SJS/TEN

Pemphigus Vulgaris

Bullous Pemhigoid

Erythroderma

Etiology:
TEN: mostly due to drug
SJS: due to drugs or infections
(commonly mycoplasma)

Serious bullous autoimmune disease.

Involves skin and mucous membranes.
Can start in mouth then skin or generalized
acute eruption of both
Painful mouth erosions

Age: 60 to 80 yrs

Redness and scaling of almost

all of the entire surface of the
skin

Drugs:
Sulfanomide
Allopurinol
Antiepileptics
NSAIDs

Skin Lesions : Flaccid bullae easily ruptured

>erosions, bleeding & crustation
- Nikolyskys sign : Positive

Often starts as urticarial lesions

bullae or directly as bullous
eruption
Skin findings:Large tense intact
bullae

Causes:
Drug eruption
eczema
psoriasis

Involvement:
Skin
Mucous membrane (less than
pemphigus)
Nikolsky sign: Negative

SJS < 10% epidermal detachment

SJS / TEN overlap 10 30 %
TEN > 30% epidermal detachment
TEN: + Nikolsky sign
SJS:
-More mucosal involvement but less
skin involvement than TEN
-Better prognosis than TEN .
Skin Pain , burning & tenderness.
Mucous membrane : mouth lesions
are painful and tender.

VARIANT:
-Pemphigus Vegetan
-Pemphigus Foliaceus
-Pemphigus erythematosus
-Drug induced pemphigus (captopril)
-Paraneoplastic pemhigus

Complications of Erythroderma
-Secondary Infections
-Loss of Thermoregulation
-Renal Failure
-Hypoalbuminemia

Complications;

Skin Biopsy:

Skin Biopsy:

-Fluid and electrolyte imbalance

Intraepidermal blister with acantholysis

(separation of keratinocyte from each
other)

Subepidermal blisters with

eosinophils

-Infection (due to loss of skin barrier)

-Skin: Scarring and dyspigmentation
-Eye: Adhesions blindness
-Acute renal failure

Immunoflourscence :
(Patient skin ) Direct: IgG & C3 positive at
intercellular space in epidermis

Immunofluroscence:
-Direct : Linear deposition of IgG &
C3(along the basement
membrane)

(Patient serum) Indirect: autoantibody IgG

+Ve
against desmoglein III

-Indirect : autoantibodies against

BPAg1 & BPAg2

Management:

Management:

Management :

MANAGEMENT

- Stop the causative agent

-Admit to burn unit or ICU
-IVF and electrolyte replacement like
-burn patient
- IV Abx for infection
-Skin dressing daily
-Eye care by opthalmologist daily
- High calori intake
- Folys catheter/ NG tube

-Correction Of Fluid & electrolyte loss

-superpotent topical steroids (for

mild cases)

Wet dressings

Diagnosis confirmation : skin biopsy

-treatment of infection by antibiotics

-systemic steriods (high dose)
-Immunosuprresive therapy

- Systemic steroids

Manage fluid balance and

temperature

(-+) Immunosuppressive agents

Antibiotics
Antihistamines
Aggressively Treat Underlying
Condition