1. Treatment of severely ill dermatologic patients presented with :
Erythroderma
Steven Jonson syndrome
Toxic Epidermolysis Necrosis

2. 1- Erythroderma
Exfoliative dermatitis (ED) is a definitive term that refers to a scaling erythematous dermatitis involving 90% or more of the cutaneous surface. Exfoliative dermatitis is characterized by erythema and scaling involving the skin's surface and often obscures the primary lesions that are important clues to understanding the evolution of the disease.

4. Etiology
Frequently the result of the generalization of an underlying dermatosis : Psoriasis , Atopic dermatitis , chronic actinic dermatitis. Seborrheic dermatitis , Pityriasis rubra pilaris , Allergic contact dermatitis
Drug eruptions
Idiopathic
Malignancy:
Cutaneous T-cell lymphoma
Paraneoplastic erythroderma

5. Drug eruptions : The most commonly implicated drugs include:
• Anti-epileptics
• Allopurinol
• Antibiotics : Penicillin , Sulfonamides , Vancomycin.
•Calcium channel blockers
• Cimetidine
• Dapsone
• Gold
• Lithium
• Quinidine

6. How it is present : skin appears inflamed, edematous and scaly
Systemically unwell with lymphadenopathy and malaise

7. Pathophysiology :
An increased skin blood perfusion that results in temperature dysregulation and possible high-output cardiac failure. The basal metabolic rate rises to compensate for the resultant heat loss. Fluid loss by transpiration is increased in proportion to the basal metabolic rate. The situation is similar to that observed in patients following burns . Hypoalbuminemia results, in part, from decreased synthesis or increased metabolism of albumin. Edema is a frequent finding, probably resulting from fluid shift into the extracellular spaces.

8. 1) Attention to nutrition, fluid and electrolyte replacement.
Management :
Initial approach to the management of erythroderma of any etiology includes:
1) Attention to nutrition, fluid and electrolyte replacement. 
2) Institution of gentle local skin care measures. 
3) Wet dressings to weeping or crusted sites should be followed by application of topical corticosteroids

9. Stevens-Johnson syndrome

10. What is Stevens-Johnson syndrome :
Stevens-Johnson syndrome is a rare, serious disorder of your skin and mucous membranes.
It's usually a reaction to a medication or an infection.
Often, Stevens-Johnson syndrome begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters.
Then the top layer of the affected skin dies and sheds.
Stevens-Johnson syndrome is a medical emergency that usually requires hospitalization

12. The causes :
Various etiologic factors have been implicated as causes of Stevens-Johnson syndrome. Drugs most commonly are blamed. The 4 etiologic categories are as follows:
Infectious
Drug-induced
Malignancy-related
Idiopathic:
Stevens-Johnson syndrome is idiopathic in 25-50% of cases.
Drugs and malignancies are most often implicated as the etiology in adults and elderly persons. Pediatric cases are related more often to infections.

13. Infectious causes:
Viral diseases that have been reported to cause Stevens-Johnson syndrome include the following:
Herpes simplex virus (possibly; remains a debated issue)
AIDS
Coxsackie viral infections
Influenza
Hepatitis
Mumps
In children, Epstein-Barr virus and enteroviruses have been identified as being associated with the development of Stevens-Johnson syndrome.
More than half of the patients with Stevens-Johnson syndrome report a recent upper respiratory tract infection.

15. Bacterial infections:
Group A beta-hemolytic streptococci
Diphtheria
Brucellosis
Lymphogranuloma venereum
Mycobacteria
Mycoplasma pneumoniae 
Rickettsial infections
Tularemia
Typhoid
Possible fungal causes include coccidioidomycosis, dermatophytosis, and histoplasmosis. Malaria and trichomoniasis have been reported as protozoal causes.

16. Drug induce : Analgesics, cough and cold medication NSAIDs
Antibiotics are the most common cause of Stevens-Johnson syndrome
Analgesics, cough and cold medication
NSAIDs
Psycho-epileptics
Antigout drugs

17. Genetic factors:
There is strong evidence for a genetic predisposition to severe cutaneous adverse drug reactions such as Stevens-Johnson syndrome. Carriage of the following human leukocyte antigens has been associated with increased risk

18. Management : Early diagnosis and withdrawal of suspected drug(s).
Patients are best cared for in an intermediate or intensive care unit. 

