2004

The International Society of Dermatology International Journal of Dermatology

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39

Blackwell Publishing Ltd. Oxford, UK IJD International Journal of Dermatology 0011-9059 Blackwell Publishing Ltd, 2003 45

Education

Erythroderma/exfoliative dermatitis Sehgal, Srivastava, and Sardana

Erythroderma/exfoliative dermatitis: a synopsis

Virendra N. Sehgal,

MD

, Govind Srivastava,

MD

, and Kabir Sardana,

MD, MNAMS

From the Dermato-Venereology (Skin/ VD)
Centre, Sehgal Nursing Home, Panchwati,
Azadpur, Delhi, Skin Institute and School of
Dermatology, Greater Kailash, New Delhi,
and Department of Dermatology and STD,
Lady Hardinge Medical College, New Delhi,
India

Correspondence


Virendra N. Sehgal,

MD

,
Dermato-Venereology (Skin/ VD) Centre,
Sehgal Nursing Home, A/6,
Panchwati, Delhi 110 033,
India
E-mail: drsehgal@ndf.vsnl.net.in

Introduction

Exfoliative dermatitis is an extreme state of skin irritation
resulting in extensive erythema and/or scaling of the body.
Several skin disorders may ultimately culminate in the pres-
entation of erythroderma/exfoliative dermatitis. Largely, it is
a secondary process; therefore, it is mandatory to establish its
etiopathology in order to facilitate its precise management.

1

An increasing number of new drugs have been incriminated in
the recent past.

29

Its clinical pattern is fascinating and has
been the subject of detailed studies.

4,8,1016

Its changing sce-
nario in various age groups,

1719

its presentation postopera-
tively,

2022

and its occurrence in human immunodeficiency
virus (HIV)-positive individuals

2325

are vivid indicators.
Several factors may be responsible for the causation of this
extensive skin disorder. Subtle/sudden generalization of pre-
existing dermatoses may be an intriguing dilemma, and may
reflect an individual variation. A detailed outline of a
patients history to elicit possible triggering events, namely
infections, drug ingestion, topical application of medica-
ments, sun/ultraviolet light exposure, and other factors,

16

may be imperative. It is also challenging to manage the con-
dition, because the intricate process puts an extensive strain
on an already compromised body system. Furthermore, it is
probable that the original dermatosis may be masked by
extensive erythema/scaling, thus making it difficult to obtain
a clear-cut diagnosis. This synopsis is an endeavor to revisit
and update the information available on erythroderma/
exfoliative dermatitis.

Denition

Erythroderma and exfoliative dermatitis are largely synony-
mous; however, erythroderma is the preferred term

1,13,14

and
is currently in vogue. Nonetheless, it is imperative to define
these conditions. The former is characterized by extensive
and pronounced erythema coupled with perceptible scaling,
whereas the latter is conspicuous by the presence of wide-
spread erythema and marked scaling. Accordingly,

90%
skin surface involvement is considered as a salient prerequisite
to make a clinical diagnosis of exfoliative dermatitis.

1,16,2628

Incidence

It is hard to obtain a precise incidence for erythroderma/
exfoliative dermatitis as most reports are retrospective, and do
not address the issue of overall incidence.

8

This aspect was
dealt with for the first time by Sehgal and Srivastava

16

in a
large prospective study from the Indian subcontinent, where the
incidence was recorded as 35 per 100,000 dermatologic out-
patients. In another survey from The Netherlands, the annual
incidence was recorded as 0.9 per 100,000 inhabitants.

29

A
study based on an analysis of 138 consecutive erythroderma
patients from South Africa found that 75% were black,
22.5% Indian and 2.5% white.

24

In addition, a large number
of patients were HIV positive, and a drug reaction was the
most common cause of erythroderma. Furthermore, males
were affected 23 times more frequently, with an average
male to female ratio of 2.3 : 1.

8

In another study from Spain,
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Erythroderma/exfoliative dermatitis

Sehgal, Srivastava, and Sardana

the male to female ratio was found to be as high as 4 : 1.

14

The
incidence as a function of age is usually variable, and any age
group may be affected; however, affected patients are usually
over 45 years of age

1

(excluding hereditary disorders/atopic
dermatitis), with an average age of onset at 55 years. On the
Indian subcontinent, the age of onset was found to be slightly
lower.

