ARTICLE
Auteur(s) : Burkhard Kreft,
Wolfgang Christian Marsch
Department of Dermatology and Venereology,
Martin-Luther-University Halle-Wittenberg, Department of
Dermatology and Venereology, Ernst-Kromayer-Str. 5, D-06097 Halle
(Saale), Germany
accepté le 31 Juillet 2006
Lupus erythematosus is a disease which may have many clinical
variants [1]. It is necessary to distinguish subacute-cutaneous
lupus erythematosus from other dermatological diseases.
Subacute-cutaneous lupus erythematosus (SCLE) is defined as a
nonfixed and nonscarring subset of LE with exacerbating and
remitting nature [2]. It is regarded as an illness intermediate
between discoid LE and severe systemic LE. The clinical diagnosis
is based on erythematous and papulosquamous lesions in
characteristic distribution (UV-exposed skin) often resolving with
grayish hypopigmentation. Musculoskeletal complaints and abnormal
serologic findings are often present [2]. Annular polycyclic
lesions are observed in about one-third of patients with subacute
cutaneous lupus erythematosus.The so-called “lupus erythematosus
gyratus repens” is assumed to be a rare, clinically and
histologically specific annular variant of subacute-cutaneous lupus
erythematosus (SCLE). The differential diagnosis with other annular
subtypes of SCLE such as erythema anulare centrifugum-like SCLE,
can be difficult. Concerning the nomenclature there is no general
consent. However in lupus erythematosus gyratus repens, a certain
clinical and histological independence of the disease must be
assumed. We therefore think that it is important to define lupus
erythematosus gyratus repens as a separate variant of SCLE. A
characteristic feature of LE gyratus repens is double-contoured
concentric scaly erythemas. This is not seen in other annular
erythemas. Typical findings in histology are a marked atrophy of
the epidermis and a hydropic degeneration of the basal layer.
Immunoserologically, proof of antinuclear antibodies is often found
which, however, may be directed against different target antigen
structures. Therapeutically, monotherapy with topical
glucocorticoids is apparently often not adequately effective. The
response to, and effect of, antimalarials is subject to some
controversy [3]. To date, only 8 patients [3-11] with lupus
erythematosus gyratus repens have been described, of whom 3 cases
are in the French literature. We wish to add another case and
thereby emphasize the importance of differential-diagnostic
delineation from other dermatoses.
Case report
A 74-year-old man reported a pruriginous, annular reddening with
scaling which had developed over a period of months on his trunk
and extremities. His general well-being was otherwise unaffected.
Under outpatient systemic treatment with glucocorticosteroids at a
dose of 10-50 mg prednisolone daily, the findings initially
improved considerably, but the disease always returned when therapy
was withdrawn. Due to pre-existing arterial hypertension and a
peripheral arterial occlusive disease, the patient was on long-term
medication with benalapril, nitrendipin and acetylsalicylic acid.
The disease was, however, manifest before these drugs were
prescribed.
On first examination of the patient in our clinic, we observed
moderate pruriginous annular and garland-like configured, slightly
infiltrated, sometimes confluent, livid-red erythema with
pityriasiform, periphery-emphasized scaling on the back and
neckline and on the extensor sides of the arms. Impressive was the
formation of new, ring-formed, periphery-emphasized,
erythemato-squamous lesions within already existing skin changes,
which was especially recognizable on the back (( figure 1A )).
The histological examination of a tissue sample taken from the
edge of a lesion showed a sharply defined, perivascular, dense
lymphocytic infiltrate in the upper corium, which could also be
found periadnexial (e.g. around the dermal sweat gland excretion
pores). On the interfollicular epidermis, there was lymphocytic
aggression on keratinocytes of the Stratum basale with formation of
keratinocyte necrobioses, some with vacuoles, some with hyaline.
