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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.3 Rio de Janeiro May/June 2004
Subacute cutaneous lupus erythematosus (SCLE) manifested clinically as annular erythema centrifugum*
Ney RomitiI; Sandra Lopes Mattos e DinatoII; José Roberto Paes de AlmeidaIII; Angela LapoliIV; Angelo SementilliV
IProfessor/Lecturer in Dermatology.
Head of Dermatology, UNILUS
IIPh.D., Professor of Dermatology, FMUSP. Head of the Internal Medicine Department, UNILUS
IIIProfessor, Specialist in Dermatology. Working toward a Master's Degree, UNILUS
IVSecond-year dermatology resident, HGA/UNILUS
VProfessor. Master's Degree in Pathological Anatomy, FMUSP. Professor, Pathological Anatomy Department, UNILUS
The authors describe a case of subacute cutaneous lupus erythematosus (SCLE) manifested clinically as a case of annular erythema centrifugum. The clinical and laboratorial differentiation of SCLE from systemic lupus erythematosus is emphasized, and the best therapeutic conduct is indicated.
Keywords: lupus erythematosus, cutaneous.
Subacute cutaneous lupus erythematosus (SCLE) was characterized in 1979 by Sontheimer et al. as an isolated anatomoclinical entity.1
In most cases the cutaneous condition is diagnosed according to two patterns: a psoriasiform or papulous, squamous subset or an annular subset with maculopapulous lesions. It predominates on light-exposed areas, such as the shoulders, anterior side of the thorax, arms, forearms and dorsal aspect of the hands, which explains its marked photosensitivity varying between 60 to 80% of cases.2 The lesions regress, leaving hyperpigmentation or, more frequently, pseudo-vitiligo hypopigmentation with telangiectasias, although without atrophy.3 In 70% of cases, it affects female, young adult and Caucasian patients.4
The objective of this study is to report a case of SCLE which appeared as centrifugal annular erythema.
An 18 year-old Caucasian female, born and residing in Sao Sebastiao, Sao Paulo State..
She reported annular erythematous lesions as appearing two years earlier with desquamative inner borders. The lesions grew centrifugally, were localized initially on the thorax, and progressed toward the face, upper limbs and (front and back) trunk. The patient reported worsening of the condition after sun exposure, and improvement after using injectable corticoids.
Nine months ago she presented with diarrheic episodes (liquid, with mucus, three to four episodes daily), interspersed with intestinal constipation. She denied having other systemic symptoms or taking other medications.
The examination by HE of the skin fragment obtained from the upper right limb revealed hydropic degeneration of the basal layer cells of the epidermis. The dermis showed edema, with dilated vessels and diffuse lymphocytic inflammatory infiltration, and was predominantly perivascular (Figures 2 and 3).
Staining by PAS showed focal thickening of the basal layer.
Right immunofluorescence was negative.
- Normal tests: hemogram with platelet count, fasting glycemia, kidney and hepatic function tests, total and fractional proteins, reactive C protein, mucoprotein, rheumatoid factor, VDRL, Anti-Sm, total and partial complement, urine I and feces protoparasitology.
- Pelvic and abdominal USG: without alterations
- Colonoscopy: enanthema, whose biopsy showed non-specific histopathologic alterations
- VHS: 48 (normal value up to 20)
- FAN: 1/1280 homogenic pattern.
- Anti-SSA/Ro: 11.6 (normal value up to 8)
- Anti-SSB/La: 154 (normal value up to 8)
After confirming the diagnosis, pulsotherapy was initiated with methylprednisolone (1 mg per kg of weight), which resulted in rapid improvement of the cutaneous lesions and of the intestinal symptoms. The patient is currently taking chlorochine diphosphate, 250 mg daily.
