Erythroderma in Adults - UpToDate

7/1/2019 Erythroderma in adults - UpToDate
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Erythroderma in adults
Author: Mark DP Davis, MD, FAAD
Section Editor: Erik Stratman, MD
Deputy Editor: Rosamaria Corona, MD, DSc
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: May 2019. | This topic last updated: Apr 03, 2018.
INTRODUCTION
Erythroderma (literally, "red skin"), also sometimes called exfoliative dermatitis, is a severe and
potentially life-threatening condition that presents with diffuse erythema and scaling involving all or
most of the skin surface area (≥90 percent, in the most common definition). Erythroderma is a
clinical sign and, as such, may be the clinical presentation of a wide range of cutaneous and
systemic diseases (including psoriasis and atopic dermatitis), drug hypersensitivity reactions, and,
more rarely, Sézary syndrome, a leukemic subtype of cutaneous T cell lymphoma. Although
uncommon in pediatric patients, erythroderma may similarly be the clinical presentation of a wide
range of acquired and inherited diseases. The differential diagnosis for erythroderma in pediatric
patients includes infections, inflammatory skin diseases, ichthyoses, and congenital
immunodeficiencies.
This topic will discuss the clinical manifestations, diagnosis, and treatment of erythroderma in
adults. Erythroderma in neonates and infants and Sézary syndrome are discussed separately.
(See "Neonatal and infantile erythroderma" and "Clinical presentation, pathologic features, and
diagnosis of Sézary syndrome".)
EPIDEMIOLOGY
Erythroderma is a rare condition. The annual incidence has been estimated to be approximately 1
per 100,000 in the adult population [1]. In a retrospective study, erythroderma accounted for 13 in
100,000 patients presenting with skin diseases in China [2]. Erythroderma can occur at any age
and in both sexes, but is more frequent in older adults (mean age 42 to 61 years) and in males [2-
6]. Erythroderma is exceedingly rare in children; its prevalence is estimated to be approximately
0.1 percent in pediatric dermatology clinic populations [7,8].
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ETIOLOGY
Cutaneous and systemic conditions — A wide range of cutaneous or systemic diseases can
evolve to or cause erythroderma (table 1).
● Exacerbation of a preexisting inflammatory dermatosis – The most common cause of

erythroderma is the exacerbation of a preexisting inflammatory dermatosis, most often
psoriasis or atopic dermatitis [3-6]. In patients with psoriasis, triggers of erythroderma include
the abrupt discontinuation of systemic corticosteroids or other immunosuppressant therapy,
systemic illnesses, phototherapy burns, medications (eg, lithium, antimalarials), or HIV
infection [9].
● Hypersensitivity drug reaction – A hypersensitivity drug reaction is the second most

frequent cause of erythroderma (approximately 20 percent of cases). A wide variety of drugs
have been reported to be associated with erythroderma, including penicillins, sulfonamides,
carbamazepine, phenytoin, and allopurinol (table 2) [4,10]. Multiple patterns of drug reaction,
from maculopapular/morbilliform eruption to drug reaction with eosinophilia and systemic
symptoms to toxic epidermal necrolysis, may present with erythroderma.
● Uncommon causes – Uncommon causes of erythroderma include cutaneous T cell

lymphoma and other hematologic and systemic malignancies, immunobullous diseases,
connective tissue diseases, and infections (table 1).
● Idiopathic – In approximately 30 percent of cases of erythroderma, no underlying cause is

identified and erythroderma is classified as idiopathic (sometimes called "red man syndrome,"
a term which is also used to describe an infusion reaction to vancomycin) [9,11,12].
PATHOGENESIS
The pathogenesis of erythroderma is incompletely understood. A complex interaction of cytokines

(eg, interleukin-1, -2 and -8 and tumor necrosis factor), chemokines, and intercellular adhesion
molecules is believed to play a role in the massive recruitment of inflammatory cells to the skin
and elevated epidermal turnover. The increased mitotic rate and decreased transit time of
epidermal cells through the skin layers results in exfoliation, with significant loss of proteins, amino
acids, and nucleic acids through the skin.
Increased circulating levels of adhesion molecules (intercellular adhesion molecule-1, vascular cell
adhesion molecule-1, and E-selectin) have been demonstrated in patients with erythroderma
secondary to psoriasis or eczema and in patients with Sézary syndrome [13,14].
Immunohistochemical studies demonstrate a predominantly Th1 cytokine profile in the dermal

infiltrates of patients with erythroderma associated with inflammatory dermatoses and a Th2
profile in the dermal infiltrates of patients with Sézary syndrome [15]. These findings suggest that
different pathophysiologic mechanisms may lead to the relatively uniform clinical presentation of
erythroderma.
CLINICAL MANIFESTATIONS
Onset — Erythroderma may develop acutely over hours or days or evolve gradually over weeks to
months.
The onset is usually abrupt in drug hypersensitivity reactions. A morbilliform or urticarial eruption
may first appear anywhere on the skin, then erythematous patches increase in size and coalesce
into a generalized bright red erythema with occasional islands of sparing (picture 1A-B). Organ
involvement (eg, hepatitis, nephritis, pneumonia) may occur in DRESS (drug reaction with
eosinophilia and systemic symptoms). (See "Exanthematous (maculopapular) drug eruption" and
"Drug reaction with eosinophilia and systemic symptoms (DRESS)".)
Erythroderma from underlying cutaneous or systemic diseases usually develops more gradually.
Erythematous patches may occur anywhere on the skin, enlarge and coalesce over hours to days
to weeks to involve nearly the entire skin surface. Initially, the erythematous patches may have the
characteristics of the underlying disease, but the specific features of the underlying diseases are
often lost after erythroderma has fully developed.
Cutaneous signs and symptoms — By definition, over 90 percent of the skin is involved; the
skin is red and warm to the touch (picture 1B). In light-skinned patients, the color of the skin varies
from bright pink (characteristic of a drug reaction) to a dusky red (characteristic of chronic
erythroderma from many causes). In patients with darker skin tones, these features may be more
subtle. Most patients complain of severe skin pain or itching.
Linear crusted erosions and secondary lichenification may result from rubbing and scratching in
chronic erythroderma. On palpation, the skin may feel leathery and indurated. Scaling is a
common feature, particularly in erythroderma that has been present for more than a week. Scales
can be large, small, or bran-like, and are particularly abundant in patients with underlying
psoriasis.
Palmoplantar keratoderma (hyperkeratosis of the palms and soles) is most often associated with
pityriasis rubra pilaris (picture 2A), but also may occur in patients with Sézary syndrome (picture
3). Nail pitting is characteristic of psoriasis (picture 4).
Moist, crusted lesions on the face and upper trunk often precede the development of erythroderma
in patients with pemphigus foliaceous (picture 5A-B). (See "Pathogenesis, clinical manifestations,
and diagnosis of pemphigus", section on 'Pemphigus foliaceus'.)
Hair (eg, telogen effluvium, scaling of the scalp) and nail changes (paronychia, nail dystrophy, and
onychomadesis [nail shedding]) may be present. Involvement of the eyelids manifests with
blepharitis, epiphora (excessive tearing), and ectropion (eyelid eversion). These features may be
particularly prominent in patients with chronic erythroderma secondary to Sézary syndrome. (See
"Clinical presentation, pathologic features, and diagnosis of Sézary syndrome", section on 'Skin
lesions'.)
Extracutaneous findings — Patients with erythroderma often appear uncomfortable, shiver, and
complain of feeling cold. Constitutional symptoms (eg, malaise, fatigue, fever, or hypothermia) and
signs of high output cardiac failure (eg, peripheral edema, tachycardia) also may be present. (See
"Clinical manifestations, diagnosis, and management of high-output heart failure", section on
'Clinical manifestations'.)
Lymphadenopathy and hepatomegaly or splenomegaly may be observed in chronic erythroderma.