Conspicuous here was a dense, compact, increased eosinophilic,
parakeratotic horny layer over a certain area, whereby the vital
epidermis was reduced to about 2-4 layers. A diffuse increased
deposit of acidic mucins was observed in the entire upper dermis ((
figures 1B and
1C )).
Immunoserological examination revealed antinuclear antibodies
(ANA 1:320). Strongly positive Ro- (Ratio 3.5; normal < 1.0) and
positive La-antibodies (Ratio 2.2; normal < 1.0) were
conspicuous in the immunoserological profile. Ds-DNA-antibodies,
histon-antibodies, cardiolipin-antibodies and circulating immune
complexes were within the normal physiological range. Native
preparations and cultures of scaling material were negative for
dermatophytes. An orienting general examination (chest X-ray,
sono-abdomen, gastroscopy, hemoccult, PSA, CEA) brought no evidence
of malignant tumor activity.
Based on the clinical, histological and immunoserological
findings, we reached the diagnosis of a gyrate variant of cutaneous
lupus erythematosus (lupus erythematosus gyratus repens).
The outpatient treatment began several weeks previously with
prednisolone with a dose of 10 mg/d orally and was tapered off
within 10 days. Instead we applicated a potent topical
glucocorticosteroid (clobetasolpropionat) which initially
overlapped with the systemic administration of the
glucocorticosteroid. After ruling out ophthalmological
contra-indications and a glucose-6-phosphate-dehydrogenase
deficiency, we initiated, immediately after withdrawal of
prednisolone, systemic therapy with hydroxychloroquine
(Quensyl®) twice daily 200 mg based on the patient’s
ideal body weight. Treatment with topical glucocorticoids and the
established antihypertensive therapy were continued. Under this
regimen, there was not total but very extensive abatement within 6
weeks. The patient has since died of an underlying cardiac
insufficiency.
Discussion
“Lupus erythematosus gyratus repens” is viewed today both
clinically and histologically as an annular variant of subacute
cutaneous lupus erythematosus [6]. However, there appears to be a
certain uniqueness [7]. Nevertheless, concerning the nomenclature
there is no general consent and there is a tendency to not
distinguish between LE gyratus repens and other cases of annular
polycyclic subtypes of SCLE. Since in lupus erythematosus gyratus
repens there is a certain clinical and histological independence,
it should be defined as a separate variant of SCLE.
To date, only a few cases of this disease entity have been
published [3-11]. In 1959, Laugier, Lénys and Bulte [5] described
for the first time the occurrence of chronic-recurrent figurate
erythema in a patient with chronic lupus erythematosus. A similar
case was published in 1975 by Hewitt et al. [4]. The pattern was
called “lupus erythematosus gyratus repens” for the first time in
that article. Other cases have been described by Laugier, [3] and
later by Maciejewski [12].
Further to these cases, Heid et al. published a case of erythema
anulare centrifugum-like lupus erythematosus in 1977 [13], yet this
disease is not identical to lupus erythematosus gyratus repens,
although there are certain clinical and histological
similarities.
Often clinically conspicuous in lupus erythematosus gyratus
repens are moderately-infiltrated, annular erythemas, sometimes
with double-contoured edges [14]. The widespread concentric bands
of erythema - waves which appear within already existing rings –
are often more strongly infiltrated and show partially
erosive-crusted lesions and a pityriasiform-like scaling. This is
not seen in other annular erythemas. Erythema anulare
centrifugum-like lupus erythematosus, by contrast, shows
collar-like scaling and no erosive-crusted lesions [7].
Histologically, in LE gyratus repens, there is usually marked
atrophy of the epidermis with only 2-3 vital epidermal layers and
often only moderate cellular infiltrate. Furthermore there is
hydropic degeneration of the basal layer and eosinophilic necrosis
of the keratinocytes at higher levels of the epidermis [3, 4, 7,
9].
The histological pattern of so-called erythema annulare
centrifugum-like lupus erythematosus [13] can nearly be identical
with that of lupus erythematosus gyratus repens [14]. However, in
erythema anulare centrifugum-type LE forms, these histological
features are usually less pronounced. Especially, there is a lack
of pronounced hydropic degeneration of the basal layer [7].
