Transcript ERYTHEMA MULTIFORME - Dr. Raj Kumar Sharma

ERYTHEMA MULTIFORME
&
STEVENS-JOHNSON SYNDROME
(S.J.S)TOXIC EPIDERMAL NECROLYSIS
(TEN)
DR MAHESH MATHUR
MD,DVD,DCP (UK)
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
TYPES
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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
Erythema Multiforme
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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
Etiology
-HSV infection
-Contact sensitization
Sulphonamide,
Anti histaminic,
DNCB
Rose wood,
Primula,
tea tree oil,
Cinnamon
-UV Light
-Pregnancy
Pathogenesis
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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
Clinical Manifestations
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Mild Prodromal symptomsCough,
Low-grade Fever
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Sudden appearance of Typical target skin
lesionsRegular circular wheal-like Erythematous papules/plaques
fixed and of variable size,
Center of lesions become violaceous dark or vesicular bullus,
Heals without scarring
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Mucosal Lesions 70% of casesLips Palate & gingiave in most of the cases
ERYTHEMA MULTIFORME
TARGET LESION
HISTOATHOLOGY
SKIN SECTION H&E STAINING
SKIN SECTION
H&E STAINING X100
TREATMENT
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Symptomatic
Systemic Corticosteroid not Needed
Recurrent EM
Oral Acyclovir 200 mg 5 Times a Day for the
treatment
Low Dose Acyclovir400-800 mg oral per day for 6 Month may be
indicated
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
Etiology
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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
DRUGS
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Sulphasalazine
Co-Trimoxazole
Hydantoins
Carbamazepine
Barbiturates
Phenylbutazone
Ibuprofen
Piroxicam
Allopurinol
Aminopenicillens
Fluroquinolones
Clinical Manifestations
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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
DUSKY ERYTHEMATOUS MACULES
SCALDING OF SKIN
SCALDING OF SKIN
SJ SYNDROME
Ocular Manifestations
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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.
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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
Conjunctival membranes &
pseudomembrane
Cicatricial changes
conjunctivilization
MUCUS MEMBRAN LESION
CHILD WITH SJS-TEN SYNDROM
HISTOPATHOLOGY
H&E SECTION X45
IMMUNO STAINING OF CORNEAL
BIOPSIES
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
Prognosis
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INDICATOR OF POOR PROGNOSIS
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Age >40 years
Malignancy
Tachycardia
BSA >10%
Serum Urea level >10mmol/L
Serum Glucose level 14mmol/L
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FATAL OUTCOME DUE TO –
Septicemia
GI bleeding
Renal insufficiency &
shock