Lymph node biopsy often shows only the features of dermatopathic lymphadenopathy (a benign
reactive lymph node enlargement), but may be diagnostic of lymphoma in patient with cutaneous
T cell lymphoma (CTCL). (See "Clinical presentation and diagnosis of non-Hodgkin lymphoma",
section on 'Lymph node and tissue biopsy'.)
Laboratory abnormalities — Nonspecific laboratory abnormalities may occur in patients with

erythroderma due to various causes, including leukocytosis, anemia, and elevated erythrocyte
sedimentation rate. Eosinophilia may be found in patients with DRESS. (See "Drug reaction with
eosinophilia and systemic symptoms (DRESS)", section on '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. (See "Clinical presentation, pathologic features, and diagnosis of Sézary syndrome",
section on 'Peripheral blood'.)
CLINICAL COURSE
Depending upon the cause, the erythema may become generalized in hours to days, weeks, or
months. Exfoliation typically begins two to six days after the onset of erythema, starts in flexural
areas, and rapidly extends to the entire body surface. Scaling is particularly pronounced in
patients with underlying psoriasis. Over weeks to months, hair and nail changes may occur. (See
'Cutaneous signs and symptoms' above.)
The duration of erythroderma is highly variable. Erythroderma due to drug reactions usually
resolves in two to six weeks after stopping the culprit drug. In patients with drug reaction with
eosinophilia and systemic symptoms (DRESS), resolution of erythroderma may require many
weeks to months. (See "Drug reaction with eosinophilia and systemic symptoms (DRESS)".)
Erythroderma due to underlying cutaneous or systemic diseases may persist for weeks, months,
or years.
COMPLICATIONS
Erythroderma is relatively well tolerated by many patients. However, some patients, particularly
those at the extremes of age and patients with comorbidities, may experience complications. (See
'Hemodynamic and metabolic disturbances' below and 'Infection' below.)
Hemodynamic and metabolic disturbances — Profound disturbances in fluid and electrolyte

regulation, thermoregulation, and metabolic balance occur with erythroderma. Increased skin
perfusion leads to fluid loss by transpiration, and consequent electrolyte imbalance. Heat loss,
hypothermia, and compensatory hypermetabolism associated with hyperthermia may occur. The
shunting of the blood through the skin due to peripheral vasodilation may result in high-output
cardiac failure, especially in older or compromised patients. (See "Causes and pathophysiology of
high-output heart failure".)
Exfoliation of the skin results in significant protein loss that may exceed 9 g/m2 body surface per
day, particularly in patients with erythrodermic psoriasis [16]. The protein loss causes negative
nitrogen balance, hypoalbuminemia, edema, and muscle wasting.
Infection — Inflammation, fissuring, and excoriation increase the susceptibility of the

erythrodermic skin to bacterial colonization. Sepsis from Staphylococcus aureus, including
methicillin-resistant S. aureus, has been reported in erythrodermic patients and is of particular
concern in those who are HIV positive [17-20]. Widespread superinfection with herpes simplex
virus (Kaposi varicelliform eruption) also has been reported in erythrodermic patients [21,22]. (See
"Methicillin-resistant Staphylococcus aureus (MRSA) in adults: Treatment of bacteremia" and
"Epidemiology, clinical manifestations, and diagnosis of herpes simplex virus type 1 infection",
section on 'Eczema herpeticum'.)
DIAGNOSIS
The diagnosis of erythroderma is straightforward; it is made clinically in a patient presenting with

diffuse and generalized erythema and scaling involving 90 percent or more of the body surface
area (picture 1A-B). Determining the cause of erythroderma is more difficult and requires
meticulous clinical assessment and clinicopathologic correlation.
DETERMINATION OF UNDERLYING CAUSE
The underlying cause of erythroderma is often difficult to determine and may remain elusive. In
approximately one third of patients, the cause cannot be determined and erythroderma is
classified as idiopathic. However, ongoing evaluation of patients with idiopathic erythroderma is
important, since the underlying cause may become apparent over time [11,12].
The evaluation of the erythrodermic patient to determine the underlying cause involves a detailed
history, physical examination, skin biopsies, and laboratory tests. Specific tests are performed
based upon the suspected cause.
History — A detailed history is of key importance in establishing the cause of erythroderma.
Important elements of history are:
● History of presenting illness – Onset of symptoms and course of erythroderma
● Past dermatologic and medical history – History of inflammatory skin disease (eg, psoriasis,
atopic dermatitis), preexisting systemic diseases or neoplasia
● Medication history, including over-the-counter medications and supplements
● Family history of inflammatory skin diseases
Physical examination — Physical examination should include a complete examination of the

skin, hair, nails, and mucosae for any sign of underlying skin disease. Lymph node and organ
enlargement should be assessed.
Clinical signs that are nonspecific but may be helpful in suggesting the cause of erythroderma
include:
● Color of erythema – In light-skinned patients, the color of the erythema may be helpful in
ascertaining the diagnosis. Salmon pink/orange color with islands of sparing is typical of
pityriasis rubra pilaris (picture 1A) [23]. A deeper red color associated with exfoliation is
associated with psoriasis or cutaneous T cell lymphoma (picture 1B).
● Scaling – Severe scaling may indicate psoriasis. Crusted scales are seen in pemphigus
foliaceus, whereas exfoliation of large skin sheets is seen in drug reactions. Scaling between
the fingers or burrows involving the web spaces may indicate scabies.
● Bullae – The presence of bullae and the involvement of the mucous membranes may indicate
immunobullous disease (eg, pemphigus, bullous pemphigoid). Moist, crusted lesions on the
face and upper trunk often precede the development of erythroderma in patients with
pemphigus foliaceous (picture 5A-B). (See "Pathogenesis, clinical manifestations, and
diagnosis of pemphigus", section on 'Pemphigus foliaceus'.)
● Keratoderma – Waxy keratoderma of palms and soles with an orange hue is characteristic of
pityriasis rubra pilaris (picture 2A-B), but may also be observed in Sézary syndrome (picture
3).
● Nail abnormalities – Nail thickening, subungual hyperkeratosis, and splinter hemorrhages

are found in psoriasis and pityriasis rubra pilaris (picture 6) [2,24,25]. The presence of nail
pitting is a clue to the diagnosis of erythrodermic psoriasis (picture 4).
● Hair abnormalities – Diffuse alopecia is common in erythroderma from all causes but may be
particularly prominent in Sézary syndrome (picture 7) [26].
● Oral involvement – Oral mucositis is seen in most cases of erythroderma associated with
immunobullous disease, Stevens-Johnson syndrome/toxic epidermal necrolysis, and graft-
versus-host disease.
● Eye involvement – Conjunctival involvement is frequently seen in erythroderma associated

with immunobullous diseases, such as mucous membrane pemphigoid, and Stevens-Johnson
syndrome/toxic epidermal necrolysis. Chronic conjunctivitis may be complicated by the
development of trichiasis and symblepharon and may be associated with the development of
sicca syndrome and corneal perforation.
● Genitourinary involvement – The genitourinary tract may be involved in erythroderma

associated with mucous membrane pemphigoid or Stevens-Johnson syndrome/toxic
epidermal necrolysis.
Skin biopsy and histopathologic examination — Multiple skin biopsies may be necessary to
identify the cause of erythroderma. Skin samples are usually obtained by punch biopsy of multiple
involved sites. (See "Skin biopsy techniques", section on 'Punch biopsy'.)
The histopathology of erythroderma may reflect the underlying etiology. However, histology is
more often unrevealing or nonspecific. Hyperkeratosis, acanthosis, spongiosis, and perivascular
inflammatory infiltrate are frequent findings in erythroderma. The relative prominence of these
features may vary with the stage of the disease and the severity of inflammation (table 3) [27-29].
More specific histopathologic changes may become apparent later in the course of the disease.
Therefore, repeated skin biopsies over time may be needed to establish the diagnosis.
As an example, in the initial phase of Sézary syndrome histology may show a nonspecific
perivascular lymphocytic infiltrate without atypical lymphocytes and an overlying hyperplastic and
parakeratotic epidermis (picture 8) [27]. At a later stage, the infiltrate may become increasingly
pleomorphic and acquire specific diagnostic features, such as atypical cerebriform mononuclear
cells and Pautrier microabscesses (picture 8). (See "Clinical presentation, pathologic features, and
diagnosis of Sézary syndrome", section on 'Light microscopic findings'.)
Immunohistochemistry and T cell receptor gene rearrangement studies should be performed if

