EMERGENCY DERMATOLOGY

Erythroderma Key points

Joelle S Dobson C Take a good history, including a thorough drug history (pre-
Nick J Levell scribed and non-prescribed medication); diagnosis is usually
based on the history

C Thoroughly examine the skin, including all mucosal surfaces

Abstract
Erythroderma is a clinical syndrome producing generalized red skin.
C Consider dehydration, secondary sepsis and cardiac and res-
The term ‘erythroderma’ is not a diagnosis: it describes an acute
piratory failure, and check core temperature
dermatological presentation. The presentation can be acute or chronic.
In acute erythroderma, there can be ‘skin failure’ leading to life-
C Intensive care may be needed
threatening systemic manifestations requiring supportive care on an
intensive care unit. In chronic erythroderma, systemic problems are
C Liaise early with a dermatologist before doing a skin biopsy
usually absent. The condition can result from inflammatory or, rarely,
neoplastic processes. Common causes are psoriasis, eczema and
drug eruptions. The presentation can be dramatic, and doctors must
ensure they are not distracted from a diagnostic process based on care- Diagnosis
ful history-taking and examination. Specific skin therapy depends on the
Diagnosis is based mainly on the history, examination and skin
underlying cause and the severity and time course of the condition. Close
biopsy; other investigations are only sometimes useful.
collaboration between general physicians, intensive care physicians and
dermatologists is necessary for successful management.
History
Keywords Drug rash; eczema; erythroderma; psoriasis;
How did the rash start? Was it a sudden-onset generalized rash
suberythroderma
with no preceding history e perhaps suggesting a reaction to a
sudden toxic insult such as a medication, allergy or infection?
Had it been present for years, fluctuating in severity e indicating
chronic eczema or psoriasis? Did it start as a localized itchy rash
and then disseminate e more typical of eczema? Did it start as
Common causes
multiple small patches that became confluent following a sore
Erythroderma (Table 1) is not a skin diagnosis but a clinical throat e pointing towards erythroderma following guttate pso-
syndrome with many different causes. The common causes riasis? Did it start gradually and insidiously spread over months
presenting to doctors in secondary care are psoriasis, atopic to cover the body with a non-itchy rash e perhaps pointing to-
eczema, seborrhoeic eczema, other forms of eczema and drug wards cutaneous lymphoma?
eruptions. Erythroderma in the neonate may result from inheri-
ted disorders or staphylococcal scalded skin syndrome. Table 2 Does the patient have a past history of eczema (dermatitis) or
lists some causes of erythroderma. psoriasis? Many patients do not realize they have mild psoriasis.
A history of scaling of the scalp, elbows and knees can be
important. Others may not realize that their dry, discoloured or
Joelle S Dobson MB BS MRCP is a Clinical Fellow in Dermatology at sensitive skin is eczema.
the Norfolk and Norwich University Hospital, UK. She is from
Canada, and moved to the UK to train in medicine at St George’s, Have any drugs recently been started? Specifically ask about all
University of London, UK. She is doing a postgraduate certificate drugs, vitamins, herbal remedies and alternative medicines. Ask
in medical education through the University of Dundee, UK. She about remedies taken intermittently for the bowels, cramps and
enjoys teaching medical students at the Norwich Medical School
analgesia. A new medicine typically causes a rash within a few
and assisting with NIHR research projects at the Norfolk and
Norwich University Hospital. Research interests are palmoplantar days, but this can be delayed by many months or even years.
dermatitis and cutaneous viral warts. Competing interests: none Withdrawal from oral corticosteroids or initiation of lithium
declared. treatment can trigger erythrodermic psoriasis, but many other
drugs have been implicated.1
Nick J Levell MD FRCP MBA is a Consultant Dermatologist at the
Norfolk and Norwich University Hospital, UK. He is Specialty National
Lead (Dermatology) for the UK NIHR, Past-President of the British
Society for Medical Dermatology and President of the British
Association of Dermatologists. He has wide clinical research interests Types of erythroderma
working with the UK Dermatology Clinical Trials Network to deliver
Erythroderma
studies through the NIHR. He also has research interests in
epidemiology, health economics, health service delivery and history
C >90% of the skin is red
of medicine. Competing interests: as a part of the role as NIHR lead Sub-erythroderma
and BAD President, he engages with all major pharmaceutical C 70e90% of the skin is red
companies in the UK to encourage investment, financial support of
educational events and fellowship awards. Table 1

