Figurate Erythemas

Background: Figurate erythemas are a group of distinct conditions with different underlying causes and clinical presentations. They must be differentiated from a wide variety of dermatological entities including mycoses, urticaria, granuloma annulare, pseudolymphoma. Dermatologists need to be familiar with this set of conditions which include erythema annulare centrifugum, erythema gyratum repens, erythema marginatum, erythema migrans and necrolytic migratory erythema which are all important clues to underlying diseases.

Yalçın Tüzün,* MD, Meltem Antonov, MD
Address:Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul, 34098, TurkeyE-mail: yalcintuzun@yahoo.com
* Corresponding author: Prof. Yalçın Tüzün, MD, Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul, 34098, Turkey
Published:
J Turk Acad Dermatol 2007;1 (1):2
This article is available from: http://www.jtad.org/2007/1/02.pdf

ERYTHEMA ANNULARE CENTRIFUGUM
Erythema annulare centrifugum represents
a reaction to a wide variety of triggers [1].
The etiology is unknown in most cases [2].
Triggering Factors in Erythema Annulare Centrifugum
[1, 3]:
* Infection: Chronic dermatophyte infections,
intestinal Candida albicans, molluscum
contagiosum, EBV, genital herpes, Q fever,
urinary system infections, tuberculosis, ascariasis
ie.
* Malignancy: Erythema annulare centrifugum
can be considered an uncommon but
genuine paraneoplastic sign. Bronchial,
prostate, nasopharyngeal, ovarian, rectal
and hepatic tumors, lymphoma and leukemia
are examples.
* Food allergies
* Drug reactions: Aldactone, amytriptilline,
ampicillin, cimetidine, hydrochlorothiazide,
penicillin, piroxicam, salicylates, vitamin K
ie.
* Hematologic conditions: Polycythemia
vera, myelodysplastic syndrome, hypereosinophilic
syndrome, cryoglobulinemia ie.
* Endocrinologic conditions: Hyperthyroidism,
Hashimoto thydroiditis, autoimmune
progesterone dermatitis
* Other: Hepatic disease, after biliary duct
surgery
The condition does not affect a particular
sex or age group. Erythematous macules or
urticarial papules appear first and eventually
spread to form annular shapes with
central clearing [2]. Vesiculation may be
rarely seen [3]. The lesions tend to appear
on the body and proximal parts of the extremities.
Most of the cases spontaneously
recover in weeks [4].
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There are two types of erythema annulare
centrifugum. It is not known whether these
two types are variants of the same pathologic
condition [5].
In the deep type of erythema annulare centrifugum
there is an indurated and apparent
border, without desquamation and the
lesion rarely itches. In the superficial type,
there is desquamation following the advancing
border and itching is more frequent [2].
Histopathologically; in the deep type: The
epidermis is unaffected. There is a “coat
sleeve-like” lymphocytic infiltration in the
mid and deep dermis. In the superficial
type: Epidermal changes such as focal epidermal
spongiosis and focal parakeratosis
and superficial perivascular lymphohistiocytic
infiltrate are present. Endothelial cell
edema and erythrocyte extravasation may
accompany [2, 4]. Eosinophilia may be seen
in some cases both histopathologically and
in peripheral blood. Sometimes erythema
annulare centrifugum may even be an early
sign of hypereosinophilia syndrome [2].
The lesions of erythema annulare centrifugum
may wax and wane and last from
months to years. Most cases resolve spontaneously.
Topical therapies are usually of no
use [2]. Antihistamines and/or systemic
glucocorticoids can be tried but the lesions
recur when treatment is discontinued [6].
The empiric use of antibiotics and antifungal
agents has been reported to be useful in
some cases. The patient may be treated as
for chronic urticaria [2].
ERYTHEMA GYRATUM REPENS
Erythematous bands spread over the body
in waves in this condition [3]. These bands
have been likened to patterns on wood or
the stripes of a zebra [1]. There are some
clinical differences from erythema annulare
centrifugum. These are faster spreading of
the lesions (about 1 cm/day) and existence
of a more pronounced desquamation and
pruritus [3]. There is a characteristic collarlike
desquamation. The lesions appear on
the trunk and extremities [Figure 1]. The
hands, feet and face are usually not affected
[7]. Hyperkeratosis of the palms has been
reported in about 10% of the patients [3].
There is an underlying malignancy in about
80% of the cases of erythema gyratum repens
[3]. For this reason, the patient must
be analyzed carefully for malignancy [1].
The most freqently seen malignancies are
lung, breast and esophagus cancers [4].
Apart from malignancy, tuberculosis,
CREST syndrome, drug hypersensitivity
and pregnancy have also been reported. In
some cases there may be no underlying
cause [3].