atypical lymphocytes are identified in the inflammatory infiltrate by routine histologic examination.
The demonstration of an immunophenotype of T cells lacking mature T cell antigens (CD3+, CD4+,
CD7-) and the clonality of the T cell receptor gene rearrangement support the diagnosis of Sézary
syndrome. The expression of the programmed death-1 (PD-1) may also be helpful in
differentiating Sézary syndrome from erythroderma associated with inflammatory skin diseases. In
one study PD-1 was expressed by over 50 percent of neoplastic CD4+ T cells in 23 of 25 biopsies
from patients with Sézary syndrome and only in 4 of 30 biopsies from patients with erythroderma
associated with inflammatory skin diseases [30]. (See "Clinical presentation, pathologic features,
and diagnosis of Sézary syndrome", section on 'Immunophenotyping confirming T cell origin
(CD3+, CD4+)' and "Clinical presentation, pathologic features, and diagnosis of Sézary
syndrome", section on 'Clonality of the T cell receptor (TCR) gene rearrangement'.)
A predominance of CD8+ lymphocytes in the dermal infiltrate suggests chronic actinic dermatitis
(actinic reticuloid). (See "Photosensitivity disorders (photodermatoses): Clinical manifestations,
diagnosis, and treatment", section on 'Chronic actinic dermatitis'.)
Direct immunofluorescence should be performed if an immunobullous disease is suspected based

upon the presence of intraepidermal bullae or subepidermal bullae or an urticarial appearance of
the erythroderma. (See "Pathogenesis, clinical manifestations, and diagnosis of pemphigus",
section on 'Direct immunofluorescence' and "Clinical features and diagnosis of bullous pemphigoid
and mucous membrane pemphigoid", section on 'Direct immunofluorescence'.)
In approximately 30 percent of cases, the histologic features of erythroderma remain nonspecific

throughout its course and a precise diagnosis of the underlying condition cannot be made.
Laboratory and imaging tests — Laboratory testing is based upon the patient's medical history,
clinical presentation, and suspected cause of erythroderma. The initial laboratory evaluation
includes:
● Complete blood cell count and differential. Leukocytosis is common in all types of
erythroderma. Eosinophilia >700/microL may be found in drug reaction with eosinophilia and
systemic symptoms (DRESS). (See "Drug reaction with eosinophilia and systemic symptoms
(DRESS)", section on 'Laboratory abnormalities'.)
● Routine biochemistry tests including electrolytes, glucose, serum albumin, LDH, liver and
kidney function tests.
● Examination of a peripheral blood smear for the presence of Sézary cells (atypical
lymphocytes with cerebriform nuclei). Counts of Sézary cells >20 percent of examined
lymphocytes suggest Sézary syndrome; counts <10 percent can be found in erythrodermas of
different etiologies and are considered nonspecific. (See "Clinical presentation, pathologic
features, and diagnosis of Sézary syndrome", section on 'Peripheral blood'.)
● Bacterial cultures and assessment of antimicrobial susceptibilities, fungal cultures, and swabs
for polymerase chain reaction tests for herpes simplex virus and varicella zoster virus should
be performed if superinfection of skin lesions is suspected. (See "Diagnosis of varicella zoster

virus infection", section on 'Polymerase chain reaction' and "Epidemiology, clinical
manifestations, and diagnosis of herpes simplex virus type 1 infection", section on
'Polymerase chain reaction'.)
Specific tests that may be helpful in determining the underlying cause of erythroderma include:
● Peripheral blood flow cytometry and T cell clonality – Immunophenotyping and T cell
receptor gene rearrangement studies should be performed to confirm or to rule out the
diagnosis of Sézary syndrome. Findings that support the diagnosis of Sézary syndrome
include: absolute Sézary cell count ≥1000/microL; CD4:CD8 ratio greater than 10; aberrant
expression of pan-T cell markers including CD2, CD3, CD7; deficient expression of CD26 and
CD7 (CD4+CD26- ≥30 percent and CD4+CD7- ≥40 percent); and evidence of a circulating T
cell clone [31]. (See "Clinical presentation, pathologic features, and diagnosis of Sézary
syndrome", section on 'Peripheral blood'.)
● Studies for immunobullous and autoimmune disease – Direct immunofluorescence

studies of skin biopsy specimens are essential for the diagnosis of immunobullous disease.
Biopsies should be taken in perilesional skin (erythematous areas close to bullae or erosions).
The detection of circulating autoantibodies anti-desmoglein 1 and 3 or anti-bullous
pemphigoid antigens BP180 or BP230 confirms the diagnosis of pemphigus or bullous
pemphigoid, respectively. (See "Pathogenesis, clinical manifestations, and diagnosis of
pemphigus", section on 'Serology' and "Clinical features and diagnosis of bullous pemphigoid
and mucous membrane pemphigoid", section on 'Antigen-specific serologic testing'.)
The presence of antinuclear antibodies (ANA) and antibodies against extractable nuclear
antigens (ENA) suggests the diagnosis of lupus erythematosus or other autoimmune disease.
(See "Overview of cutaneous lupus erythematosus", section on 'Diagnosis'.)
Raised ANA and increased serum levels of muscle enzymes can confirm a clinical suspicion
of dermatomyositis; myositis-specific antibodies (eg, Jo-1 and other antisynthetases, anti-Mi-
2, SRP, PM-Scl) may be helpful in identifying subsets of dermatomyositis (picture 9). (See
"Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults", section
on 'Initial evaluation'.)
● Skin scrapings – Scrapings of burrows for mites should be performed and examined under a
microscope in patients with suspected crusted scabies. Potassium hydroxide (KOH)
preparations may be useful to identify hyphae and arthrospores if a generalized dermatophyte
infection is suspected. (See "Scabies: Epidemiology, clinical features, and diagnosis", section
on 'Scabies preparation' and "Dermatophyte (tinea) infections", section on 'Diagnosis'.)
● Imaging studies – In patients in whom erythroderma is suspected to be the manifestation of

occult malignancy, radiologic workup may include chest radiograph, computed tomography
(CT) or magnetic resonance imaging (MRI) (or, alternatively, positron emission

tomography/computed tomography [PET/CT]) of the abdomen and pelvis, colonoscopy,
mammography in women, or ultrasonography of the prostate in men.
TREATMENT
Initial management — Patients with acute or symptomatic erythroderma and patients who are in
any way unstable (particularly patients who are hemodynamically unstable) may require
hospitalization for initial evaluation and treatment. Regardless of the specific etiology, the initial
management involves:
● Assessment and management of skin