SCLE has been unusually described5 as having similar manifestations to polymorphic erythema (Rowell's syndrome). The papulous, desquamative subsets may be confused with psoriasis, and the annular subsets with annular erythema centrifugum.5 The present case demonstrates the importance of the diagnostic differential of this infirmity. The annular lesions show a variable incidence of 17 to 83% of cases in the different literature data,6,7 while papulous, desquamative lesions vary from 15 to 50% of cases.6,7
SCLE is characterized by encountering the anti-Ro (SS-A) marker in 63% of patients,1 mainly in the annular subsets,8,9 This has been well documented in association with HLA-DR2 and HLA-DR3.10 The antinuclear antibodies (FAN) are positive in 70 to 90% of cases. The antiLa (SS-B) antibody, when present, coexists in most cases with the anti-Ro (SS-A), though it is rare to find it in isolation.11,12
The anti-Ro (SS-A) antibody is detected in all drug-induced cases of SCLE.13 On the other hand, most of the drugs inducing SCLE also determine other photosensitivity reactions. These observations suggest a hypothesis that these medications in association with ultraviolet radiation and the presence of anti-Ro/SS-A antibody, lead to inducing SCLE cutaneous lesions.13 It is interesting to note that the antihistonal antibody, i.e. the serological marker of drug-induced systemic lupus erythematosus (SLE), is not found to be increased in these patients. This suggests that different pathological mechanisms are at work.13,14 Nonetheless physicians must also query into the patient's medicinal background. In the present case, it was negative.
Regarding histopathology and according to some authors, in 80% of cases it is possible to differentiate SCLE from chronic cutaneous lupus erythematosus (CCEL). The main alterations characteristic of the latter are hyperkeratosis and density of the dermal inflammatory infiltrate. For others, this test shows poor results.15,16
In turn, right immunofluorescence in SCLE shows immunoglobulin deposits and/or complements at the dermoepidermal junction in 60% of lesioned skin cases.2 Positive results were found in only 29 %17 of the annular subtype.
The patient in the present study showed compatible clinical and laboratory conditions with the diagnosis of SCLE. Negative immunofluorescence does not exclude SCLE, mainly when dealing with the annular subtype. Nor do these results have a relation with the prognosis.2
It should be emphasized that in SCLE 58% of patients fulfill four or more of the 11 criteria established by the American Rheumatological Association for the diagnosis of SCLE (cf. Table 1).5 As such, the differentiation between these two subsets of lupus erythematosus becomes difficult.4,5,18
Moderate systemic involvement, above all articular and muscular involvement, the need for less aggressive therapy19 and infrequent finding of anti-DNA, anti-Sm and antiU1-RNP in cases of SCLE (less that 20% of all cases) collaborate in its diagnosis.20 In general, kidney and neurological manifestations are moderate when present (20 to 30% of cases).2 Nevertheless, severe complications have already been reported, with diffuse proliferative glomerulonephritis (DPGN) and death due to central nervous system involvement.12
In Richer-Cohen and Crosby's report of 14 cases, 11 showed severe manifestations, but the majority of these patients showed the papulous and squamous form, which is related to a worse prognosis.21 A study comparing SCLE patients with SLE (with and without cutaneous lesions) showed a better prognosis for the latter. These patients also showed a lower incidence of nephropathy, serositis and involvement of the central nervous system.19 Tebbe et al. cite FAN titers above 1/320, the presence of arthralgias and signs of (proteinuria, hematuria and hemoglobinuria) nephropathy as factors in the misprognosis of SCLE.22
It is important to point out that 20% of SCLE patient cases may present association with CCLE lesions12 and 10% in association with SLE.19
In relation to therapy, in most studies the frontline drug is chlorochine, which has a satisfactory response in four to eight weeks in 90% of cases.5 Sometimes, monotherapy with chlorochine is not enough to control the disease. It is necessary to associated it with thalidomide.23 Other options, were there problems with the anti-malarial drugs, are dapsone and corticoids.5
The patient referred to in the present paper showed indications of severe systemic involvement. But due to high FAN titers pulsotherapy was opted for with methylprednisolone. Rapid improvement was obtained for the cutaneous and intestinal lesions. The patient is currently taking chlorochine, with no active lesions present.
In the end, it may be concluded that the obligatory inclusion of SCLE in the diagnostic differential of centrifugal annular erythematous lesions may work as a particularly important warning sign for young dermatologists.
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Correspondence to Received in December, 10th of 2001.
done at "Faculdade de Ciências Médicas de Santos - Centro Universitário
Lusíada - UNILUS - Hospital Guilherme Álvaro".
Sandra Lopes Mattos e Dinato
Rua Bento de Abreu, 65 - Boqueirão
11045-140 Santos SP
Tel.: (13) 3233-2964
Approved by the Consultive Council and accepted for publication in July, 11th of 2003.
Received in December, 10th of 2001.
* Work done at "Faculdade de Ciências Médicas de Santos - Centro Universitário Lusíada - UNILUS - Hospital Guilherme Álvaro".