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 EMERGENCY DERMATOLOGY

Has the patient had previous allergic reactions to drugs? Ask

Causes of erythroderma about allergies to creams (some drugs are topical). Ask about
C Psoriasis application of cosmetics and sun creams.
C Eczema (e.g. atopic, seborrhoeic)
C Drug eruptions (e.g. drug reaction with eosinophilia and systemic Is the patient photosensitive? Ask about sun exposure and
symptoms, acute generalized exanthematous pustulosis, toxic sunbeds. Consider phototoxic drug reactions, other photo-
epidermal necrolysis) dermatoses and systemic causes such as systemic lupus and
C Pityriasis rubra pilaris variegate porphyria.
C Cutaneous lymphoma (e.g. Sezary syndrome)
C Crusted scabies General history: important systemic symptoms of skin failure
C Staphylococcal scalded skin syndrome and toxic shock syndrome include thirst, due to dehydration, and abnormal temperature
C Congenital ichthyoses (many different diseases) perception with shivering. Ask about symptoms relating to un-
C Pemphigus and bullous pemphigoid derlying malignancy using a review of systems.1
C Netherton’s syndrome and other rare genodermatoses
Contacts: if family or friends are itching, consider scabies.
Table 2 Crusted (Norwegian) scabies is highly contagious.

Management of erythroderma

History, examination & resuscitation

e.g. adult respiratory distress syndrome,
sepsis, high output cardiac failure

Acute Chronic

Stable Unstable Stable

Admit ± ITU input if Consider manage as outpatient (OP)

critically unwell with frequent OP follow-up

Remove suspected triggers or exacerbating factors

e.g. stop new drug, keep away from UV exposure

Temperature control Fluid balance Barrier Other

• Isolation room to better • Monitor input and output – • Barrier nurse • Analgesia
regulate environment’s consider urinary catheter • Sepsis management – • Nutritional support –
temperature sepsis screen, involve dietitian
• If hypothermic – antibiotics • If eye involvement –
add clothing layers, • Emollients opthalmologist input
bear hugger • Dressings –
• If hyperthermic – non-adhesive,
strip clothing, fan circumferential self
adherent

Investigate underlying cause (e.g. skin biopsy) and tailor treatment accordingly

Figure 1

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 EMERGENCY DERMATOLOGY

Clinical examination Treatment

General examination must include the following: measurement
General principles
of core temperature to identify hypothermia; assessment for
Skin controls body temperature, retains fluid and acts as a barrier
dehydration; and examination of the respiratory and cardiovas-
against infection. Patients with erythroderma can develop skin
cular systems for signs of sepsis, adult respiratory distress syn-
failure and loss of these important functions. Skin failure is
drome and high-output cardiac failure.2
characterized by erythroderma, oedema, tachycardia, hypother-
mia or hyperthermia; it is more dangerous in neonates and in the
Skin examination: full examination of the skin, mucosae, scalp
elderly, who are more susceptible to secondary infection. Inten-
and nails is needed. Are there islands of uninvolved skin
sive care monitoring of fluid balance and core temperature is
sparing e suggesting pityriasis rubra pilaris (PRP)? Are there
sometimes needed in acute severe erythroderma. In extreme
blisters and crusts e think about bullous autoimmune (e.g.
cases, particularly in elderly individuals, high-output cardiac
bullous pemphigoid) and secondary infections? Is there pitting
failure and acute adult respiratory distress syndrome can occur.
or onycholysis of the nail bed e think about psoriasis? In acute
Advice from multiple specialists, particularly intensivists in the
erythroderma, the nail bed can completely separate (onychol-
acute phase, may be needed.
ysis) and be lost. Clues to the cause of the erythroderma can be
Good skin care is essential. Avoid adhesive tape on fragile
subtle.
skin to avoid tearing; hold dressings in place with circumferential
An examination of other systems is important. Palpate for
bandaging that adheres to itself. Use beds or mattresses that
lymphadenopathy and hepatosplenomegaly. Lymphadenopathy
minimize pressure damage. Pain relief is essential in acute
is a common finding in erythroderma of all causes because of
erythroderma.
the inflammation in the skin; however, haematological malig-
Swab the skin and affected mucosa regularly in patients with
nancy or paraneoplastic disease should be considered if the
acute disease to identify secondary infection early. Reverse bar-
lymphadenopathy fails to resolve as the skin clears. Examine
rier nurse to protect against hospital infections.
the lungs and heart for signs of high-output cardiac failure or of
Patients are catabolic, and serum albumin can fall consider-
an infection that might have triggered the erythroderma. Signs
ably. Nutritional support may be needed, and expert dietetic
suggestive of chronic erythroderma are ectropion of the eyes,
advice should be sought.
diffuse alopecia and nail dystrophy.1
Figure 1 provides a summary.
Dermatoscopy: burrows may be seen in Norwegian scabies.
Dermatoscopic differences in erythroderma have been suggested
in a small series by Errichetti et al.3 in patients with psoriasis,
atopic eczema, mycosis fungoides (MF) and PRP, although this is
controversial.