● Symptomatic treatment of skin inflammation and pruritus
● Assessment and management of oral mucosa, eyes, genitourinary tract
● Monitoring of the hemodynamic status
● Replacement of fluid and electrolytes
● Monitoring of body temperature
● Nutritional support
● Treatment of cutaneous superinfections
Patients should be placed in a warm (30 to 32°C) and humid environment to prevent hypothermia.
Symptomatic relief of skin pain and itching may include intensive skin care with emollients and wet
dressings.
For the symptomatic treatment of skin inflammation and pruritus, we also suggest low- to mid-
potency topical corticosteroids. We generally use low-potency topical corticosteroids (groups six
and seven (table 4)) for the face and body folds and mid-potency corticosteroids (groups four and
five) for other body areas two to three times per day until improvement.
Oral antihistamines may be helpful in reducing itching in some patients. We prefer first-generation
antihistamines for their sedating effect, especially at night to help with sleep (eg, diphenhydramine
[25 to 50 mg orally every four to six hours for adults and children ≥12 years] or hydroxyzine [25 mg
orally three to four times per day for adults and children ≥6 years]).
Cutaneous infection from S. aureus, including methicillin-resistant S. aureus, is common and

requires prompt institution of systemic antibiotic therapy. (See "Methicillin-resistant
Staphylococcus aureus (MRSA) in adults: Treatment of skin and soft tissue infections", section on
'Antibiotic selection'.)
Cutaneous infection with herpes simplex virus requires treatment with antiviral medications such
as acyclovir, valacyclovir, or famciclovir. (See "Treatment of herpes simplex virus type 1 infection
in immunocompetent patients", section on 'Oral antiviral therapy'.)
If eyes appear involved, ophthalmologic assessment is appropriate. If oral involvement precludes

eating and drinking, consideration can be given to placement of a nasogastric tube or intravenous
fluids. If genitourinary involvement is present, consideration can be given to placement of a urinary
catheter.
Treatment of underlying conditions — After the underlying etiology of erythroderma has been
determined, appropriate treatment of the underlying condition should be added to the initial
management measures. (See 'Initial management' above.)
If a drug hypersensitivity reaction is suspected, all medications that are not essential should be
withdrawn. For these patients, a short course of moderate/high-dose systemic corticosteroids (eg,
prednisone 1 to 2 mg/kg per day) may be beneficial. (See "Exanthematous (maculopapular) drug
eruption", section on 'Management' and "Drug reaction with eosinophilia and systemic symptoms
(DRESS)", section on 'Management'.)
If a patient has Stevens-Johnson syndrome/toxic epidermal necrolysis, consideration should be

given to moving the patient to an environment where expert skin care can be appropriately
provided (eg, burn unit, intensive care unit with expert nursing). (See "Stevens-Johnson syndrome
and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae".)
Patients with erythrodermic psoriasis require systemic therapies including methotrexate,

cyclosporine, acitretin, or biologics (eg, infliximab, etanercept, adalimumab, ustekinumab) [32,33].
(See "Treatment of psoriasis in adults", section on 'Erythrodermic psoriasis'.)
Patients with erythrodermic atopic dermatitis may benefit from systemic corticosteroids or other
immunosuppressants such as cyclosporine, methotrexate, or azathioprine, although most patients
respond to intensive topical treatments alone. (See "Treatment of atopic dermatitis (eczema)".)
Patients with Sézary syndrome may require systemic and skin-directed therapies that target the
circulating Sézary cells and control the skin manifestations. These treatments, which include
extracorporeal photochemotherapy, systemic retinoids, methotrexate, interferon, and brentuximab
vedotin, are discussed separately. (See "Treatment of advanced stage (IIB to IV) mycosis
fungoides".)
For patients with pityriasis rubra pilaris, treatment options include systemic retinoids,
methotrexate, TNF-alpha inhibitors, cyclosporine, and azathioprine. (See "Pityriasis rubra pilaris".)
Management of patients with idiopathic erythroderma — There are no specific treatments for
idiopathic erythroderma. Most patients respond to skin care measures (eg, wet dressings) and
symptomatic treatment of skin inflammation with low- to mid-potency topical corticosteroids and
oral antihistamines. (See 'Initial management' above.)
Patients who fail to respond to topical treatments are often empirically treated with systemic
corticosteroids or other immunosuppressants (eg, methotrexate, cyclosporine). However, evidence
for efficacy of systemic corticosteroids or other immunosuppressant therapy for patients with
erythroderma is scant and limited to small case series and their use in the absence of a precise
diagnosis remains controversial [2,4,12,34]. (See 'Initial management' above.)
For patients with idiopathic erythroderma that does not respond to topical therapy, systemic agents
may be considered if symptoms are severe. We suggest initial treatment with systemic
corticosteroids rather than methotrexate or cyclosporine because they have a more rapid onset of
action (usually within days rather than weeks). We generally use prednisone 0.5 to 1 mg/kg per
day for 7 to 10 days up to a maximum dose of 60 mg per day; prednisone is then slowly tapered
over several weeks to minimize the chances of rebound. During this time other
immunosuppressive agents, such as methotrexate, azathioprine, or mycophenolate mofetil, can
be introduced.
A frequent problem with this regimen is that patients have a great difficulty in weaning from
prednisone because of the high chance of rebound after the interruption of treatment. Patients
should be monitored for potential adverse effects from systemic corticosteroids (in particular, fluid
retention, hypertension, hyperglycemia, increased risk of infection). (See "Major side effects of
systemic glucocorticoids".)
Cyclosporine (4 to 5 mg/kg per day) or methotrexate (10 to 20 mg weekly) may be alternative

therapies for patients for whom systemic corticosteroids are contraindicated. However, these
agents have a slower onset of action and a less predictable antiinflammatory effect than systemic
corticosteroids. Cyclosporine or methotrexate may be continued until the erythroderma is under
control and then gradually weaned to the lowest dose that satisfactorily controls the skin
inflammation. It is generally recommended that cyclosporine be used for less than one year to
avoid renal damage. (See "Cyclosporine and tacrolimus nephrotoxicity".)
Monitoring — It is important that patients with idiopathic erythroderma be reevaluated

periodically, at least every six months. Over time, repeated skin biopsies and other laboratory or
imaging studies may reveal the underlying cause of erythroderma.
PREVENTION
Relapses of erythroderma may be prevented by controlling the underlying cause and avoiding
triggers. Known triggers of erythroderma in patients with psoriasis or atopic dermatitis include the
abrupt discontinuation of corticosteroids or other immunosuppressants; topical application of
irritants; medications (eg, lithium salts and antimalarials in patients with psoriasis); and
phototherapy burns. Patients with a history of erythrodermic drug eruption must avoid reexposure
to the culprit drug.
PROGNOSIS
Erythroderma is a serious disorder associated with both increased morbidity and mortality. The
mortality rate for erythroderma ranges from 4 to 64 percent, with higher rates reported in older
case series. Advanced age, comorbidities, and need for hospitalization are associated with
unfavorable prognosis. In a population-based cohort study from Denmark, 31 and 40 percent of
patients with erythrodermic psoriasis and exfoliative erythroderma, respectively, died within the
first three years following hospital admission, compared with 14 percent of patients hospitalized for
severe psoriasis vulgaris [35].
The prognosis may vary depending upon the underlying condition:
● Erythroderma secondary to psoriasis or atopic dermatitis usually improves within several

weeks to several months after starting appropriate treatment. However, chronic or recurrent
erythroderma is not uncommon in these patients [36].
● Erythroderma due to drug reactions usually resolves in two to six weeks after stopping the
culprit drug but can last for longer.
● Erythroderma in Sézary syndrome and paraneoplastic erythroderma are often refractory to

therapy and have a poor prognosis [37,38].
● Most patients with idiopathic erythroderma have a favorable prognosis. However, they require
clinical monitoring because the underlying diagnosis may become apparent over months to
years (see 'Monitoring' above). In some of these patients, erythroderma may represent a
prelymphomatous condition. In a study of 28 patients with idiopathic erythroderma observed
over a mean period of 33 months, 15 improved, 10 went into remission, and 2 patients
developed a cutaneous T cell lymphoma [11]. In another study, 4 of 38 patients with
erythroderma of unknown etiology followed up for a median of 30 months developed mycosis
fungoides [12].
SUMMARY AND RECOMMENDATIONS
● Erythroderma (exfoliative dermatitis) is a severe and potentially life-threatening condition that

presents with diffuse erythema and scaling involving ≥90 percent of the skin surface area.
(See 'Introduction' above.)
● The most common causes of erythroderma include exacerbation of a preexisting

inflammatory dermatosis, hypersensitivity reactions to drugs, and cutaneous T cell
lymphomas. Less common causes of erythroderma are summarized in the table (table 1). In
approximately one third of patients, the cause of erythroderma remains undetermined
(idiopathic erythroderma); this group requires repeated reassessment as the underlying