16,30

Erythroderma in children has a mean age of onset
of 3.3 years, with a male to female ratio of 0.89 : 1.

18

Etiology

Erythroderma/exfoliative dermatitis may embrace or be
caused by any pre-existing dermatosis, drug-induced reac-
tion, malignancy, and miscellaneous or idiopathic disorder.

Pre-existing dermatoses

Several dermatologic disorders

per se

, or their therapy, can
result in exfoliative dermatitis (Table 1). This is the single
most common cause of adult exfoliative dermatitis in the
majority of studies.

1,11,12,14,16,3136

Psoriasis is the most common cause of exfoliative dermati-
tis amongst the dermatologic disorders;

12,16,30,36

however, in
children, psoriasis is the second most common cause of the
disease, with drugs as the main culprit.

18

Drugs

Topical and systemic medications are notorious for precipi-
tating erythroderma/exfoliative dermatitis. An apparent
increase in the incidence of the disease may be directly pro-
portional to the introduction of new drugs.

8

Apart from the
well-known allopathic medicines, homeopathic, unani,
ayurvedic, herbal, and common home remedies have been
incriminated.

16

Many drug eruptions that commonly present
as morbilliform, lichenoid, or urticarial forms may often
progress to extensive erythema and exfoliation.

8

The inventory of drugs causing erythroderma/exfoliative
dermatitis is increasing;

29,3747

however, the most common
are shown in Table 2.
It is therefore pertinent to provide a inventory of drugs fre-
quently responsible for causing such an episode.
Drug-induced erythroderma due to dapsone/antileprosy
drug hypersensitivity may often mimic cutaneous T-cell lym-
phoma in terms of both clinical features and histopathology.
Fortunately, it resolves after withdrawal of the offending
drug(s) and the administration of supportive therapy.

8

Malignancies

Erythema/exfoliative dermatitis may ultimately be one of the
clinical expressions of reticuloendothelial neoplasms and
internal blood vessel malignancies. The latter invariably
affect older individuals, and erythema/exfoliative dermatitis
is considered to be a salient cutaneous marker of internal
malignancy. Its incidence is around 1%.

8

Lymphomas in gen-
eral and T-cell lymphoma [comprising mycosis fungoides and
Sezary syndrome (its leukemia variant, in particular)] are
often reported to present as exfoliative dermatitis. They con-
stitute over 2540% of cases of malignancy-related erythro-
dermas.

8,2628,32,4851

Exfoliative dermatitis may precede,
accompany, or follow T-cell lymphomas, and its appearance
may be identical to that of benign erythroderma.

8

An immu-
nophenotypic study with the use of advanced antibody panels
may be required to distinguish it from the benign form.

26,52

Reticular cell sarcoma, acute and chronic leukemia, and
malignant histiocytosis are a few other implicated condi-
tions.

8,50,53

Carcinoma of the colon, lung, prostate, thyroid,
fallopian tubes, larynx, and esophagus have also been alleged
to cause the condition.

8,5457

An insidious, debilitating, pro-
gressive course, absence of a history of a previous skin dis-
order, and recalcitrant nature may warrant an exploration of
the possibility of an underlying malignancy.

4,9,54

Miscellaneous/idiopathic disorders

Hepatitis, irradiation, acquired immunodeficiency syndrome
Table 1 Dermatoses frequently resulting in exfoliative dermatitis


Table 2 Common drugs causing exfoliative dermatitis
8

Common Uncommon
Psoriasis Candidiasis
Airborne contact dermatitis Dermatophytosis
Seborrheic dermatitis Mastocytosis
Atopic dermatitis Lichen planus
Staphylococcal scalded
skin syndrome
Reiters syndrome
Phytophotodermatitis Toxic epidermal necrolysis
Photosensitive dermatitis Diffuse/erythrodermic mastocytosis
Pityriasis rubra pilaris Sarcoidosis
Pemphigus foliaceus Pemphigoid
Stasis dermatitis Lupus erythematosus
Ichthyosiform erythroderma Crusted (Norwegian) scabies
Acetaminophen Minocycline
Actinomycin-D Nitrofurantoin
Allopurinol Omeprazole
Arsenic Para-amino salicylic Acid
Barbiturates Penicillin
Captopril Phenothiazine
Chloroquine diphosphate Phenytoin
Chlorpromazine Quinidine
Cemetidine Rifampicin
Dapsone Streptomycin
Gold Sulfadiazine
Hydantoin sodium Sulfonyl urea
Interferon Tetracycline
Isoniazid/ isonicotinic hydrazide Thalidomide
Isotretinoin Tolbutamide
Lithium Vancomycin
Mercurials
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Sehgal, Srivastava, and Sardana

