Vous êtes sur la page 1sur 63

Life-threatening Cases in

Maya Wahdini, dr., Mkes, SpKK
Epidermal Necrolysis
• Acute life-threatening mucocutaneous
reaction characterized by extensive necrosis
and detachment of the epidermis
• Based on skin detachment percentage:
Stevens-Johnson Syndrome (SJS) <10%
Toxic epidermal necrolysis (TEN) >30%
Overlap SJS-TEN 10-30%
• Most important etiologic factor  drugs (first
8 weeks of treatment)
• Others:
• infectious agents (Mycoplasma pneumoniae,
viral, immunization)
• Idiopathic
Clinical Findings
• History:
• Begins 8 weeks (usually 4-30 days) after the
onset of drug exposure
• Nonspecific symptoms (fever, headache,
rhinitis, cough, malaise) may preceed the
mucocutaneous lesions by 103 days
• Pain on swallowing and burning or stinging of
the eyes
• Mucocutaneous lesions:
• Simetrically distributed on the face, upper
trunk, and proximal part of limbs
• Distal portion of the arms and legs are
relatively spared, but rash can rapidly extend
to the rest of the body within few days and
even hours
• Initial lesion erythematous, dusky red,
purpuric macules, irregularly shaped which
progresively coalesce
• Confluence or necrotic lesions leads to
extensive and diffuse erythema
• Nicholsky’s sign positive
• Lesion evolve to flaccid blisters, which spread
with pressure and break easily
• The necrotic epidermis is easily detached at
pressure points or by frictional trauma,
revealing large areas of exposed, red,
sometimes oozing dermis
• Mucous membrane involvement:
• Nearly always on at least two sites
• Observed in nearly 90% of cases and can
precede or follow the skin eruption
• Erythema  painful erosion of the oral,
ocular, and genital mucosa
• Eye: photophobia, conjungtivitis, painful
micturition, redness, discharge  corneal
ulcer, uveitis
Laboratory Test
• No specific laboratory test
• Massive transdermal water loss  electrolyte
imbalance, hypoalbuminemia, hypoproteinemia,
mild and transient renal insufficiency
• Mild leukocytosis
• Histopathology: apoptotic keratinocytes in
suprabasal layer, rapidly evolves to a full-
thickness necrosis and subepidermal detachment
Differential Diagnosis
• Significant fluid loss from erosion 
hypovolemia, electrolyte imbalance
• Sepsis  multiple organ failure
• Eye: fibrosis, trichiasis, symblepharon
• Hypo/hyperpigmentation
• Nail changes
• Mortality rate: SJS 5-12%, TEN >30%
• Symptomatic:
• Intensive care/burn unit
• Maintenance of hemodynamic equilibrium
• Fluid replacement
• Prophylactic antibiotic  not indicated
• Specific treatment: corticosteroids,
intravenous immunoglobulin, cyclosporine A,
• skin lesion consisting of a wheal-and-
flare reaction in which localized
intracutaneous edema (wheal)
surrounded by an area of redness
(erythema) that is typically pruritic.
• Last to 30 minutes to as long as 36
• Size 1 mm until 6–8 inches in
diameter (giant urticaria).
• Angioedema can be caused by the
same pathogenic mechanisms as
urticaria but the pathology is in the
deep dermis and subcutaneous tissue
and swelling is the major
• The overlying skin may be
erythematous or normal.
• There is less pruritus, but there may
be pain or burning.
• Acute: last less than 6 weeks
• Mostly due to adverse reactions to
medications or foods and in children,
to viral illnesses.
• Chronic: episodes persisting beyond 6
(1) chronic autoimmune urticaria
(2) chronic idiopathic urticaria (55%)
• Ideal tratment: identification and removal of its
• Acute urticaria:
• Antihistamine
• Corticosteroid (40-60 mg/day for 3days and taper
by 5-10 mg/day)
• Epinephrine can relieve severe symptoms of
urticaria and angioedema (generalized urticaria,
severe pruritus, accelerating angioedema) and is
indicated if laryngeal edema is present
Erythroderma/exfoliative dermatitis
• Diffuse erythema and scaling of the skin
involving more than 90% of the total body skin
surface area.
• Common underlying etiologies are psoriasis,
atopic dermatitis, and other spongiotic
dermatoses, drug hypersensitivity reactions,
and cutaneous T-cell lymphoma (CTCL). The
cause of ED is unknown (idiopathic) in
approximately 20% of cases
Differential diagnosis
• Most Likely
• Spongiotic dermatitis (20%–24%) (atopic, 9%;
contact dermatitis, 6%; seborrheic dermatitis,
4%; chronic actinic dermatitis, 3%)
• Psoriasis (23%)
• Drug hypersensitivity reaction (15%)
• Cutaneous T-cell lymphoma (5%)
• Idiopathic (approximately 20%)
• Consider
• Contact dermatitis
• Immunobullous disease (superficial pemphigus, bullous