diagnosis may become apparent over months to years. (See 'Etiology' above.)
● Erythroderma may develop acutely over hours or days or evolve gradually over weeks to
months. Patients typically present with erythematous patches that increase in size and
coalesce into a generalized bright red erythema with occasional islands of sparing (picture
1A-B). The skin feels warm to the touch and dry. Patients appear uncomfortable, shiver, and
complain of feeling cold. Scaling begins two to six days after the onset of erythema and may
become prominent. Extracutaneous symptoms include fever or hypothermia, peripheral
edema, and tachycardia. (See 'Clinical manifestations' above and 'Clinical course' above.)
● Erythroderma is relatively well tolerated by many patients. However, patients at the extremes
of age and patients with comorbidities may experience complications, including high-output
heart failure, fluid and electrolytic imbalance, heat loss, hypothermia, compensatory
hypermetabolism, protein loss and negative nitrogen balance, hypoalbuminemia, edema,
muscle wasting, and secondary skin infection. (See 'Complications' above.)
● Determining the cause of erythroderma is often difficult and involves a detailed history,
physical examination, skin biopsy, and laboratory studies. In some cases histology reveals the
underlying etiology (table 3), but it is more often nonspecific, and repeated skin biopsies may
be necessary. (See 'Diagnosis' above.)
● Patients who are hemodynamically unstable or those with severe symptoms may require
hospitalization. Regardless of the specific etiology, the initial management involves:
• Replacement of fluid and electrolytes

• Monitoring of the hemodynamic status
• Monitoring of body temperature
• Nutritional support
• Symptomatic treatment of skin inflammation and pruritus
• Treatment of edema
• Treatment of cutaneous superinfections
● For the symptomatic treatment of skin inflammation and pruritus, we suggest topical
corticosteroids and oral antihistamines (Grade 2C). We generally use low-potency to mid-
potency topical corticosteroids (groups 4 to 7 (table 4)) two to three times per day until
improvement. (See 'Initial management' above.)
● After the underlying etiology of erythroderma has been determined, appropriate treatment of
the underlying condition must be initiated promptly. (See 'Treatment of underlying conditions'
above.)
● For patients with idiopathic erythroderma that does not respond to topical therapy, we suggest
systemic corticosteroids rather than methotrexate or cyclosporine (Grade 2C). We generally
use prednisone 0.5 to 1 mg/kg per day for 7 to 10 days. Prednisone is then slowly tapered
over several weeks to avoid rebound while introducing other immunosuppressive treatments.
(See 'Management of patients with idiopathic erythroderma' above.)
● Patients with idiopathic erythroderma should be closely monitored with clinical, histologic,
laboratory, or imaging evaluation, since an underlying etiology may become apparent over
time. (See 'Monitoring' above.)
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REFERENCES
1. Sigurdsson V, Steegmans PH, van Vloten WA. The incidence of erythroderma: a survey
among all dermatologists in The Netherlands. J Am Acad Dermatol 2001; 45:675.
2. Li J, Zheng HY. Erythroderma: a clinical and prognostic study. Dermatology 2012; 225:154.
3. Pal S, Haroon TS. Erythroderma: a clinico-etiologic study of 90 cases. Int J Dermatol 1998;
37:104.
4. Akhyani M, Ghodsi ZS, Toosi S, Dabbaghian H. Erythroderma: a clinical study of 97 cases.

BMC Dermatol 2005; 5:5.
5. Rym BM, Mourad M, Bechir Z, et al. Erythroderma in adults: a report of 80 cases. Int J
Dermatol 2005; 44:731.
6. Sigurdsson V, Toonstra J, Hezemans-Boer M, van Vloten WA. Erythroderma. A clinical and

follow-up study of 102 patients, with special emphasis on survival. J Am Acad Dermatol
1996; 35:53.
7. Sarkar R, Garg VK. Erythroderma in children. Indian J Dermatol Venereol Leprol 2010;
76:341.
8. Sarkar R, Basu S, Sharma RC. Neonatal and infantile erythrodermas. Arch Dermatol 2001;
137:822.
9. Rothe MJ, Bernstein ML, Grant-Kels JM. Life-threatening erythroderma: diagnosing and
treating the "red man". Clin Dermatol 2005; 23:206.
10. Sheen YS, Chu CY, Wang SH, Tsai TF. Dapsone hypersensitivity syndrome in non-leprosy
patients: a retrospective study of its incidence in a tertiary referral center in Taiwan. J
Dermatolog Treat 2009; 20:340.
11. Sigurdsson V, Toonstra J, van Vloten WA. Idiopathic erythroderma: a follow-up study of 28
patients. Dermatology 1997; 194:98.
12. Thestrup-Pedersen K, Halkier-Sørensen L, Søgaard H, Zachariae H. The red man

syndrome. Exfoliative dermatitis of unknown etiology: a description and follow-up of 38
patients. J Am Acad Dermatol 1988; 18:1307.
13. Groves RW, Kapahi P, Barker JN, et al. Detection of circulating adhesion molecules in
erythrodermic skin disease. J Am Acad Dermatol 1995; 32:32.
14. Sigurdsson V, de Vries IJ, Toonstra J, et al. Expression of VCAM-1, ICAM-1, E-selectin, and
P-selectin on endothelium in situ in patients with erythroderma, mycosis fungoides and
atopic dermatitis. J Cutan Pathol 2000; 27:436.
15. Sigurdsson V, Toonstra J, Bihari IC, et al. Interleukin 4 and interferon-gamma expression of
the dermal infiltrate in patients with erythroderma and mycosis fungoides. An immuno-
histochemical study. J Cutan Pathol 2000; 27:429.
16. Kanthraj GR, Srinivas CR, Devi PU, et al. Quantitative estimation and recommendations for
supplementation of protein lost through scaling in exfoliative dermatitis. Int J Dermatol 1999;
38:91.
17. Green MS, Prystowsky JH, Cohen SR, et al. Infectious complications of erythrodermic
psoriasis. J Am Acad Dermatol 1996; 34:911.
18. Jaffe D, May LP, Sanchez M, Moy J. Staphylococcal sepsis in HIV antibody seropositive
psoriasis patients. J Am Acad Dermatol 1991; 24:970.
19. Bakri FG, Al-Hommos NA, Shehabi A, et al. Persistent bacteraemia due to methicillin-
resistant Staphylococcus aureus with reduced susceptibility to vancomycin in a patient with
erythrodermic psoriasis. Scand J Infect Dis 2007; 39:457.
20. Talpur R, Bassett R, Duvic M. Prevalence and treatment of Staphylococcus aureus

colonization in patients with mycosis fungoides and Sézary syndrome. Br J Dermatol 2008;
159:105.
21. Garg G, Thami GP. Psoriasis Herpeticum due to Varicella Zoster Virus: A Kaposi's
Varicelliform Eruption in Erythrodermic Psoriasis. Indian J Dermatol 2012; 57:213.
22. Santmyire-Rosenberger BR, Nigra TP. Psoriasis herpeticum: three cases of Kaposi's
varicelliform eruption in psoriasis. J Am Acad Dermatol 2005; 53:52.
23. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol 1980; 5:105.
24. Klein A, Landthaler M, Karrer S. Pityriasis rubra pilaris: a review of diagnosis and treatment.
Am J Clin Dermatol 2010; 11:157.
25. Suzuki M, Oki T, Sugiyama T, et al. Muscarinic and alpha 1-adrenergic receptor binding
characteristics of saw palmetto extract in rat lower urinary tract. Urology 2007; 69:1216.
26. Bi MY, Curry JL, Christiano AM, et al. The spectrum of hair loss in patients with mycosis
fungoides and Sézary syndrome. J Am Acad Dermatol 2011; 64:53.
27. Ram-Wolff C, Martin-Garcia N, Bensussan A, et al. Histopathologic diagnosis of