Organ involvement, as in systemic lupus erythematosus, is not
usually given in LE gyratus repens, but should always be ruled out.
Piqué et al. reported on an erythema gyratum repens-like
manifestation in systemic lupus erythematosus [15]. In that case,
however, the histological pattern was of leukocytoclastic
vasculitis with no evidence of erythema gyratum repens or
subacute-cutaneous LE [15].
Analysis of the cases published to date (table 1( Table 1 )) shows that immunoserological antinuclear
antibodies can by no means be regularly found in lupus
erythematosus gyratus repens. The association of antibodies to Ro-
and La-antigens in classical subacute cutaneous lupus erythematosus
in about 60-80% of the cases may possibly not be given in lupus
erythematosus gyratus repens. To date, the proof of positive Ro-
and La-antibodies is missing in the literature and could only be
brought in our own case thus far. However, the number of available
publications is too small to permit reliable statements on
this.
Occasionally, a medication trigger is discussed in
subacute-cutaneous lupus erythematosus, especially in connection
with frequently-used antihypertensives like calcium-channel
blockers or thiazide diuretics [16-19]. In our case this was
disregarded because there was no evidence of elevated
histon-antibodies in the patient’s serum and no coincidence of the
skin disease with the administration of the prescribed medications.
Histon-antibodies rather play a role in drug-induced systemic lupus
erythematosus and cannot necessarily be proven in a drug-induced
subacute-cutaneous lupus erythematosus [20]. The casuistics
published to date on lupus erythematosus gyratus repens (table 1)
show no associations with pharmacological substances. However,
there has been repeated discussion as to whether the antimalarials
chloroquine and hydroxychloroquine used in the therapy of lupus
erythematosus might not possibly be a trigger factor, or lead to a
transformation of chronic lupus erythematosus into lupus
erythematosus gyratus repens [4-6].
Unlike erythema gyratus repens Gammel, lupus erythematosus
gyratus repens is apparently not a paraneoplastic dermatosis,
although 2 cases with associated tumor are described in the
literature [5, 9]. Most likely, a chance coincidence can be assumed
in these patients. However, examination should always be made for
an underlying malignant process [1]. In our case, no tumor process
could be discovered.
The therapeutic measures of LE gyratus repens correspond to
those in the treatment of subacute-cutaneous lupus erythematosus.
Antimalarials can be given alone or combined with systemic
glucocorticosteroids, whereby the response to an effect of
antimalarials, as mentioned earlier, is partly controversial [3].
Monotherapy with topical glucocorticoids often appears to be
inadequately effective. Dapsone and retinoids are described as
effective. Immunosuppressives like azathioprine, methotrexate and
cyclophosphamide are indicated in manifestations transcending the
skin organ. The use of highly effective sunscreens should be
recommended.
The importance of this variant of lupus erythematosus lies, as
could be impressively demonstrated in our case, in the great
clinical similarity with Tinea superficialis which should be
excluded by a native preparation and fungus culture tests.
But other diseases must also be ruled out by differential
diagnosis (table 2( Table 2 )). In
particular, the presence of erythema gyratum repens (Gammel) must
be histologically excluded, since this latter disease is typically
associated with malignant tumors [21-23]. Erythema anulare
centrifugum Darier or erythema anulare centrifugum-like Psoriasis
vulgaris, even psoriasis pustulosa or erythema gyratum repens-like
psoriasis may be clinically very similar [24-27].
By recording subtle anamnesis and development process, and by
means of histological, mycological and immunoserological
examinations, the diagnosis can be ascertained in most cases. Proof
of a special antibody profile is not reliable in lupus
erythematosus gyratus repens and thus little-suited as a single
measure for diagnosis confirmation. Decisive to the diagnostic
definition are the clinical and histological characteristics. The
extent to which triggering by medications may be possible remains
unclear.