Although the appearance of the lesions is
typical, differential diagnosis from atypical
vasculitides, and fungal infections such as
tinea imbricata has to be made histopathologically.
Lupus erythematosus, pemphigoid,
annular psoriasis may also present
similarly [3]. Histopathologically there is
perivascular lymphocytic infiltration resembling
erythema gyratum repens but the infiltration
is concentrated in the superficial
dermis. Additionally epidermal changes are
more frequent [1]. These changes are acanthosis,
spongiosis and parakeratosis [3].
Some authors have identified granular C3,
C4 or IgG deposition in the sublamina
densa region of the basal membrane. This
may indicate that the condition has an immunologic
basis [4].
Treatment of erythema gyratum repens
should be directed to the underlying condition.
Antihistamins may be used for intense
pruritus [1].
ERYTHEMA MARGINATUM
(Erythema Circinatum, Erythema Annulare
Rheumaticum)
It is a sign seen in about 20% of patients
with acute rheumatic fever.1 It is one of the
major Jones’ criteria, along with carditis, migratory
polyarthritis, chorea and subcutaneous
nodules [3]. It is thought to be a response
to streptococcal antigens [1]. It is fre-
J Turk Acad Dermatol 2007; 1 (1): 2. http://www.jtad.org/2007/1/02.pdf
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Figure 1. Erythema gyratum repens
quently seen in children[3] and patients with
active carditis [1]. It is more pronounced in
fair skinned individuals [2]. The trunk is
most freqently affected. It usually appears
after a fever spike in the afternoon. The lesions
are pink, macular or papular and circular.
The disappear within hours or in
maximum 2-3 days [1, 3].
Histopathologically, this condition may be
differentiated from other erythematous conditions
due to infiltration with polymorphic
leukocytes [3].
Erythema marginatum has also been reported
in conditions such as psittacosis and
hereditary angioneurotic edema [3].
ERYTHEMA MIGRANS
Erythema migrans is a lesion that forms at
the location of the tick bite in Lyme disease
[4]. Sometimes the patient is not aware of
the bite [2]. The involved species of Borrelia
are B. burgdorferi sensu lato, B. afzelli or B.
garinii [3].
Classification of Lyme disease [2]:
Early Lyme Borreliosis
Localized infection: Erythema migrans, borrelial
lymphocytoma. No signs of disseminated
infection. Symptoms such as localized
lymphadenopathy and/or malaise may
be present.
Early disseminated infection: Multiple erythema
migrans-like skin lesions. Neuroborreliosis,
arthritis, carditis or other organ
symptoms.
Late Lyme Borreliosis
Chronic infection: Acrodermatitis chronica
atrophicans. Neurologic, joint or other organ
involvement—these should last at least
12 months.
The name erythema “chronicum” migrans is
a misnomer [3]. Most patients do not have
seropositivity against B. burgdorferi. Thus,
even though the gold standard is culture,
diagnosis depends solely on clinical recognition
[8]. The lesion begins as an erythematous
area or red papule 3 to 30 days after
the tick bite. It enlarges in a few weeks and
the center fades [Figure 2]. It reaches a diameter
of 25 cm. The duration of the lesion
is 4-10 weeks.
On histopathologic examination, perivascular
infiltrate containing plasma cells and
eosinophils is seen. Spirochetes are seen
most frequently in the advancing border of
the lesion [4].
Treatment consists of tetracyclin [5].
(doxycyclin 2 x 100 mg/day, 2-3 weeks).
ANNULAR ERYTHEMA OF INFANCY
This condition has typically no difference
from erythema annulare centrifugum but it
is classified as a different condition due to
differences in underlying causes. Whereas
superficial mycoses and malignancies are
rare causes in this age group, lupus erythematosus
and infections definitely should be
ruled out. No cause can be identified in
most cases. C. albicans colonization in the
intestine and EBV infection have been reported.
Histopathology is the same as erythema annulare
centrifugum [3].
There are many types of figurate erythemas
in infancy. In some cases the lesions are
scaly and may resemble T. versicolor. Some
types where there is central atrophy are
named erythema gyratum repens atrophicans
transiens. Due to its histopathological
appearance, there is also a condition named
neutrophilic figurate erythema of infancy.
Treatment consists of a wait and watch policy
if no underlying cause is found [1].
ERYTHEMA GYRATUM PERSTANS
This condition is named familial annular
erythema. The lesions are identical to erythema
annulare centrifugum but there is
autosomal dominant inheritance. It begins
early, sometimes right after birth. Most patients
also have dermographism [1]. Al-
J Turk Acad Dermatol 2007; 1 (1): 2. http://www.jtad.org/2007/1/02.pdf
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Figure 2. Erythema migrans
though the lesions last shorter than erythema
annulare centrifugum, the disease itself
persists for years [7].