lymphomatous versus inflammatory erythroderma: a morphologic and phenotypic study on
47 skin biopsies. Am J Dermatopathol 2010; 32:755.
28. Zip C, Murray S, Walsh NM. The specificity of histopathology in erythroderma. J Cutan
Pathol 1993; 20:393.
29. Vasconcellos C, Domingues PP, Aoki V, et al. Erythroderma: analysis of 247 cases. Rev
Saude Publica 1995; 29:177.
30. Çetinözman F, Jansen PM, Willemze R. Expression of programmed death-1 in skin biopsies
of benign inflammatory vs. lymphomatous erythroderma. Br J Dermatol 2014; 171:499.
31. Nagler AR, Samimi S, Schaffer A, et al. Peripheral blood findings in erythrodermic patients:
importance for the differential diagnosis of Sézary syndrome. J Am Acad Dermatol 2012;
66:503.
32. Rosenbach M, Hsu S, Korman NJ, et al. Treatment of erythrodermic psoriasis: from the
medical board of the National Psoriasis Foundation. J Am Acad Dermatol 2010; 62:655.
33. Viguier M, Pagès C, Aubin F, et al. Efficacy and safety of biologics in erythrodermic
psoriasis: a multicentre, retrospective study. Br J Dermatol 2012; 167:417.
34. Khaled A, Sellami A, Fazaa B, et al. Acquired erythroderma in adults: a clinical and
prognostic study. J Eur Acad Dermatol Venereol 2010; 24:781.
35. Egeberg A, Thyssen JP, Gislason GH, Skov L. Prognosis after Hospitalization for
Erythroderma. Acta Derm Venereol 2016; 96:959.
36. Boyd AS, Menter A. Erythrodermic psoriasis. Precipitating factors, course, and prognosis in
50 patients. J Am Acad Dermatol 1989; 21:985.
37. Kubica AW, Davis MD, Weaver AL, et al. Sézary syndrome: a study of 176 patients at Mayo
Clinic. J Am Acad Dermatol 2012; 67:1189.
38. Kim YH, Bishop K, Varghese A, Hoppe RT. Prognostic factors in erythrodermic mycosis
fungoides and the Sézary syndrome. Arch Dermatol 1995; 131:1003.
Topic 13659 Version 14.0
GRAPHICS
Causes of erythroderma (exfoliative dermatitis)
Idiopathic erythroderma
Inflammatory dermatoses
Psoriasis
Atopic dermatitis
Dermatitis (other than atopic)
Pityriasis rubra pilaris
Lichen planus
Chronic actinic dermatitis
Papuloerythroderma of Ofuji
Sarcoidosis
Drug reactions
Malignancies: hematologic
Cutaneous T cell lymphoma, Sezary syndrome
Other lymphomas (Hodgkin's, non-Hodgkin's)
Leukemias
Malignancies: solid organ

Renal cell carcinoma
Hepatocellular carcinoma
Lung
Colon
Immunobullous disease
Pemphigus foliaceus, vulgaris
Bullous pemphigoid
Paraneoplastic pemphigus
Connective tissue disease

Dermatomyositis
Subacute lupus erythematosus
Infections
Scabies
Dermatophytosis
Congenital cutaneous candidiasis
Staphylococcus scalded skin syndrome
Beta-hemolytic streptococcal erythroderma syndrome
Blood disorders
Hypereosinophilic syndrome
Mastocytosis
Graft-versus-host disease (GVHD)
Graphic 87714 Version 5.0
Drugs most frequently reported as cause of erythroderma
ACE inhibitors (enalapril, lisinopril)
Allopurinol
Bevacizumab
Carbamazepine
Chlorpromazine
Dapsone
Erythropoietin
Gold salts
Hydroxychloroquine
Imatinib
Isoniazid
Penicillin
Phenobarbital
Phenytoin
Piroxicam
Proton pump inhibitors (omeprazole, esomeprazole, pantoprazole)
Retinoids (acitretin, isotretinoin)
Streptomycin
Sulfasalazine
Terbinafine
Thalidomide
Trimethoprim/sulfamethoxazole
Vancomycin
ACE: angiotensin-converting enzyme.
Data from: Grant-Kels JM, Fedeles F, Rothe MJ. Exfoliative dermatitis. In: Fitzpatrick's Dermatology in General Medicine,
8th ed, Goldsmith L, Katz S, Gilchrest B, et al (Eds), McGraw-Hill, 2012.
Erythroderma in pityriasis rubra pilaris
Diffuse erythroderma with characteristic islands of sparing in a patient with

pityriasis rubra pilaris.
Reproduced with permission from: www.visualdx.com. Copyright VisualDx. All rights

reserved.
Erythroderma in Sézary syndrome
Erythroderma, defined as erythema covering at least 80 percent of body surface

area, is present in all patients with Sézary syndrome.
Reproduced with permission from: Non-Hodgkin Lymphomas, 2nd ed, Armitage JO,
Mauch PM, Harris NL, et al. (Eds). Lippincott Williams & Wilkins, Philadelphia 2010.
Copyright © 2010 Lippincott Williams & Wilkins. www.lww.com.
Palmar keratoderma in pityriasis rubra pilaris
Palmar hyperkeratosis associated with pityriasis rubra pilaris.
Reproduced with permission from: www.visualdx.com. Copyright VisualDx. All rights reserved.
Keratoderma in Sézary syndrome
A thickened retention of keratin on the palms and soles, so-called keratoderma

is a common finding and one that can also help to differentiate Sézary syndrome
from other causes of erythroderma.
Nail pits in psoriasis
Numerous pits are present on the nail of this patient with psoriasis.

reserved.
Pemphigus foliaceous
Scales and crusts on a diffuse erythematous base with bacterial superinfection in a patient with
pemphigus foliaceous.
Pemphigus foliaceus
The skin is erythrodermic and covered with large scales and crusts in this patient with pemphigus
foliaceus.
Keratoderma associated with pityriasis rubra pilaris
A waxy keratoderma with an orange hue is characteristic of pityriasis rubra pilaris.
Nail abnormalities in pityriasis rubra pilaris
Nail thickening and subungual hyperkeratosis in patient with pityriasis rubra pilaris.
Diffuse alopecia in Sézary syndrome
Alopecia is common in patients with mycosis fungoides and Sézary syndrome

and can present as reduced hair density diffusely over the scalp and body or as
patches of alopecia.
Histopathologic features of erythroderma
Causes of erythroderma Key histopathologic features
Idiopathic erythroderma Nonspecific features including hyperkeratosis,

parakeratosis, acanthosis, and inflammatory infiltrate
Inflammatory dermatoses
Psoriasis Epidermal acanthosis, parakeratosis, Munro

microabscesses, dilated tortuous papillary blood vessels
Atopic dermatitis Acanthosis, hyperkeratosis, spongiosis
Dermatitis (other than atopic) Acanthosis, hyperkeratosis, spongiosis
Pityriasis rubra pilaris Acanthosis, hyperkeratosis, alternating ortho- and

parakeratosis (horizontal and vertical)
Lichen planus Lymphohistiocytic infiltrate with band-like distribution

upper dermis; obscures the dermoepidermal junction
Chronic actinic dermatitis Acanthosis, hyperkeratosis, spongiosis, perivascular

chronic inflammatory cell infiltrate, occasional atypical
cerebriform lymphoid cells
Papuloerythroderma of Ofuji
Sarcoidosis Noncaseating epithelial cell-like granulomas in the deep

dermis
Drug reactions Histology varies according to the reaction pattern
In exanthematous type, interface dermatitis, vacuolar