Table 1 Lupus erythematosus gyratus repens: Case
reports in the literature
|
No.
|
Year
|
Author
|
Age
|
Gender
|
Immunoserology
|
Drug association
|
Neoplasia
|
|
|
|
|
|
ANA
|
ENA
|
|
|
|
1
|
1959
|
Laugier et al. [4]
|
47
|
m
|
nm*
|
nm*
|
Chloroquine?
|
Ca of the Sinus piriformis
|
|
2
|
1975
|
Hewitt et al. [3]
|
74
|
m
|
neg.
|
no
|
no
|
no
|
|
3
|
1980
|
Marghescu et al. [6]
|
41
|
f
|
1:10
|
nm*
|
no
|
no
|
|
4
|
1980
|
Went [10]
|
48
|
m
|
neg.
|
no
|
no
|
no
|
|
5
|
1982
|
Blanc et al. [8]
|
66
|
m
|
neg.
|
no
|
no
|
Lung-Ca
|
|
6
|
1983
|
Polla et al. [9]
|
72
|
m
|
neg.
|
no
|
no
|
no
|
|
7
|
1985
|
Puiatti [7]
|
71
|
m
|
pos.
|
nm*
|
no
|
no
|
|
8
|
2001
|
Hochedez et al. [5]
|
86
|
f
|
1:320
|
no
|
Hydroxychloroquine ?
|
no
|
|
|
|
|
|
Anti-DNA pos.
|
|
|
|
|
9
|
2006
|
Kreft et Marsch
|
74
|
m
|
1:320
|
pos.
|
no
|
no
|
Table 2 Differential diagnosis of annular erythema
|
Annular Erythema without scaling
|
Infectious erythema
|
|
Urticaria
|
|
Erythema migrans
|
|
Granuloma anulare
|
|
Circinate sarcoidosis
|
|
Necrobiosis lipoidica
|
|
ARRAY(0x212990)
|
|
Annular Erythema with scaling
|
Figurate eczematides
|
|
Tinea superficialis
|
|
Erythema gyratum repens
|
|
Lupus erythematosus gyratus repens
|
|
Pityriasis rosea
|
|
Erythema anulare centrifugum
|
|
Erythema anulare centrifugum-like Psoriasis vulgaris
|
|
Sweet syndrome
|
Acknowledgements
Financial support: None. Conflict of interest: None.
References
1 Wollina U, Hein G. Lupus erythematosus: uncommon
presentations. Clin Dermatol 2005; 23: 470-9.
2 Sontheimer RD, Thomas JR, Gilliam JN. Subacute
cutaneous lupus erythematosus: a cutaneous marker for a distinct
lupus erythematosus subset. Arch Dermatol 1979; 115: 1409-15.
3 Laugier P. Lupus erythémateux gyratus repens. Ann
Dermatol Venereol 1977; 104: 464-6.
4 Hewitt J, Benveniste M, Kaufmann P,
Lessana-Leibowitch M. Les éruptions figurées centrifuges au
cours du lupus érythémateux. A propos d’un cas de lupus
erythematosus gyratus repens. Ann Dermatol Syphiligr (Paris) 1975;
102: 481-9.
5 Laugier P, Lénys R, Bulte C. Erythème figuré
chronique récidivant pigmentogène chez un sujet atteint de L. E.
chronique traité par la Nivaquine. Bull Soc Franc Derm Syph 1959;
66: 813-5.
6 Hochedez P, Vasseur E, Staroz F,
Morelon S, Roudier Pujol C, Saiag P. Lupus subaigu
gyratus repens. Ann Dermatol Venereol 2001; 128: 244-6.
7 Marghescu S, Rudolph PO, Linker P. Lupus
erythematodes gyratus repens. Hautarzt 1980; 31: 270-2.
8 Puiatti P. Lupus erythematosus gyratus repens.
Descrizione di un caso. G Ital Dermatol Venereol 1985; 120:
67-70.