Investigation
Skin biopsy: this may not be necessary if there is a clear history
and signs of generalized spread of pre-existing eczema or psori-
asis. Histology can be useful to confirm severe drug rashes (see
Drug eruptions on pages xxx of this issue), to distinguish be-
tween different inflammatory conditions of the skin where there
is no history or precedent, and to diagnose cutaneous T cell
lymphoma.
Skin biopsy for immunofluorescence (for autoimmune
bullous disorders) and immunohistochemical testing (e.g. T cell
markers in cutaneous T cell lymphoma) can be useful. Several
biopsies can be required from morphologically different areas or
if the presentation changes. PRP and MF produce characteristic
histology, and in lymphoma a T cell clone may be isolated.
In all cases of erythroderma, consult with a dermatologist
before taking the biopsy to ensure that samples are taken from
the correct area and for the appropriate tests. An inexperienced
person can accidentally biopsy a coincidental benign cutaneous
lesion, mistaking it for a florid area of the rash and causing
diagnostic confusion. Biopsies should be taken from areas that
have not been scratched to avoid confusion because of secondary
changes.

HIV infection: severe psoriasis and crusted scabies can be found

in HIV. The seroconversion illness can produce generalized Figure 2 Erythrodermic eczema demonstrating lichenification behind
erythema. the knees. The skin markings are more obvious than normal.

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 EMERGENCY DERMATOLOGY

Specific conditions causing erythroderma (Table 2)

Eczema
Lichenification (thick skin caused by scratching and rubbing) is
characteristic of chronic eczema (Figure 2). Acute eczema tends
to be weepy and crusty, and can blister. Secondary infection with
either bacteria (Staphylococcus, Streptococcus) or virus (herpes
simplex) must be considered. If multiple tiny vesicular or crusted
lesions are present, consider eczema herpeticum (caused by
herpes simplex). Take swabs for bacterial and herpes viral cul-
ture (in the correct medium) and treat with oral antibiotics and/
or aciclovir.
Acute weepy eczema can require antiseptic soaks such as
potassium permanganate or saline. Dryer areas require emol-
Figure 4 Pustular psoriasis e the pustules may be hard to see without
lients such as a 50/50 mixture of liquid paraffin and white soft a lens.
paraffin. An emulsifying ointment should be used for bathing.
Very potent topical corticosteroids are needed in acute eczema.
Patients with chronic erythrodermic eczema require assess- Acute unstable or pustular psoriasis is usually treated with
ment by a consultant dermatologist to exclude underlying trig- twice-daily topical moderate-potency corticosteroids and bed
gering factors such as allergy. Such patients may require rest. Streptococcal infection should be treated. Systemic treat-
systemic azathioprine or ciclosporin as longer term ments with oral ciclosporin or methotrexate are used. In patients
corticosteroid-sparing agents. who do not respond to these medications, anti-tumour necrosis
factor and other biological treatments are used in the UK ac-
Psoriasis cording to National Institute for Health and Care Excellence
Erythrodermic psoriasis ranges in colour from deep red to guidelines.4 Systemic corticosteroids should be avoided in pso-
salmon pink, often with scale (Figure 3). There can be pustules riasis as withdrawal can be difficult, leading to severe flares.
or peeling (desquamation) if it is acute (Figure 4). In chronic
psoriasis, there can be considerable scaling. Psoriasis is associ- Drug reactions
ated with arthritis, nail pits and onycholysis. The suspected offending drugs should be withdrawn, but it can
take several months for the rash to settle completely (see Drug
eruptions on pages xxx of this issue). In toxic epidermal nec-
rolysis, a condition with a high mortality, the whole epidermis
can necrose and slough off. Drug reaction with eosinophilia and
systemic symptoms (DRESS) is associated with hepatitis and,
more rarely, nephritis and pneumonitis.5 Acute generalized
exanthematous pustulosis is associated with fever and eryth-
roderma with pustules.

