Transcript Erythroderma

Erythroderma
introduction
Erythroderma (literally, “red skin)
sometimes called exfoliative dermatitis
severe and potentially life-threatening
presents with diffuse erythema and scaling
involving all or most of the skin surface
area (≥90 percent)
ETIOLOGY 1
• most common cause: exacerbation[ek,sæsə'beɪʃən]
of a preexisting inflammatory dermatosis—psoriasis
or atopic dermatitis.
• In patients with psoriasis, triggers include abrupt
discontinuation of systemic corticosteroids or other
immunosuppressant therapy, systemic illnesses,
phototherapy burns, medications (eg, lithium,
antimalarials), or HIV infection
10/17/2013
ETIOLOGY 2
• Hypersensitivity drug reaction
• Drugs associated with erythroderma
including:
•
•
•
•
•
10/17/2013
penicillins
sulfonamides
carbamazepine[,kɑ:bə'mæzəpi:n]
phenytoin [fə'nitəuin]
allopurinol [,ælə(ʊ)'pjʊərɪnɒl]
ETIOLOGY 3
• Uncommon causes include:
• cutaneous T cell lymphoma and other
hematologic malignancies
• Systemic malignancies
• immunobullous diseases
• connective tissue diseases
• infections
10/17/2013
ETIOLOGY 4
Erythroderma of unknown origin
•~ 30 percent of cases
•no underlying cause is identified
•classified as idiopathic (sometimes called
“red man syndrome”, which is also used to
describe an infusion reaction to
vancomycin[,væŋkə'maɪsɪn])
10/17/2013
PATHOGENESIS
• incompletely understood
• complex interaction of cytokines,
chemokines, and intercellular
adhesion[əd'hiːʒ(ə)n] molecules
• massive recruitment of inflammatory cells to
the skin and elevated epidermal turnover.
10/17/2013
CLINICAL MANIFESTATIONS
• Onset
• develop acutely over hours or days or evolve
gradually over weeks to months
• onset usually abrupt in drug hypersensitivity
reactions.
• A morbilliform or urticarial eruption may first appear
anywhere on the skin
• then erythematous patches increase in size
• coalesce into a generalized bright red erythema with
occasional islands of sparing.
10/17/2013
CLINICAL MANIFESTATIONS
• Onset
• Organ involvement (eg, hepatitis, nephritis,
pneumonia) may occur in DRESS (drug reaction
with eosinophilia and systemic symptoms).
• Erythroderma from underlying cutaneous or
systemic diseases usually develops more gradually.
• Initially, erythematous patches may have
characteristics of underlying disease
• but specific features often lost after erythroderma
has fully developed.
10/17/2013
CLINICAL MANIFESTATIONS
• Cutaneous symptoms —
• Over 90 percent of the skin is red and
warm to the touch
• severe skin pain or itching
10/17/2013
CLINICAL MANIFESTATIONS
• Linear crusted erosions and secondary
lichenification
• skin may feel leathery and indurated.
• Scaling is a common feature.
• Scales particularly abundant in patients with
underlying psoriasis.
10/17/2013
CLINICAL MANIFESTATIONS
• Involvement of the eyelids
• manifests with blepharitis[,blefə'raɪtɪs],
epiphora[ɪ'pɪfərə] (excessive tearing),
and ectropion[ɛk'tropɪən] (eyelid
eversion).
• particularly prominent in patients with
chronic erythroderma secondary to
Sézary syndrome
10/17/2013
CLINICAL MANIFESTATIONS
• Extracutaneous findings
• Patients often appear ill, with shivering, and
complain of feeling cold.
• Constitutional symptoms (eg, malaise, fatigue, fever,
or hypothermia)
• signs of high output cardiac failure (eg, peripheral
edema, tachycardia) may be present.
• Lymphadenopathy and hepatomegaly or
splenomegaly may be observed in chronic
erythroderma.
10/17/2013
complications
Hemodynamic and metabolic disturbances
•fluid loss by transpiration, and consequent electrolyte
imbalance.
•Heat loss, hypothermia, and compensatory
hypermetabolism associated with hyperthermia may
occur.
•peripheral vasodilation may result in high-output
cardiac failure, especially in older or compromised
patients.