Erythroderma/exfoliative dermatitis

Education

(AIDS), graft-vs.-host disease, Ofuji papuloerythroderma, and
Omenns syndrome can also cause the condition.

8,12,14,51,5861

Despite the best endeavors, a small proportion of patients
remain in whom no clear-cut etiology can be defined and
these are classified under idiopathic disorders. Sustained
efforts during the course of follow-up may lead to the precise
definition of the etiology.

8

Pathogenesis

The pathogenesis of erythroderma/exfoliative dermatitis is
unclear. Currently, it is believed that the condition is second-
ary to an intricate interaction of cytokines and cellular adhe-
sion molecules, including interleukins-1, -2, and -8,
intercellular adhesion molecule-1 (ICAM-1), and tumor
necrosis factor (TNF).

15

These interactions result in a dra-
matic increase in the epidermal turnover rate, causing a
higher than normal mitotic rate and an increase in the abso-
lute number of germinative skin cells. Furthermore, the time
required for cells to mature and travel through the epidermis
is decreased, and is manifested as an increased loss of epider-
mal material, together with a significant loss of protein and
folate.

62

In contrast, the exfoliation of normal epidermis is
much less and contains very little important viable material,
such as nucleic acids, soluble proteins, or amino acids.

63

Abel

et al

.

26

studied the immunophenotypic characteristics
of benign (psoriasis, dermatitis, drug-induced) and malignant
(Sezary syndrome, mycosis fungoides) forms of erythro-
derma, and found them to be similar. In immunohistochemi-
cal studies conducted by Sigurdsson

et al

.,

64,65

the dermal
infiltrate in patients with Sezary syndrome mainly showed a
T-helper-2 cytokine profile, while benign reactive erythro-
derma showed a T-helper-1 cytokine profile, indicating that,
although clinically similar, they have different underlying
pathogenic mechanisms.

Clinical presentation

Exfoliative dermatitis starts as patch(es) of erythema accom-
panied by pruritus. The patch(es) enlarge and coalesce to
form extensive areas of erythema which eventually spread to
cover whole/most of the skin surface. Exfoliative dermatitis
is also associated with profuse scaling, which has its onset
26 days after erythema with individual variations.

1,16,63

The
acute form is heralded by the formation of large scales, whilst
the chronic form is recognized by small scales.

8

The skin is
conspicuously bright red, dry, scaly, hot, and indurated (Fig. 1).
Mild to severe pruritus is usually present. Lichen simplex
chronicus may be its ultimate expression (Fig. 2). In addition,
the nails become thick, lusterless, dry, brittle, and show ridg-
ing of the nail plate. Periorbital skin inflammation and edema
cause ectropion and epiphora. Lymphadenopathy, hepat-
osplenomegaly, edema of the feet/ankles, and gynecomastia
may also be observed. In black people, a widespread loss of
pigmentation is usual. The basal metabolic rate is increased
and a catabolic state causes significant weight loss over
time.

1,8

At times, patients can slip into an irreversible hypo- or
hyperthermia. The former may result in ventricular bradycar-
dia and hypotension. An increased peripheral blood flow may
result in high-output cardiac failure. All body systems may be
affected by these manifestations.

8

The general picture is modified accordingly to the nature of
the underlying disorder, whose etiology and prompt treat-
ment should be addressed. Generalized dermatitis usually
occurs in the sixth or seventh decades of life; however, atopic
dermatitis may occur at any age. Pruritus is at its worst in old
age. Frenk

et al

.