changes, necrotic keratinocytes, inflammatory
infiltrate with eosinophils
In Stevens Johnson syndrome/toxic epidermal
necrolysis, partial to full-thickness skin necrosis
Malignancies: hematologic
Cutaneous T cell lymphoma, Sézary syndrome Findings may be subtle and variable. Sézary cells (highly
convoluted cerebriform lymphocytes) along the basal
layer, lichenoid infiltrate, epidermotropism, Pautrier
microabscesses. Absence of spongiosis.
Other lymphomas (Hodgkin's, non-Hodgkin's) Atypical lymphocytes in dermis and epidermis
Leukemias Leukemic cells in dermis and epidermis
Malignancies: solid organ Nonspecific, variable findings
Renal cell carcinoma
Hepatocellular carcinoma
Lung
Colon
Immunobullous disease
Pemphigus foliaceus, vulgaris Acantholysis
Bullous pemphigoid Subepidermal separation
Paraneoplastic pemphigus Acantholysis, lichenoid infiltrate, necrotic keratinocytes
Connective tissue disease
Dermatomyositis Interface dermatitis
Subacute lupus erythematosus Interface dermatitis
Infections
Scabies Scabies mite at junction of epidermis and stratum

corneum; spongiosis, dermal lymphocytic infiltrate
Dermatophytosis Hyphae
Congenital cutaneous candidiasis Hyphae and yeast-like forms
Staphylococcus scalded skin syndrome Vesiculation at level of granular cell layer
Beta-hemolytic streptococcal erythroderma syndrome
Blood disorders
Hypereosinophilic syndrome Dermal eosinophilic infiltrates
Mastocytosis Dermal mast cell infiltrates
Graft-versus-host disease (GVHD) Interface dermatitis
Erythroderma occurring in the first year of life (congenital/neonatal/infant):

additional diagnoses to be considered
Inherited ichthyoses
Epidermolytic hyperkeratosis (bullous congenital Hyperkeratosis, acanthosis, superficial vacuolation,

ichthyosiform erythroderma) clumps of irregular eosinophilic granules in the granular
layer
Nonbullous congenital ichthyosiform erythroderma Acanthosis, hyperkeratosis, focal parakeratosis
Netherton syndrome Epidermal psoriasiform hyperplasia
Rare ichthyoses Hyperkeratosis
Primary immunodeficiencies Nonspecific features including hyperkeratosis,

parakeratosis, acanthosis, and inflammatory infiltrate
Severe combined immunodeficiency (SCID)
Severe combined immunodeficiency - Omenn

syndrome (a form of SCID)
X-linked ectodermal dysplasia with immunodeficiency
Ectodermal dysplasias
Ankyloblepharon-ectodermal defects-cleft Nonspecific features including hyperkeratosis,

lip/palate syndrome parakeratosis, acanthosis, and inflammatory infiltrate
Miscellaneous
Menkes disease
Histologic features of erythroderma associated with

cutaneous T cell lymphoma or inflammatory dermatoses
Morphologic features in representative samples of erythrodermic CTCL and

inflammatory dermatoses. A, C, and E show examples of erythrodermic CTCL,
whereas B, D, and F are from the EID group.
(A) In this SS, skin biopsy did not allow a definite diagnosis, only showing a
perivascular lymphocytic infiltrate without atypical lymphocyte, with an overlying
hyperplastic and parakeratotic epidermis with slight spongiosis.
(B) In this erythrodermic drug reaction sample, a common perivascular
lymphocytic infiltrate is seen, whereas no interface dermatitis and no apoptotic
keratinocytes are identified.
(C) This SS sample shows a very discrete infiltrate in the dermis, but atypical
lymphocytes and Pautrier microabcesses can be identified in the epidermis.
CTCL: cutaneous T cell lymphoma; EID: erytrodermic inflammatory dermatoses; SS:

Sézary syndrome.
Reproduced with permission from: Ram-Wolff C, Martin-Garcia N, Bensussan A, et al.

Histopathologic diagnosis of lymphomatous versus inflammatory erythroderma: a
morphologic and phenotypic study on 47 skin biopsies. Am J Dermatopathol 2010;
32:755. DOI: 10.1097/DAD.0b013e3181cfbfbf. Copyright © 2010 International Society
of Dermatopathology. Unauthorized reproduction of this material is prohibited.
Dermatomyositis
Confluent, violaceous erythema is present on the posterior neck, upper back,

and shoulders. This presentation is often referred to as the "shawl sign" of
dermatomyositis.

reserved.
Comparison of representative topical corticosteroid preparations (classified

according to the US system)
Available
strength(s),
Potency Vehicle Trade names
Corticosteroid percent
group* type/form (United States)
(except as
noted)
Super-high Betamethasone Ointment, optimized Diprolene 0.05

potency dipropionate,
Lotion Diprolene 0.05
(group 1) augmented
Gel Diprolene 0.05
Clobetasol Ointment Temovate 0.05

propionate
Cream Temovate 0.05
Cream, emollient Temovate E 0.05

base
Gel Temovate 0.05
Lotion Clobex 0.05
Foam aerosol Olux-E 0.05
Foam aerosol (scalp) Olux 0.05
Shampoo Clobex 0.05
Solution (scalp) Temovate, Cormax 0.05
Spray aerosol Clobex 0.05
Diflucortolone Ointment, oily cream Nerisone Forte 0.3

valerate (not (United Kingdom,
available in United others)
States)
Fluocinonide Cream Vanos 0.1
Flurandrenolide Tape (roll) Cordran 4 mcg/cm 2
Halobetasol Ointment Ultravate 0.05

propionate
Cream Ultravate 0.05
Lotion Ultravate 0.05
High potency Amcinonide Ointment Cyclocort ¶, Amcort ¶ 0.1

(group 2)
Betamethasone Ointment Diprosone 0.05
dipropionate
Cream, augmented Diprolene AF 0.05
formulation (AF)
Clobetasol Cream Impoyz 0.025

propionate
Desoximetasone Ointment Topicort 0.25
Cream Topicort 0.25
Spray Topicort 0.25
Gel Topicort 0.05
Diflorasone diacetate Ointment ApexiCon ¶, Florone ¶ 0.05
Cream, emollient ApexiCon E 0.05
Fluocinonide Ointment Lidex ¶ 0.05
Gel Lidex ¶ 0.05
Cream anhydrous Lidex ¶ 0.05
Solution Lidex ¶ 0.05
Halcinonide Ointment Halog 0.1
Cream Halog 0.1
Halobetasol Lotion Bryhali 0.01

propionate
High potency Amcinonide Cream Cyclocort ¶, Amcort ¶ 0.1

(group 3)
Lotion Amcort ¶ 0.1
Betamethasone Cream, hydrophilic Diprosone 0.05

dipropionate emollient
Betamethasone Ointment Valisone ¶ 0.1

valerate
Foam Luxiq 0.12
Desoximetasone Cream Topicort LP 0.05
Diflorasone diacetate Cream Florone ¶ 0.05
Diflucortolone Cream, oily cream, Nerisone (Canada, 0.1

valerate (not ointment United Kingdom,
available in United others)
States)
Fluocinonide Cream aqueous Lidex-E ¶ 0.05

emollient
Fluticasone Ointment Cutivate 0.005

propionate
Mometasone furoate Ointment Elocon 0.1
Triamcinolone Ointment Kenalog ¶ 0.5

acetonide
Cream Triderm, Aristocort 0.5
HP ¶
Medium potency Betamethasone Spray Sernivo 0.05

(group 4) dipropionate
Clocortolone pivalate Cream Cloderm 0.1
Fluocinolone Ointment Synalar ¶ 0.025

acetonide
Flurandrenolide Ointment Cordran 0.05
Hydrocortisone Ointment Westcort 0.2

valerate
Mometasone furoate Cream Elocon 0.1
Lotion Elocon 0.1
Solution Elocon ¶ 0.1
Triamcinolone Cream Kenalog ¶ 0.1

acetonide
Ointment Kenalog ¶ 0.1
Aerosol spray Kenalog 0.2 mg per 2 second

spray
Lower-mid Betamethasone Lotion Diprosone 0.05

potency dipropionate
(group 5)
Betamethasone Cream Beta-Val, Valisone ¶ 0.1
valerate
Desonide Ointment DesOwen, 0.05

