Subacute cutaneous lupus erythematosus (SCLE) manifested clinically as annular erythema centrifugum

Abstracts

Os autores relatam caso de lúpus eritematoso cutâneo subagudo (LECSA) manifestado clinicamente como eritema anular centrífugo. Ressaltam a diferenciação clínica e laboratorial entre LECSA e lúpus eritematoso sistêmico. Também discutem a conduta terapêutica.

lúpus eritematoso cutâneo

COMMUNICATION

Subacute cutaneous lupus erythematosus (SCLE) manifested clinically as annular erythema centrifugum^*

Ney Romiti^I; Sandra Lopes Mattos e Dinato^II; José Roberto Paes de Almeida^III; Angela Lapoli^IV; Angelo Sementilli^V

^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

Correspondence

SUMMARY

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.

INTRODUCTION

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.

CASE REPORT

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.

Histopathology

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.

Complimentary tests

- 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)

Progression

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.

DISCUSSION

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.

REFERENCES

• 1. 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.
• 2. Doutre MS, Beylot-Barry M, Beylot C. Lupus érythémateux cutanés subaigu. Presse Med 2000;29(23):1311-6.
• 3. David-Bajar KM. Subacute cutaneous lupus erythematosus. J Invest Dermatol 1993; 100:52-8.
• 4. Sontheimer RD. Subacute cutaneous lupus erythematosus. A decade's perpective. Med Clin North Am 1989;73:1073-90.
• 5. Drake LA, Dineheart SM, Farmer ER et al Guidelines of care for cutaneous lupus erythematosus. J Am Acad Dermatol 1996;34(5):830-6.
• 6. Callen JP, Klein J. Subacute cutaneous lupus erythematosus: clinical, serologic, immunogenetic, and therapeutic considerations in seventy-two patients. Arthritis Rheum 1988;31:1007-13.
• 7. Herrero C, Bielsa I, Font J, Lozano F, Ercilla G, Lecha M et al Subacute cutaneous lupus erythematosus: clinical pathologic findings in 13 cases. J Am Acad Dermatol 1988;19:1057-62.
• 8. Callen JP. Is subacute cutaneous lupus erythematosus a subset of systemic lupus erythematosus or a distinct entity? Br J Dermatol 2001;144(3):449-1.
• 9. Sontheimer RD, Maddison PJ, Reichlin M, Jordon RE, Stastny P, Gilliam JN. Serologic and HLA associations in subacute cutaneous lupus erythematosus, a clinical subset of systemic lupus erythematosus. Ann Intern Med 1982;87:664-71.
• 10. Provost TT, Watson R. Anti-Ro HLA-DR3 positive women: the interrelationship between some ANA negative , SS, SCLE and NLE mothers and SS (LE overlap female patients ) J Invest Dermatol 1993;100:14-20.
• 11. Hochberg MC, Boyd RE, Ahearn JM. Systemic lupus erythematosus: a review of clinicolaboratory features and immunogenetic markers in 150 patients with emphasis on demographic subset. Medicine 1985;64:285-95.
• 12. Chlebus E, Wolska H, Blaszczyk M, Jablonska S. Subacute cutaneous lupus erythematosus versus systemic lupus erythematosus: diagnostic criteria and therapeutic implications. J Am Acad Dermatol 1998;38(3):405-12.
• 13. MacCauliffe DP. Distinguishing subacute cutaneous from other types of lupus erythematosus Lancet 1998;351(9115):1527-8.
• 14. Crowson AN, Magro CM. Subacute cutaneous lupus erythematosus arising in the setting of calcium channel blocker therapy. Human Pathol 1997;28:67-73.
• 15. Judan MS, Hood AF, Moore GW, Callen JP. Histopathologic comparison of the subsets of lupus erythematosus. Arch Dermatol 1990;126:52-5.
• 16. David-Bajar KM, Bennion SD, Despain JD, Golitz LE, Lee LA. Clinical, histologic and imunofluorescent distinctions between subacute cutaneous lupus erythematosus and discoid lupus erythematosus. J Invest Dermatol 1992;99:251-7.
• 17. Ruzicka T, Faes J, Bergner T, Uwe R, Braun-Falco O. Annular erythema associated with Sjögren's syndrome: A variant of systemic lupus erythematosus. J Am Acad Dermatol 1991;25(3):557-60.
• 18. Drosos AA, Dimou GS, Siamopoulou-Mavidrou A, Hatzis J, Moutsopoulos HM. Subacute cutaneous lupus erythematosus in Greece: a clinical, serologic and genetic study. Ann Med Intern 1990;141:421-4.
• 19. Lopes-Longo FJ, Monteagudo I, González CM, Grau R, Carreńo L. Systemic lupus erythematosus : clinical expression and anti-Ro response in patients with and without lesions of subacute cutaneous lupus erythematosus. Lupus 1997;6(1):32-9.
• 20. Fonseca E, Alvarez R, Gonzalez MR, Pascual D. Prevalence of cardiolipin antibodies in subacute cutaneous lupus erythematosus. Lupus 1992;1:265-8.
• 21. Richer Cohen M, Crosby D. Systemic disease in subacute cutaneous lupus erythematosus: a controlled comparison woth systemic lupus erythematosus. J Rheumatol 1994;21:1665-9.
• 22. Tebbe B, Mansmann U, Wollina U et al Markers in cutaneous lupus erythematosus indicating systemic involvement. A multicenter study on 296 patients. Acta Derm Venereol 1997;77:305-8.
• 23. Versapuech J, Beylot-Barry M, Doutre MS, Beylot C. Lupus cutanés subaigu. Presse Med 2000;29(29):1596-9.

*

Trabalho realizado na Faculdade de Ciências Médicas de Santos - Centro Universitário Lusíada - UNILUS - Hospital Guilherme Álvaro.

Publication Dates

• Publication in this collection
  10 Nov 2011
• Date of issue
  May 2004

History

• Received
  10 Dec 2001
• Accepted
  11 July 2003