66

reported senile erythrodermic patches with
increased serum immunoglobulin E (IgE) and lactic dehydro-
genase levels together with eosinophilia.
Psoriasis is the most common underlying disorder, and its
features may be present until the whole body develops exfo-
liative dermatitis. In a few cases, generalized pustular psoriasis
may also be present. There may be a history of preceding
plaque(s), treatment with tar, potent steroids, or psoralen
Figure 1 Erythroderma/exfoliative dermatitis, whole of the skin
surface was bright red, dry, scaly, hot and indurated
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Erythroderma/exfoliative dermatitis

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plus UVA (PUVA) therapy, intermittent infections, or emo-
tional stress.

1,4,8,63

A history of drugs for certain dermatoses/
systemic disorders may be elicited prior to the onset of exfo-
liative dermatitis. Erythema is acute in onset and progresses
to generalized exfoliation, which may resolve over the course
of 26 weeks.

1

Erythroderma following pityriasis rubra pilaris is fairly
diagnostic, as it usually starts in childhood or adulthood and
the lesions occupy the hair follicle in the form of papules and/
or plaques with islands of sparing.
Other uncommon causes are lichen planus, pemphigus
foliaceus (Fig. 3), dermatophytosis, ichthyosiform erythro-
derma, crusted (Norwegian) scabies, graft-vs.-host disease,
and irradiation.

1,12,14,16,59,6769

Postoperative erythroderma, a
type of graft-vs.-host disease appearing several days after
surgery along with blood transfusion, is characterized by
erythroderma, fever, pancytopenia, hepatic insufficiency, and
diarrhea, and may prove fatal.

21,22,70

Exfoliative dermatitis
may also develop during seroconversion in HIV-infected
patients with florid manifestations.

23,24,71

Exfoliative dermatitis associated with lymphoma may
show the classical features; however, it may fail to respond to
corticosteroid therapy. It may continue to progress, even if
repeated investigations over months or years fail to pinpoint
any convincing etiology. Efforts should be continued to make
a diagnosis as, sooner or later, lymphoma will be revealed.
Sezary cells/immature neutrophils are diagnostic pointers.
Hodgkins disease may also show unexplained eosinophilia.

1

Hemodynamic/metabolic disturbances

The disease may cause an enormous aberration of body
metabolism. The increased skin blood flow may cause hypo-
thermia and profound heat loss. Compensatory hyperme-
tabolism and an increased basal metabolic rate without any
primary increase in thyroid activity may ensue. Excessive
protein loss through scaling and leaking through skin, hemo-
dilution due to the increased plasma volume, and
hypermetabolism may contribute to hyperalbuminemia and
severe edema. Furthermore, high-output cardiac failure may
occur at any time.

1,7275

Histopathology

The histopathology of exfoliative dermatitis often reveals a
nonspecific picture consisting of orthokeratosis (hyperkera-
tosis, parakeratosis), acanthosis, and a chronic perivascular
inflammatory infiltrate with or without eosinophilia. Botella-
Estradas

et al

.

14

observed that the clinicopathologic correla-
tion in erythroderma is difficult, because the specific features
of the dermatosis are masked by the nonspecific features of
Figure 2 Erythroderma/exfoliative dermatitis, Lichen simplex
chronicus was conspicuous and was characterized by
pigmentation, thickening of the skin and exaggerated skin
marking and marked by pruritis
Figure 3 Erythema/exfoliative dermatitis in pemphigus
foliaceus
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Sehgal, Srivastava, and Sardana

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erythroderma. In a study on Sezary syndrome, the diagnosis
was established by the clonal population of T cells in the
blood, despite a lack of diagnostic features on biopsy.

4

Walsh

et al

.

76

advocated that the submission of multiple simultane-
ous biopsies from the affected skin enhanced the accuracy of
the histopathologic diagnosis, and the cause could be identi-
fied in up to one-half of cases. The stage of the disease can
modify the histopathologic picture; in the acute stage, spong-
iosis and parakeratosis are prominent, whereas, in the
chronic stage, acanthosis and elongated rete ridges are seen.
Zip

et al

.

77

reported that, despite the uniformity of the clinical
expression of erythroderma, diagnostic histopathologic fea-
tures of the underlying disease are retained in the majority of
patients. Skin biopsies from characteristic clinical lesions may
often confirm the diagnosis of psoriasis, pityriasis rubra pilaris,
ichthyosiform erythroderma, or pemphigus foliaceus.