pemphigoid, paraneoplastic pemphigus)
• Infection (scabies, dermatophytosis)
• Toxin-mediated (toxic shock syndrome, staphylococcal
scalded-skin syndrome)
• Chronic actinic dermatitis
• Pityriasis rubra pilaris
• Collagen vascular disease
• Paraneoplastic (solid tumors and hematologic)
• Primary immunodeficiency
• Congenital ichthyoses
• Always Rule Out
• Cutaneous T-cell lymphoma
• Drug-induced hypersensitivity syndrome
• Paraneoplastic
Clinical Findings
• History: for diagnosing the underlying etiology
• Cutaneus Lesion:
• Erythematous patches increase in size and
coalesce into generalized red erythema with a
shiny appearance, involves more than 90% of
the patient’s skin surface
• A few days after the onset of erythema, fine
white or yellow scaling begins, classically
arising in the flexures.
• Plate-like scaling on the palms and soles.
• The scaling
progresses further,
giving the skin a
dull red
• With chronicity,
edema and
lichenification lead
to skin induration.
• Ectropion and
epiphora may
secondary to
• Palmoplantar
keratoderma (80%
of patients with
chronic ED)
• Some patients: hair and nails.
• Hair: scaling of the scalp, alopecia, diffuse
• Nail changes: onycholysis, subungual
hyperkeratosis, splinter hemorrhages,
paronychia, Beau’s lines.
• Related physical findings due to ED of any
• Tachycardia (due to increased blood flow to the
skin and fluid loss due to disrupted epidermal
• Thermoregulatory disturbances can result in
hyperthermia and feeling chilly.
• Generalized lymphadenopathy (in >1/3 patients)
• Hepatomegaly (in 1/3 patients and is more
commonly seen in drug-induced ED)
• Peripheral pedal or pretibial edema (occur in up
to 54% of patients)
Laboratory Test
• Most often non diagnostic and non specific
• Anemia, leukocytosis, lymphocytosis,
eosinophilia, hypoalbumin, electrolyte
abnormalities, abnormal renal function
• Histopathology to determine the underlying
etiology. But should be done multiple and
repeated over time.
• fluid and electrolyte imbalances
• Thermoregulatory disturbance
• high-output cardiac failure
• cardiogenic shock
• acute respiratory distress syndrome
• Hypoalbuminemia caused by increase of protein
loss via scaling (by 10%–15% in nonpsoriatic ED
and up to 25%–30% in psoriatic muscle wasting
and edema
• sepsis
• Require inpatient management due to
significant fluid and electrolyte imbalance and
hemodynamic or respiratory compromise.
• Regardless of etiology, the initial management
involves fluid, electrolyte, and nutritional
Staphylococcal Scalded Skin Syndrome
• SSSS is a term used to describe a blistering
skin disease caused by the epidermolytic
toxin-producing S. aureus.
• Tends to occur most often in neonates and
young children.
• Its severity may range from mild, localized
blistering to widespread exfoliation.
• S. aureus  epidermolytic (or exfoliative) toxins
(ET) cleaving the epidermis at the superficial
level of the stratum granulosum.
• ET target desmoglein 1, a cell-cell adhesion
molecule found in desmosomes of the superficial
• Why affects neonates and children?
• – lack of protection from antitoxin antibodies,
• -- decreased renal excretion of the toxin.
• In adults rare, except in the setting of
immunosuppression, malignancy, heart disease,
or diabetes
Clinical findings

• Localized infection of the conjunctivae, nares,

perioral region, perineum, or umbilicus.
• Fever, malaise, lethargy, irritability, poor feeding
subsequent develop, and the generalized
eruption begins.
• Rash: erythema that progresses to large,
superficial fragile blisters that rupture easily 
denuded, desquamating, erythematous, and
tender skin
• Most marked in flexural creases, but may
involve the entire surface area of skin.
• The Nikolsky sign is positive.
• Severe denudation of skin  decreased
thermoregulatory ability, extensive fluid
losses, and electrolyte imbalance, secondary
infection and sepsis.
• With appropriate management, the skin heals
without scarring.
• SSSS is usually diagnosed based on the clinical
• Confirmed by isolation of S. aureus.
• It must be remembered that the majority of
blisters in SSSS are sterile, as they are caused
by the hematogenous dissemination of the
bacterial toxin, not the bacteria itself.
• Biopsy (cleavage in the superficial epidermis at
the level of the granular layer)
Differential diagnosis

• TEN (mucosal involvement)

• Burns
• Epidermolysis bullosa
• Nutritional deficiency dermatosis
• Bullous ichthyosis (in the neonate).