•significant protein loss that may exceed 9 g/m2 body
surface per day, particularly in patients with
erythrodermic psoriasis
10/17/2013
complications
Infection
•increase susceptibility[sə,septɪ'bɪlɪtɪ] of the erythrodermic skin
to bacterial colonization.
•Sepsis from S. aureus, including methicillin-resistant S. aureus,
has been reported in erythrodermic patients and is of particular
concern in those who are HIV positive
•Widespread superinfection with herpes simplex virus (Kaposi
varicelliform eruption) also has been reported in erythrodermic
patients
10/17/2013
LABORATORY ABNORMALITIES
Nonspecific. Including:
•leukocytosis
•anemia
•elevated erythrocyte sedimentation rate
•Eosinophilia may be found in patients with
DRESS.
10/17/2013
LABORATORY ABNORMALITIES
• Atypical lymphocytes with cerebriform nuclei
(Sézary cells) are often observed in
erythroderma regardless of cause.
• Counts of Sézary cells greater than 20 percent
of the circulating peripheral blood
lymphocytes are found in Sézary syndrome, a
leukemic variant of cutaneous T-cell
lymphoma
10/17/2013
DIAGNOSIS
• diagnosis of erythroderma is clinical
• in a patient presenting with diffuse and
generalized erythema and scaling involving 90
percent or more of the body surface area.
10/17/2013
DIAGNOSIS
• Determining the cause of erythroderma is
more difficult
• evaluation involves a detailed history, physical
examination, skin biopsies, and laboratory
tests.
10/17/2013
DIAGNOSIS
History —— A detailed history is of
key importance in establishing the
cause of erythroderma.
10/17/2013
Diagnosis
Important elements of history are:
•History of inflammatory skin disease (eg, psoriasis,
atopic dermatitis)
•Family history of inflammatory skin diseases
•Medication history, including over the counter
medications and supplements
•Preexisting systemic diseases or neoplasia
•Onset of symptoms and course of erythroderma
DIAGNOSIS
• Physical examination should include a
complete examination of
• the skin
• Nails
• mucosae for any sign of underlying skin
disease.
• Lymph node and organ enlargement
should be assessed.
10/17/2013
DIAGNOSIS
Clinical signs that are nonspecific but may be helpful
in suggesting the cause of erythroderma include:
•Scaling –
•Bullae –
•Color of erythema –
10/17/2013
Erythroderma in
Sézary syndrome
10/17/2013
Moist, crusted lesions on the face and upper trunk
often precede the development of erythroderma in
patients with pemphigus foliaceous.
10/17/2013
Pemphigus foliaceous
The skin is erythrodermic and covered with large
scales and crusts
10/17/2013
Hair abnormalities – Diffuse alopecia is common in
erythroderma from all causes but may be
particularly prominent in Sézary syndrome
Diffuse alopecia in
Sézary syndrome
10/17/2013
DIAGNOSIS
• Keratoderma – Waxy keratoderma of palms
and soles with an orange hue is characteristic
of pityriasis rubra pilaris, but may also be
observed in Sézary syndrome.
10/17/2013
Palmar keratoderma in
pityriasis rubra pilaris
10/17/2013
waxy keratoderma associated with
pityriasis rubra pilaris
10/17/2013
Keratoderma in Sézary syndrome
10/17/2013
Nail abnormalities – Nail thickening, subungual
hyperkeratosis, and splinter hemorrhages are found in
psoriasis and pityriasis rubra pilaris. The presence of nail
pitting is a clue to the diagnosis of erythrodermic psoriasis.
Nail thickening
and subungual
hyperkeratosis
in patient with
pityriasis rubra
pilaris
10/17/2013
THANK YOU
key words
exacerbation[ek,sæsə'beɪʃən]
malarial [mə'lɛrɪəl]
vancomycin[,væŋkə'maɪsɪn]
malaise[mæ'leɪz]
blepharitis[,blefə'raɪtɪs]
epiphora[ɪ'pɪfərə]
ectropion[ɛk'tropɪən]
susceptibility[sə,septɪ'bɪlɪtɪ]
aureus['ɔ:riəs]
pityriasis rubra pilaris