1,16,78,79

Drug-induced exfoliative dermatitis may often reveal a lichenoid
interface dermatosis histopathology.

80

In erythroderma due
to lymphoma, the infiltrate may gradually become polymorphic
until it acquires specific diagnostic features. This makes
repeated skin biopsies, additional investigations of lym-
phocytes in peripheral blood, and sustained follow-up in
dubious situations mandatory to reveal the correct diagnosis.

81

Microscopic clues to the diagnosis of erythroderma, if reviewed
systematically, can reveal the underlying diagnosis (Table 3).
Additional tests to increase the diagnostic specificity include
immunophenotyping and direct immunofluorescence.

4

Investigations/diagnosis

Mild anemia, leukocytosis, increased erythrocyte sedimen-
tation rate, hypoalbuminemia, hyperglobulinemia, and
hyperuricemia are frequent findings.

4,14,32,50

Increased IgE may
be observed in erythroderma when caused by atopic derma-
titis and drug reactions, although it has also been reported in
other settings.

4

In a report by Griffiths

et al

.,

82

a decreased CD4

+

T-cell count was observed in patients with erythroderma in
the absence of HIV disease, as a consequence of sequestration
of the lymphocytes in the skin.

82

Circulating Sezary cells at
greater than 20% are indicative of Sezary syndrome, but at
less than 10% are a nonspecific finding in erythroderma.

10,11

Immunophenotyping, flow cytometry, and, in particular,
B-cell and T-cell gene rearrangement analysis may be helpful
in confirming the diagnosis of lymphoma when it is strongly
suspected.

4,11

Actinic retinoid is differentiated from Sezary
syndrome by the increased CD8

+

T cells in the latter and the
nuclear contour index of peripheral blood lymphocytes.

4

A
detailed guide to investigations is given in Table 4.
Table 3 Histologic clues to the diagnosis of erythroderma

Disease Histologic clues
Psoriasis Parakeratosis, Munros microabscess, suprapapillary plate thinning, squirting papillae,
regular acanthosis
CTCL/Sezary Exocytosis of mononuclear cells, epidermotropism, Pautriers microabscesses
Drug reaction Vascular change, necrotic keratinocytes
Actinic reticuloid Hyperkeratosis, acanthosis, supercial and deep mixed dermal inltrate with some atypical mononuclear cells
Pityriasis rubra pilaris Alternating orthokeratosis and parakeratosis (vertically and horizontally) with or without
keratotic plugging
Sarcoidosis Dermal noncaseating epithelioid naked cell granulomas, occasional giant cells
surrounded by sparse lymphocytes
Contact dermatitis Spongiosis, eosinophils within dermal inltrate
Lymphoproliferative diseases Interstitial pattern of atypical cells between collagen bundles
Scabies Perivascular and interstitial inltrates with eosinophils, scabetic mite/scybala in stratum
corneum
Dermatophytosis Focal parakeratosis, hyphae in stratum corneum
Pemphigus Suprabasal intraepidermal cleavage, acantholytic keratinocytes (acantholytic cells),
direct immunouorescence depicting IgG bound to cell surface, circulating antibodies
Pemphigoid Subepidermal bulla with eosinophils
Acute GVHD Vacuolar change, satellite cell necrosis
Atopic dermatitis Spongiosis, eosinophils within dermal inltrate
Seborrheic dermatitis Parakeratosis with neutrophils at lips of follicular ostia
Dermatomyositis/subcutaneous
lupus erythematosis (SCLE)
Vacuolar change, colloid bodies, increased dermal mucin
Idiopathic subacute Parakeratosis, spongiosis, epidermal hyperplasia, papillary dermal edema, supercial
perivascular lymphohistiocytic inltrate
Idiopathic chronic Compact hyperkeratosis, psoriasiform hyperplasia, little spongiosis, papillary dermal thickening
CTCL, cutaneous T-cell lymphoma; GVHD, graft-vs.-host disease; IgG, immunoglobulin G; SCLE, ?.
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44 Education

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Sehgal, Srivastava, and Sardana