NECROLYTIC MIGRATORY ERYTHEMA
This condition is a paraneoplastic sign seen
when there is an underlying glucagonsecreting
malignant pancreas alpha-cell tumor.
There are rare reports of idiopathic cases
or cases due to other gastrointestinal causes
(chronic pancreatitis, chronic hepatitis, colon
cancer) which is called the pseudoglucagonoma
syndrome. Necrolytic migratory erythema
is seen frequently in postmenopausal
women. Male/femal ratio is 3/1. Glucagon or
its metabolites are thought to be responsible.
The lesions start as red-brown macules in perioral
or inguinal regions and later necrotize
and become covered with crusts. Glossitis
may accompany. This appearance is similar to
C. albicans infection. The macules may become
vesicular, widespread and may desquamate
[1].
Epidermal necrosis, pale basal cells,
dyskeratotic cells, acantholysis, subcorneal
pustule formation containing neutrophils
are seen on histopathology. A perivascular
infiltrate composed of lymphocytes and
histiocytes are seen in the dermis.
Serum glucagon levels are quite high. Weight
loss, malaise, intermittent diarrhea, hypokalemia,
resistant diabetes mellitus and anemia
are seen. Since symptoms start before
the tumor can be identified by radiologic
methods, no tumor may be identified.
Zinc deficiency and Hailey-Hailey disease
should be included in the differential diagnosis.
Pustular psoriasis, subcorneal pustular
dermatosis and pemphigus foliaceus
should be included as well.
Symptoms reside after resection of the tumor.
Recurrence of symptoms is a sensitive
indicator of recurrence of the tumor [1].
Keypoints of figurate erythemas are outlined
in Table 1.
Diagnostic approach to figurate erythemas [2]
1. Are there any signs or symptoms of malignancy,
infection or other systemic disease?
2. Are there other findings of tick bite or
Lyme disease?
There are one or two annular lesions
around the tick bite in Lyme disease.
Erythema migrans usually transforms
into plaque form, which is very rare for
erythema annulare centrifugum.
Lesions are multiple in erythema annulare
centrifugum.
3. Are there lesions of urticaria or angioedema?
Urticaria lasts shorter and itches
more than erythema annulare centrifugum.
4. Are there bullous lesions? Bullous pemphigoid
and linear IgA disease also have
urticarial phases.
5. Erythema multiforme should be considered
if the lesions have an oral and acral
distribution.
6. KOH examination should be done.
7. If the lesions are psoriasiform, psoriasis
and subacute lupus erythematosus
should be considered. Rarely Sjogren
syndrome may present with annulare lesions.
Ro/La antibodies should be investigated.
8. Are there any other findings of acute
rheumatismal fever? Erythema marginatum
is the shortest lasting of the figurate
erythemas.
9. Are the lesions located orally or in intertriginous
locations? Are there any other
signs of glucagon excess?
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Erythema annulare
centrifugum
Slowly advancing lesions,
mostly idiopathic
Erythema gyratum
repens
Rapidly advancing lesions,
mostly indicates malignancy
Erythema migrans Annular lesions arising at
location of tick bite, indicator
of Lyme disease
Erythema marginatum
Specific to acute rheumatismal
fever, seen right before
joint involvement
Necrolytic migratory
erythema
Finding of glucagonoma, acral
and perioral location
Annular erythema of
infancy
It is a group of conditions,
underlying causes must be
examined
Familial annular erythema
Very rare, autosomal dominant
Table 1. Keypoints of Figurate Erythemas [1]
10. Is there family history of similar lesions?
Is there anyone in the family with
granulomatous disease? Are phagocytic
functions normal? Annular lesions may
be seen in carrier females with chronic
granulomatous disease.
11. Is the patient an infant? Neonatal lupus
erythematosus must be ruled out in this
age group. Although mycoses are not
seen commonly in infants, they should
be ruled out.
Differential Diagnosis of
Figurate Erythemas [3]
Mycoses, annular urticaria, granuloma annulare,
mycosis fungoides, pseudolymphomas
(especially erythema arciforme et palpible migrans),
bullous pemphigoid, pemphigus, dermatitis
herpetiformis, linear IgA disease, erythema
multiforme, sarcoidosis, Still disease,
annular psoriasis, erythrokeratoderma variabilis,
chronic granulomatous disease, pityriasiform
seborrheic dermatitis, neutrophilic
dermatoses, vasculitides, acute hemorrhagic
edema of childhood, lepra, leishmania, trypanosomiasis.
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