9 Blanc D, Kienzler JL. Lupus erythematosus gyratus
repens. Report of a case associated with lung carcinoma. Clin Exp
Dermatol 1982; 7: 129-34.
10 Polla L, Laugier P. Lupus erythémateux gyratus
repens. Dermatologica 1983; 167: 172-5.
11 Went M. Lupus erythematosus gyratus repens. In: Szekeres
R. Kontaktdermatitis-Symposium der Ungarischen Dermatologischen
Gesellschaft vom 26. bis 28. Juni 1980 in Szeged. Z Hautkr 1981;
56: 688-9.
12 Maciejewski W. Annular Erythema as an Unusual
Manifestation of Chronic Disseminated Lupus Erythematosus. Arch
Dermatol 1980; 116: 450-3.
13 Heid E, Meyer E, Grosshans E, Reuter G.
Lupus erythémateux à type d’ erythème annulaire centrifuge. Ann
Dermatol Venereol 1977; 104: 397-9.
14 Marghescu S, Lubach D. Figurierte Erytheme. In:
Braun-Falco O, Wolff HH, eds. Fortschritte der
praktischen Dermatologie und Venerologie. 9th edition. Heidelberg
New York: Springer Verlag Berlin, 1979: S. 17-S. 20.
15 Piqué E, Palacios S. Santana Zaraida.
Leukocytoclastic vasculitis presenting as an erythema gyratum
repens-like eruption on a patient with systemic lupus
erythematosus. J Am Acad Dermatol 2002; 47: S254-S256.
16 Shapiro M, Sosis AC, Junkins-Hopkins JM,
Werth VP. Lupus erythematosus induced by medications,
ultraviolet radiation, and other exogenous agents: A review, with
special focus on the development of subacute cutaneous lupus
erythematosus in a genetically predisposed individual. Int J
Dermatol 2004; 43: 87-94.
17 Callen JP. Drug-induced cutaneous lupus erythematosus, a
distinct syndrome that is frequently unrecognized. J Am Acad
Dermatol 2001; 45: 315-6.
18 Bonsmann G, Schiller M, Luger T,
Ständer S. Terbinafine-induced subacute cutaneous lupus
erythematosus. J Am Acad Dermatol 2001; 44: 925-31.
19 Matthes T, Hagedorn M. Terbinafin-induzierter
subakut-kutaner Lupus erythematodes. Hautarzt 2004; 55: 653-7.
20 Fernandez-Diaz ML, Herranz P,
Suarez-Marrero MC, Borbujo J, Manzano R,
Casado M. Subacute cutaneous lupus erythematosus associated
with cilazapril. Lancet 1995; 345: 398.
21 Wolff H. Kutane Paraneoplasien. Hautarzt 2001; 52:
159-72.
22 Eubanks LE, McBurney E, Reed R. Erythema
gyratum repens. Am J Med Sci 2001; 321: 302-5.
23 Bakos N, Krasznai G, Bégány A. Erythema
gyratum repens an immunological paraneoplastic dermatosis. Pathol
Oncol Res 1997; 3: 59-61.
24 Gudi VS, Armour A, Burden AD. Erythema anulare
centrifugum associated with disseminated prostatic carcinoma. Acta
Derm Venereol 2002; 82: 306-7.
25 Vocks E, Worret WI, Ring J. Erythema annulare
centrifugum-type psoriasis: a particular variant of acute-eruptive
psoriasis. J Eur Acad Dermatol Venereol 2003; 17: 446-8.
26 Degos R, Civatte J, Arrouy M. Psoriasis et
psoriasis pustuleux à type d’erythème annulaire centrifuge. Bull
Soc Fr Dermatol Syphiligr 1966; 73: 356-8.
27 Jablonska S, Blaszczyk M, Kozlowska A.
Erythema gyratum repens-like psoriasis. Int J Dermatol 2000; 39:
695-7.
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