A detailed history of the sequence of events leading to the
development of erythroderma/exfoliative dermatitis is a pre-
requisite in all patients. Often the clues obtained may help in
the diagnosis and appropriate management. A thorough clin-
ical examination is required in order to diagnose the etiology
of exfoliative dermatitis and to allow appropriate urgent
symptomatic treatment. An astute practitioner will be able to
identify the nature of the underlying dermatosis, and proceed
to confirm his or her suspicions.
Histopathology is paramount and is rewarding in over 50%
of cases if a diligent effort is made. Fine needle aspiration
cytology (FNAC) may be vital to distinguish between dermat-
opathic lymphadenopathy and malignant lymphadenopathy.

83

In a recent development, Charry

et al

.

84

concluded that het-
eroduplex analysis of T-cell receptor gamma gene rearrange-
ment can be used as an important diagnostic tool in skin
biopsies to classify the underlying etiology of erythroderma.

Management

All cases should be considered as a dermatologic emergency
and should preferably be hospitalized for treatment. Serious
general medical problems may occur in due course if not
appropriately treated. The initial management of all types of
erythroderma is the same regardless of the etiology. The prin-
ciple of management is to maintain skin moisture, avoid
scratching, avoid precipitating factors, apply topical steroids,
and treat the underlying cause and complications.

4,8,10

The
patient requires a regulated environmental temperature,
avoiding cooling and overheating.

1,4,16

Together with general
management, all unnecessary medication should be avoided.
Cutaneous applications should be soothing and mild due to
the already inflamed skin. Mild topical steroids/emollients
after lukewarm washing can act as an antipruritic. Antihista-
minics (H

1

receptor) can be administered to enhance the
effect. Once the acute irritated state of the skin has improved,
further treatment can be undertaken according to the etiology.
Antimicrobials can be added to control secondary infections.
Any hemodynamic or metabolic aberrations must be
addressed appropriately. Each case requires regular monitor-
ing of protein, electrolyte balance, circulatory status, and
body temperature. Blood urea, serum electrolyte, and fluid
balance should be monitored.
Erythroderma commonly resists therapy until the underly-
ing disease is treated (e.g. phototherapy, systemic medica-
tions in psoriasis).

4

The outcome is unpredictable in
idiopathic erythroderma and the course is marked by multiple
exacerbations; prolonged glucocorticoid therapy is often
needed.

8,10

Appropriate inpatient /outpatient medications are
influenced by the underlying etiology of erythroderma. For
example, prednisone may be contraindicated in exfoliative
dermatitis secondary to psoriasis, whereas retinoids are an
excellent choice for this disease.

4,31

Systemic steroids may be
helpful in some cases, but should be avoided in suspected
cases of psoriasis and staphylococcal scalded skin syn-
drome.

31

Low-dose methotrexate or cyclosporine can be
safely administered in erythrodermic psoriasis.

4,85

Smith and
Skelton

86

found carbamazepine to be effective in the treatment
of psoriatic erythroderma; however, the same drug has caused
exfoliative dermatitis/erythroderma in a few studies.

8789

Similarly, methotrexate therapy for psoriasis has been reported
to cause exfoliative dermatitis.

90

The ideal treatment for
erythrodermic cutaneous lymphoma is still elusive. Various
modalities, such as systemic steroids, PUVA, total body elec-
tron-beam irradiation, topical nitrogen mustard, systemic
chemotherapy, and extracorporeal plasmapheresis, have
been tried with variable results.

9195

A proposed plan of treat-
ment is given in Table 5.

Complications and prognosis

Exfoliative dermatitis is a complex disorder involving many
factors, but the net outcome depends on the underlying dis-
ease. The disease course is rapid if it results from drug allergy,
lymphoma, leukemia, contact allergens, or staphylococcal
scalded skin syndrome.

