin or transaminases more than twice the normal upper limit
• Albumin and calcium: commonly low on admission with streptococcal disease and throughout the
clinical course
• Lactic acid: elevated in severe sepsis and septic shock
• Elevated creatine kinase (CK) suggests necrotising fasciitis or myositis. CK may also be elevated in
staphylococcal TSS
• Coagulation profile: shows increased prothrombin and partial thromboplastin times in
staphylococcal disease in conjunction with DIC

36. Management
• The treatment starts with:
• Removing the source of infection ie tampons,
vaginal sponges, or nasal packing
• Draining and cleaning the site of wound.
• Start antibiotic

37. • Supportive care-
• Intravenous fluids to treat shock and prevent organ
damage
• Vasopressor for patients with very low blood
pressure
• Dialysis in patients who develop renal failure
• Administration of blood products
• Oxygen and mechanical ventilation to assist with
breathing

38. In suspected case Start Empirical
Antibiotic Therapy
• IV clindamycin: 25 - 40 mg/kg per day in 3 divided
dose
+ IV vancomycin: 40 mg/kg/ day in 4 divided doses

39. Confirmed case
1. Methicilin susceptible S. Aureus ( MSSA ) :
• IV clindamycin: 25 - 40 mg/kg per day in 3 divided dose
+ Oxacillin / nafcillin ( 100 -1 50 mg/kg per day in four
divided doses )
• 2. MRSA :
• IV clindamycin: 25 - 40 mg/kg per day in 3 divided dose
+ IV vancomycin: 40 mg/kg/ day in 4 divided doses
Duration 1 -2 weeks

40. Prognosis
• Early diagnosis and appropriate treatment prevents
progression of the disease and possible
complications such as heart
problems, acute renal failure, adult respiratory
distress syndrome and disseminated intravascular c
oagulation.
• The mortality rate of toxic shock syndrome is
approximately 5–15%, and recurrences have been
reported in as many as 30–40% of cases

41. Staphylococcal scalded
skin syndrome ( SSSS)

42. • An illness characterised by red blistering skin that
looks like a burn or scald.
• It is caused by the release of two exotoxins
(epidermolytic toxins A and B) from toxigenic
strains of the bacteria Staphylococcus aureus.
• A.k.a Ritter disease or Lyell disease when it appears
in newborns or young infants.

43. Risk factors
• Children less than 5 years old
• Immunocompromised patients
• Pt with renal failure

44. Clinical Presentation
• It usually starts with fever, irritability and
widespread redness of the skin.
• Within 24-48 hours fluid-filled blisters form. These
rupture easily, leaving an area that looks like a
burn.

47. Rash characters :
• Tissue paper-like wrinkling of the skin is followed by the
appearance of large fluid-filled blisters (bullae) in the
armpits, groin and body orifices such as the nose and
ears.
• Rash spreads to other parts of the body including the
arms, legs and trunk. In newborns, lesions are often
found in the diaper area or around the umbilical cord.
• Top layer of skin begins peeling off in sheets, leaving
exposed a moist, red and tender area.
• Nikolsky sign is positive (ie gentle strokes result
in exfoliation)

48. Diagnosis
• History and physical examination
• Skin biopsy- which shows intraepidermal cleavage
at the granular layer
• CnS : from skin, blood, urine or umblical cord
sample (in a newborn baby)

49. DDx

50. Treatment
• A penicillinase-resistant, anti-staphylococcal antibiotic
such as flucloxacillin is used.
• Other antibiotics include nafcillin, oxacillin,
cephalosporin and clindamycin.
• Vancomycin is used in infections suspected
with methicillin resistance (MRSA).
• Depending on response to treatment, oral antibiotics
can be substituted within several days. The patient may
be discharged from hospital to continue treatment at
home.
• Corticosteroids slow down healing and hence are not
given to patients with SSSS.

51. • Other supportive treatments for SSSS include:
• Paracetamol when necessary for fever and pain.
• Monitoring and maintaining fluid and electrolyte
intake.
• Skin care (the skin is often very fragile). Petroleum jelly
should be applied to keep the skin moisturised.
• Newborn babies affected by SSSS are usually kept in
incubators.

52. Referrence
• BMJ Best Practice.com
• Uptodate.com
• https://www.dermnetnz.org