Tridesilon ¶
Gel Desonate 0.05
Fluocinolone Cream Synalar ¶ 0.025

acetonide
Flurandrenolide Cream Cordran 0.05
Lotion Cordran 0.05
Fluticasone Cream Cutivate 0.05

propionate
Lotion Cutivate 0.05
Hydrocortisone Ointment Locoid 0.1

butyrate
Cream Locoid, Locoid 0.1
Lipocream
Lotion, spray Cortizone 10 0.1

maximum
Lotion Locoid 0.1
Solution Locoid 0.1
Hydrocortisone Cream Pandel 0.1

probutate
Hydrocortisone Cream Westcort ¶ 0.2

valerate
Prednicarbate Cream, emollient Dermatop 0.1
Ointment Dermatop 0.1
Triamcinolone Lotion Kenalog ¶ 0.1

acetonide
Ointment Kenalog ¶ 0.025
Low potency Alclometasone Ointment Aclovate 0.05

(group 6) dipropionate
Cream Aclovate 0.05
Betamethasone Lotion Beta-Val, Valisone ¶ 0.1

valerate
Desonide Cream DesOwen, 0.05

Tridesilon ¶
Lotion DesOwen, LoKara 0.05
Foam Verdeso 0.05
Fluocinolone Cream Synalar ¶ 0.01

acetonide
Solution Synalar ¶ 0.01
Shampoo Capex 0.01
Oil (scalp) Δ Derma-Smoothe/FS 0.01

Scalp
Oil (body) Δ Derma-Smoothe/FS 0.01

Body
Triamcinolone Cream Kenalog ¶, 0.025

acetonide Aristocort ¶
Lotion Kenalog ¶ 0.025
Least potent Hydrocortisone Ointment Hytone 2.5

(group 7) (base, ≥2%)
Cream Hytone, Nutracort ¶ 2.5
Lotion Hytone, Ala Scalp, 2.5 or 2

Scalacort
Solution Texacort 2.5
Hydrocortisone Ointment Cortaid, Hytone, 1

(base, <2%) Nutracort
Cream Cortaid, Hytone, 1

Synacort
Lotion Aquanil HC, Sarnol- 1

HC, Cortizone 10
Spray Cortaid 1
Solution Cortaid, Noble, Scalp 1

relief
Ointment Cortaid 0.5

Cream Cortaid 0.5
Hydrocortisone Ointment Pramosone 1 or 2.5

acetate with
Cream Pramosone, 1 or 2.5
pramoxine 1%
Analpram-HC
combination
Lotion Pramosone, 1 or 2.5
Analpram-HC
Aerosol foam Epifoam 1
US: United States.

* Listed by potency according to the US classification system: group 1 is the most potent, group 7 is the least potent.
Other countries use a different classification system with only four or five groups.
¶ Inactive United States trade name for specific product; brand may be available outside United States.
Δ 48% refined peanut oil.
Data from:
1. Lexicomp Online. Copyright © 1978-2019 Lexicomp, Inc. All Rights Reserved.
2. Tadicherla S, Ross K, Shenefelt D. Topical corticosteroids in dermatology. Journal of Drugs in Dermatology 2009;
12:1093.
3. U.S. Food & Drug Administration Approved Drug Products with Therapeutic Equivalence (Orange Book). Available
at: https://www.accessdata.fda.gov/scripts/cder/ob/default.cfm (Accessed on June 18, 2017).
Contributor Disclosures
Mark DP Davis, MD, FAAD Nothing to disclose Erik Stratman, MD Nothing to disclose Rosamaria
Corona, MD, DSc Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform
to UpToDate standards of evidence.
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● Exacerbation of a preexisting inflammatory dermatosis – The most common cause of

● Hypersensitivity drug reaction – A hypersensitivity drug reaction is the second most

● Uncommon causes – Uncommon causes of erythroderma include cutaneous T cell

● Idiopathic – In approximately 30 percent of cases of erythroderma, no underlying cause is

The pathogenesis of erythroderma is incompletely understood. A complex interaction of cytokines

Immunohistochemical studies demonstrate a predominantly Th1 cytokine profile in the dermal

Lymphadenopathy and hepatomegaly or splenomegaly may be observed in chronic erythroderma.

Laboratory abnormalities — Nonspecific laboratory abnormalities may occur in patients with

Hemodynamic and metabolic disturbances — Profound disturbances in fluid and electrolyte

Infection — Inflammation, fissuring, and excoriation increase the susceptibility of the

The diagnosis of erythroderma is straightforward; it is made clinically in a patient presenting with

DETERMINATION OF UNDERLYING CAUSE

History — A detailed history is of key importance in establishing the cause of erythroderma.

Important elements of history are:

● History of presenting illness – Onset of symptoms and course of erythroderma

● Medication history, including over-the-counter medications and supplements

● Family history of inflammatory skin diseases

Physical examination — Physical examination should include a complete examination of the

● Nail abnormalities – Nail thickening, subungual hyperkeratosis, and splinter hemorrhages

● Eye involvement – Conjunctival involvement is frequently seen in erythroderma associated

● Genitourinary involvement – The genitourinary tract may be involved in erythroderma

Immunohistochemistry and T cell receptor gene rearrangement studies should be performed if

Direct immunofluorescence should be performed if an immunobullous disease is suspected based

In approximately 30 percent of cases, the histologic features of erythroderma remain nonspecific

be performed if superinfection of skin lesions is suspected. (See "Diagnosis of varicella zoster

● Studies for immunobullous and autoimmune disease – Direct immunofluorescence

● Imaging studies – In patients in whom erythroderma is suspected to be the manifestation of

(CT) or magnetic resonance imaging (MRI) (or, alternatively, positron emission

● Assessment and management of skin

Cutaneous infection from S. aureus, including methicillin-resistant S. aureus, is common and

If eyes appear involved, ophthalmologic assessment is appropriate. If oral involvement precludes

If a patient has Stevens-Johnson syndrome/toxic epidermal necrolysis, consideration should be

Patients with erythrodermic psoriasis require systemic therapies including methotrexate,

Cyclosporine (4 to 5 mg/kg per day) or methotrexate (10 to 20 mg weekly) may be alternative

Monitoring — It is important that patients with idiopathic erythroderma be reevaluated

The prognosis may vary depending upon the underlying condition:

● Erythroderma secondary to psoriasis or atopic dermatitis usually improves within several

● Erythroderma in Sézary syndrome and paraneoplastic erythroderma are often refractory to

SUMMARY AND RECOMMENDATIONS

● Erythroderma (exfoliative dermatitis) is a severe and potentially life-threatening condition that

● The most common causes of erythroderma include exacerbation of a preexisting

(idiopathic erythroderma); this group requires repeated reassessment as the underlying

• Replacement of fluid and electrolytes

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4. Akhyani M, Ghodsi ZS, Toosi S, Dabbaghian H. Erythroderma: a clinical study of 97 cases.

6. Sigurdsson V, Toonstra J, Hezemans-Boer M, van Vloten WA. Erythroderma. A clinical and

12. Thestrup-Pedersen K, Halkier-Sørensen L, Søgaard H, Zachariae H. The red man

20. Talpur R, Bassett R, Duvic M. Prevalence and treatment of Staphylococcus aureus

27. Ram-Wolff C, Martin-Garcia N, Bensussan A, et al. Histopathologic diagnosis of

Topic 13659 Version 14.0

Causes of erythroderma (exfoliative dermatitis)

Dermatitis (other than atopic)

Pityriasis rubra pilaris

Chronic actinic dermatitis

Other lymphomas (Hodgkin's, non-Hodgkin's)

Malignancies: solid organ

Connective tissue disease

Subacute lupus erythematosus

Congenital cutaneous candidiasis

Staphylococcus scalded skin syndrome

Beta-hemolytic streptococcal erythroderma syndrome

Graft-versus-host disease (GVHD)