4,11,16

The disease course is gradual if it
Table 4 Investigations/laboratory tests


Basic investigations
Weight, temperature, pulse, respiratory rate charting
Fluid intake/output charting
Complete hemogram, total and differential leukocyte counts, absolute
platelet count, erythrocyte sedimentation rate
Liver and kidney function tests, including serum electrolytes
Histopathology
Scheduled urine macro- and microscopy
Electrocardiogram (ECG) and chest radiograph
Disease-specic investigations
Skin scrapings/KOH (Norwegian scabies/extensive tinea corporis)
Patch test (after recovery, for suspected allergic contact dermatitis,
photoallergic contact dermatitis, airborne contact dermatitis)
Serum immunoglobulin E (atopic dermatitis)
Serum and urine protein electrophoresis (multiple myeloma)
Angiotensin-converting enzyme levels, serum calcium (sarcoidosis)
Cultures may show bacterial overgrowth or the herpes simplex virus
CD4
+
T-cell count /CD8
+
T-cell count
Human immunodeciency virus 1 and 2 testing, including Western blot
test, to exclude acquired immunodeciency syndrome
Immunology antinuclear antibody, rheumatoid factor, anti-DNA
Fine needle aspiration cytology, lymph nodes, bone marrow examination
(lymphoma/ leukemia)
Direct immunouorescence (pemphigus foliaceus, lichen planus, lupus
erythematosus, graft-vs.-host disease)
Immunophenotyping, ow cytometry, and, in particular, B-cell and T-cell
gene rearrangement analysis if lymphoma is strongly suspected
Work-up for occult malignancy, if suspected: chest radiograph, computed
tomography scan, ECG, ultrasonography of abdomen, stool for occult
blood, mammography, sigmoidoscopy, prostate examination, cervical
smear, as indicated
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45 Sehgal, Srivastava, and Sardana Erythroderma/exfoliative dermatitis Education
results from the generalized spread of a primary skin disease
(e.g. psoriasis, atopic dermatitis).
11,16,31
Drug-induced cases of
exfoliative dermatitis recover completely if initial medical
management is promptly undertaken.
4
Despite skilled efforts, exfoliative dermatitis can some-
times prove fatal, especially in elderly patients. Secondary
infection, dehydration, electrolyte imbalance, temperature
dysregulation, and high-output cardiac failure are potential
complications in all cases.
11,51
Postinflammatory hypopig-
mentation or hyperpigmentation may occur, especially in
individuals with dark skin.
15
Generalized vitiligo or pyogenic
granuloma have also been recorded after exfoliative dermati-
tis.
96,97
Nevi and keloid formation are rare benign sequelae,
as are alopecia and nail dystrophies.
8
In initial documented
studies, the recorded death rate varied from 18 to 64%;
4,16,4850
however, the mortality has been reduced due to advances in
diagnosis and therapy.
References
1 Weismann K, Graham RM. Systemic disease and the skin.
In: Champion RH, Burlain JL, Burns DA, et al., eds.
Textbook of Dermatology, Vol. 3, 6th edn. Oxford:
Blackwell Science, 1998: 27032758.
2 Borroas-Blasco J, Navarro-Ruiz A, Novarro-Blasco F, et al.
Erythroderma induced by omeprazole. Int J Clin Pharmacol
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Table 5 Treatment of erythroderma

General Specic (topical) Specic (systemic) Disease specic
Inpatient care required
Adequate bed rest and sedation
Monitor uid intake/electrolyte balance/temperature
High-protein diet /nutritional support
Discontinue all unnecessary medications
Topical steroids
(triamcinolone
acetonide cream,
0.0251.0%)
under wet dressing
Apply tap water wet
dressings 23-hourly;
gradually reduce
frequency, followed
by application of
emollients
Daily tepid bath
may be soothing
Sedative antihistamine
(H
1
receptor) (hydroxyzine
hydrochloride, 2550 mg
orally every 46 h)/any other
Institute systemic
antimicrobials (to cover
secondary infection
by S. aureus)
Systemic steroids (used
with caution) in atopic
and seborrheic dermatitis
Avoid in psoriasis and infections
Tapered down
Psoriasis: methotrexate
retinoids, phototherapy
Atopic dermatitis: systemic
steroids, antimicrobials,
antivirals
Pityriasis rubra pilaris:
retinoids, methotrexate,
systemic steroids
Toxic epidermal
necrolysis: intravenous
immunoglobulins
Lymphoma: extracorporeal
phototherapy, PUVA,
alkylating agents
Scabies: permethrin, 5%;
ivermectin, 200 g/kg
PUVA, psoralen plus UVA.
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