Cutaneous T cell lymphoma

Sezary’s syndrome causes erythroderma. In MF, there can be
widespread red, scaly plaques and, less commonly, tumour
nodules, ulcers, lymphadenopathy and organomegaly. Skin

Figure 5 Congenital bullous ichthyosiform erythroderma in a neonate

e a very similar clinical appearance to staphylococcal scalded skin
Figure 3 Suberythrodermic psoriasis. syndrome.

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 EMERGENCY DERMATOLOGY
lymphoma should always be managed by dermatologists as part
of the multidisciplinary cancer team. Treatments include topical
corticosteroids and phototherapy in the early stages.
Pityriasis rubra pilaris
This rare cause of erythroderma produces scaling with charac-
teristic small areas or ‘islands’ of skin sparing, perifollicular er-
ythema and hyperkeratotic palms, which have a characteristic
orange appearance. It tends to spread cephalocaudally (from
head to toe).
Staphylococcal scalded skin syndrome
Sheets of bright red skin peel off (desquamate) e often in neo-
nates or young children e from the action of a bacterial toxin.
The appearances can be very similar to congenital bullous ich-
thyosiform erythroderma (see Figure 5) and other inherited skin
diseases, so liaise early with a paediatric dermatologist when
managing erythrodermic neonates. A
TEST YOURSELF
To test your knowledge based on the article you have just read, please complete the questions below. The answers can be found at the
end of the issue or online here.
Question 1
A 70-year-old man presented with a 24-hour history of an itchy,
widespread, red pustular rash. He had been discharged 1 week
previously after an exacerbation of severe bronchiectasis.
On clinical examination, he was shivering, with a temperature of
37.0 C, blood pressure 100/70 mmHg, pulse 100 beats/minute,
respiratory rate 20 breaths/minute and oxygen saturation on
room air 96%. The jugular venous pressure was not visible, the
heart sounds were unremarkable, the chest was clear and
abdominal examination was unremarkable. More than 90% of
the skin was erythematous.
What is the most likely triggering medication for the rash?
A. Ceftazidime
B. Lactulose
C. Paracetamol
D. Prednisolone
E. Salbutamol
Question 2
A 20-year-old man presented with a 3-week history of eryth-
roderma. He had recently lost weight unintentionally. His past
medical history included asthma and allergic rhinitis.
On clinical examination, there were clear fluid-filled vesicles on
his hands with fissured, weeping and lichenified skin but no
crusting. There were enlarged lymph nodes in the neck and axillae.
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KEY REFERENCES
1 Sterry W, Steinhoff M. Erythroderma. In: Bolognia JL, Jorizzo JL,
Schaffer JV, eds. Dermatology. 3rd edn. Philadelphia, PA: Elsevier
Saunders, 2012; 171e81.
2 Rothe MJ, Bernstein ML, Grant-Kels JM. Life-threatening, eryth-
roderma: diagnosing and treating the ‘red man’. Clin Dermatol
2005; 23: 206e17.
3 Errichetti E, Piccirillo A, Stinco G. Dermoscopy as an auxiliary tool
in the differentiation of the main types of erythroderma due to
dermatological disorders. Int J Dermatol 2016; 55: e616e8.
4 National Institute for Health and Care Excellence. Psoriasis:
assessment and management. 2012. Clinical Guideline no. 153,
https://www.nice.org.uk/guidance/CG153/chapter/1-
Guidance#systemic-therapy (accessed 8 May 2017).
5 Walsh SA, Creamer D. Drug rash with eosinophilia and systemic
symptoms (DRESS): a clinical update and review of current
thinking. Clin Exp Dermatol 2011; 36: 6e11.
What is the most likely cause of his lymphadenopathy?
A. HIV seroconversion
B. Neoplasm
C. Reactive
D. Secondary to herpes simplex
E. Sezary syndrome
Question 3
A 67-year-old woman presented with a 2-month history of a
widespread rash. It had started on her head and then spread
down to the trunk and limbs. She had tried a moisturizer, but the
rash had worsened over the previous few weeks. She had pre-
viously been well and had not started new medication for over a
decade.
On clinical examination, there was a widespread erythematous
rash with spared patches of uninvolved skin approximately 1 cm
across on her left knee and upper chest. The palmoplantar regions
were hyperkeratotic and orange. There was diffuse alopecia.
What is the most appropriate next step to support the likely
diagnosis?
A. Request a skin biopsy
B. Do a full blood count and blood film
C. Look for scabies burrows on the hands
D. Patch testing
E. Take a fungal skin scrapping
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