1. STEVENS-JOHNSON SYNDROME (S.J.S)- TOXIC EPIDERMAL NECROLYSIS (TEN)
ERYTHEMA MULTIFORME
&
STEVENS-JOHNSON SYNDROME (S.J.S)- TOXIC EPIDERMAL NECROLYSIS (TEN)
DR MAHESH MATHUR MD,DVD,DCP (UK)

2. ERYTHEMA MULTIFORME DEFINITION Group of Diseases encompasses
A number of acute self-limited exanthematic intolerance reaction that share at least two characteristic
1.skin lesion – Target lesions
stable Circular erythemas or urticarial plaques with areas of blistering, necrosis and /or resolution in a concentric array
2.Histologically –satellite cells and widespread necrosis of epidermis

3. TYPES Erytema multiforme
Common, mild relapsing eruption that is triggered by recurrent herpes Simplex infection
Stevens-Johnson syndrome -Toxic Epidermal Necrolysis (SJS-TEN)
An infrequent sever mucocutaneous intolerance most commonly triggered by drugs

4. Erythema Multiforme 1% of Dermatological out patient
Infrequent age <3 years & > 50 years
75% of patients are under 40 years
Equal incidence in M:F
No predominance for any ethnic groups or geographical location
Seasonal clustering of cases in Spring
Recurrent
U.V. light provocation of recurrent HSV infection

5. Etiology -HSV infection -Contact sensitization Sulphonamide,
Anti histaminic,
DNCB
Rose wood,
Primula,
tea tree oil,
Cinnamon
-UV Light
-Pregnancy

6. Pathogenesis
Cell Mediated immune reaction leading to destruction of Keratinocytes expressing HSV antigens
CD 8 + cells carry out the immunological attack on epidermal cells expressing viral DNA- leading to apoptosis of individual cells & necrosis
CD 4+ T lymphocytes & monocytes cellular infiltration in Dermis causing wheal like erythema and microvasculature damage leads to epidermal necrosis and vesiculo-Bullous formation

7. Clinical Manifestations
Mild Prodromal symptoms-
Cough,
Low-grade Fever
Sudden appearance of Typical target skin lesions-
Regular circular wheal-like Erythematous papules/plaques fixed and of variable size,
Center of lesions become violaceous dark or vesicular bullus, Heals without scarring
Mucosal Lesions 70% of cases-
Lips Palate & gingiave in most of the cases

8. ERYTHEMA MULTIFORME

9. TARGET LESION

10. HISTOATHOLOGY SKIN SECTION H&E STAINING

11. SKIN SECTION H&E STAINING X100

12. TREATMENT Symptomatic Systemic Corticosteroid not Needed Recurrent EM
Oral Acyclovir 200 mg 5 Times a Day for the treatment
Low Dose Acyclovir-
mg oral per day for 6 Month may be indicated

13. STEVENS-JOHNSON SYNDROME (S.J.S) & TOXIC EPIDERMAL NECROLYSIS (TEN)
DEFINITION
Sever, episodic, acute mucocutaneous reaction most commonly elicited by drugs
Characterized by rapidly spreading irreregular dusky erythematic maculs, necrosis of skin and detachment of skin resembling scalding of skin with involvement of more then one mucosal site
Constitutional symptoms and internal organ involvement often occurs and may be sever
Self-limited
Significant morbidity scaring & mortality

14. Etiology 2 to 3 cases per million Occurs World wide M:F ratio is 2;1
Drugs *
3 fold increase in HIV infected Population
Infections-Mycoplasma, Pneumoniae,Infectious mononucleosis, Histoplasmosis, Gram Negative septicemias
Pathogenesis
CD4+ & CD8+ cells & cytokines mediated injury to epidermal cells

15. DRUGS Sulphasalazine Co-Trimoxazole Hydantoins Carbamazepine
Barbiturates
Phenylbutazone
Ibuprofen
Piroxicam
Allopurinol
Aminopenicillens
Fluroquinolones

16. Clinical Manifestations
Non Specific sever prodormal Symptoms
Macular morbiliform rash appears on face trunk.
Positive Nikolsky’s sign
Predominantly mucous membranes involvement- in 40% of cases
Buccal mucosa, Hemorrhagic crusts at lips, bulbar conjunctiva
Respiratory tract & G.I.T involvement
Homodynamic instability & Shock

17. DUSKY ERYTHEMATOUS MACULES

18. SCALDING OF SKIN

19. SCALDING OF SKIN

20. SJ SYNDROME

21. Ocular Manifestations
Mild-lid edema,
Conjunctivitis, chemosis
Moderate - conjunctival membranes,
Corneal epithelial loss and corneal ulceration;
Severe - perforation,
Cicatricial changes.
9% of patients with EM-minor had ocular manifestations. ~ the SJS (69%)
27% of patients experiencing severe complications including, corneal melts and cicatricial changes.
up to 50% of patients with severe cutaneous disease develop severe ocular manifestations.   

22. The acute phase of disease is characterized primarily by eyelid involvement.
The lids are typically swollen and erythematous.
Soon thereafter, or coincident with this, the conjunctiva is inflamed.
The conjunctivitis frequently parallels the skin findings, with bullae formation and eruption.
Membrane or pseudomembrane formation then occurs
The healing process can result in cicatricial changes which include
symblepharon, ankyloblepharon, eyelid margin rotation, severe dry eye and conjunctivilization of the corneal Recurrent ocular inflammation can occur in patients with resolved acute ocular and systemic disease in the absence of systemic recurrence

23. Conjunctival membranes & pseudomembrane

24. Cicatricial changes

25. conjunctivilization

26. MUCUS MEMBRAN LESION

27. CHILD WITH SJS-TEN SYNDROM

28. HISTOPATHOLOGY H&E SECTION X45

29. IMMUNO STAINING OF CORNEAL BIOPSIES

30. Treatment -Identification of causative agent & it’s elimination
-Active therapy Glucocorticoids
- Immunoglobulin
- Immunomodulator
Cyclophosphamide
Cyclosporine
Thalidomide
- Plasmapheresis
-Supportive therapy-
-Maintenance of Homodynamic status
-Protein & electrolyte Homeostasis
-Antimicrobial therapy

31. Prognosis INDICATOR OF POOR PROGNOSIS Age >40 years Malignancy
Tachycardia
BSA >10%
Serum Urea level >10mmol/L
Serum Glucose level 14mmol/L
FATAL OUTCOME DUE TO –
Septicemia
GI bleeding
Renal insufficiency & shock