Manage replacement of IV fluids and electrolytes as for patient with extensive thermal burn.
Systemic glucocorticoids early in the disease and in high doses are reported helpful in reducing morbidity or mortality (as is also the experience of the authors), but this has been questioned. Late in the disease, they are contraindicated.
High-dose IV immunoglobulins halt progression of TEN if administered early. 

Diagnose and treat complicating infections, including sepsis (fever, hypotension, change in mental status).
Treat eye lesions early with erythromycin ointment.

19. Toxic epidermolysis necrosis

20. What is toxic epidermal necrolysis (TEN)
Toxic epidermal necrolysis (TEN) is a potentially life-threatening dermatologic disorder characterized by widespread erythema, necrosis, and bullous detachment of the epidermis and mucous membranes, resulting in exfoliation and possible sepsis and/or death (see the image below). Mucous membrane involvement can result in gastrointestinal hemorrhage, respiratory failure, ocular abnormalities, and genitourinary complications.

22. The pathophysiology :
The pathophysiology of TEN has not been fully elucidated; however, various theories have received wide acceptance.
TEN is believed to be an immune-related cytotoxic reaction aimed at destroying keratinocytes that express a foreign antigen.
TEN mimics a hypersensitivity reaction, with its characteristic delayed reaction to an initial exposure and an increasingly rapid reaction with repeated exposure
The widespread epidermolysis and blistering of TEN results from keratinocyte apoptosis—an organized series of biochemical reactions leading to cell changes and cell death.

23. Etiologies : Medications : Infections (e.g. Mycoplasma pneumonia)
Sulfonamide antibiotics.
Allopurinol.
Amine antiepileptics.
Phenytoin.
Carbamazepine.
Lamotrigine
NSAIDs (72)
Infections (e.g. Mycoplasma pneumonia)
Other: Vaccinations, Systemic diseases, Chemical exposure, Herbal medicines, Foods

24. TEN= > 30% of skin involvement
10-30%= SJS/TEN overlap
< 10% = Stevens-Johnson
20-30% mortality rate
Incidence of 1-2 per million

25. SCORTEN Severity of illness score Probability of hospital mortality
Similar past formula was age + TBSA

26. Variables Odds Ratio p-value Age (40  y old) 2.7 (1.0-7.5) 0.05
Heart Rate ( 120 bpm)
2.7 ( )
0.04
Cancer/hematologic malig.
4.4 ( )
BSA involved at day 1
< 10% 1
10-30%
2.9 ( )
> 30%
3.3 ( )
Serum urea level ( >10mmol)
2.5 ( )
0.09
Serum bicarb (<20 mmol/L)
4.3 ( )
0.03
Serum glucose (>14mmol/L)
5.3 ( )
<0.01
SCORTEN
2.45 ( )
<10-4

27. Mortality Rates and Relative Risks
SCORTEN
# patients
% mortality
95% CI
Odds Ratio
0-1 31
3.2
( ) 1 2 66
12.1
( )
4.1 3 34
35.3
( )
14.6 4 24
58.3
( )
42.0 5 10
90.0
( )
270.0

28. Management :
Prompt recognition of the disorder and withdrawal of all potential causative agents.
Supportive care until the epithelium regenerates.
Supportive measures include isolation, fluid and electrolyte balance, nutritional support, pain management, and protective dressings.
Early transfer of patients to a burn or intensive care unit has been shown to reduce the risk of infection, mortality rate, and length of hospitalization.
No specific treatment modality has been proven effective, but agents such as crystalloids, antibiotics, antihistamines, anticoagulants, analgesics, and antiseptic agents are important for supportive care. Use of corticosteroids is controversial.

29. Sources:
-Wiley online library
-ScienceDirect
Dermatology hand book for medical students & junior doctors

30. Done by :
Jude wael
Fatimah barghawi
Lujaina reda
Afnan alotaibi
Fatimah almuhana
Duaa aleeli
Aliaa alyousef
Zahraa aljafil
Hajar bangitah
Amani alawaji