Figurate Erythema, Photosensitivity, and Conjunctival Irritation of Recent Onset Luigi Naldi, MD; Marzia Bronzoni, MD; Luca Cavalieri d'Oro, MD; Francesco Locati, MD; Lorenzo Marchesi, MD; Tullio Cainelli, Department of Dermatology, Ospedali Riuniti di Bergamo (Italy) REPORT OF A CASE

64-year-old department gust 1988 because of a photosensitive figurate erythema A

woman was

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the upper aspect of her trunk of 2 months' duration. The year before, the patient had reported a similar eruption lasting a few weeks and disappearing after a short course of topical corticosteroid therapy. The patient had been hypertensive since the age of 45 years and had been taking indapamide and penbutolol for 2 years. Her medical history was otherwise negative. On physical examination, the lesions were found to be small, confluent, edematous erythematous papules and annular rings with an area of central grayish hypopigmentation. Slight surface scale and telangiectasias were also observed (Figs 1 and 2). Lesions involved the neck, the shoulders, the upper aspect of the back, the extensor aspect of the arms, and the dorsal aspect of the hands. They were painless and nonpruritic. On the face, the back of the neck, and the "V" of the

1.

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Figure

2.

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chest, a deep erythema was also evident, sparing character¬ istically shielded areas such as the submental region and the folds of the upper eyelids. A diffuse, nonscarring alopecia was

another feature. Besides these cutaneous lesions, edema of the eyelids and conjunctival erythema with a gritty sensation in the eyes were noted. The Schirmer test demonstrated a reduction of lacrimal se¬ cretion. Histologie examination of a minor salivary gland showed no abnormality. Other laboratory tests, including a complete blood cell count, erythrocyte sedimentation rate, search for antinuclear antibodies (rodent tissue substrate), anti-double-stranded DNA antibodies, lupus erythematosus (LE) cells and rheumatoid factor, complement levels, immunoelectrophoresis, coagulation screening, and chest roentgenogram showed normal or negative findings. A skin biopsy specimen from an annular lesion is shown in Fig 3. What is your diagnosis?

Figure 3.

DIAGNOSIS: Subacute cutaneous lupus (SCLE) and keratoconjunctivitis sicca.

erythematosus

CLINICAL COURSE

Histologie examination revealed abnormalities similar to the changes observed in discoid lupus erythematosus (DLE). In particular, hydropic degeneration of basal keratinocytes was prominent, with edema in the papillary dermis and a superfi¬ cial perivascular infiltrate of mononuclear cells; follicular plugging was not observed. Direct immunofluorescence showed negative findings both on lesional and normalappearing skin. A search for anti-Sjogren's syndrome A or Ro antigen (anti-SSA/Ro) antibodies by enzyme-linked immunosorbent assay showed positive findings. The patient's phenotype was HLA-DR3. The patient was given hydroxychloroquine (400 mg/d) and was advised to avoid solar radiation. Therapy with the ß-blocker penbutolol was withdrawn as a caution; a calcium channel blocker was substituted. The cuta¬ neous lesions healed rapidly, leaving grayish hypopigmented areas; the lacrimal secretion was persistently low and artifi¬ cial tears were given. At the last follow-up 1 year later, only her ocular symptoms were present. DISCUSSION The term SCLE refers to widespread, photosensitive lesions with many features of DLE, but without scarring or atrophy, mostly affecting young or middle-aged white women. Two clinical patterns are described: annular and papulosquamous.1-2 The early lesion is a scaly erythematous and telangiectatic papule that enlarges and becomes confluent, as¬ suming a psoriasiform appearance with a reticulate configuration, or merges into annular lesions, sometimes with an irislike appearance. Usually, the patient has only one type of lesion, but a small percentage of cases shows both annular and papulosquamous lesions. Cutaneous lesions have a char¬ acteristic distribution on the arms and the upper aspect of the trunk; the face and scalp are frequently spared and the disease rarely occurs below the waist. Pigmentary changes may be prominent, with depigmentation lasting months after the acute phase and a small percentage of patients developing

seemingly permanent vitiligolike lesions. Photosensitivity is frequently observed, and most patients experience worsening following UV exposure. A diffuse nonscarring alopecia is an¬

other feature. Histologie examination reveals a pattern similar to DLE; however, follicular changes, basement membrane thickening, and periappendageal infiltrates are generally not observed.3,4 In contrast with DLE, direct immunofluorescence of lesional skin is negative in 50% of all patients, and immune deposits at the dermoepidermal junction of clinically normal skin are found in approximately 25% of patients.3 An SCLE has been reported with a full spectrum of LE-associated cutaneous manifestations, including scarring lesions of DLE, malar rash, vasculitic lesions, periungual telangiectatic changes, livedo reticularis, and mucosal lesions.12,5 Clinical conditions associ¬ ated with SCLE include systemic lupus erythematosus (SLE), Sjögren's and sicca syndromes, idiopathic thrombocytopenic purpura, urticarial vasculitis, and C2 deficiency. The concept that patients with SCLE as their major feature represented a distinct LE subset was introduced by Sontheimer and coworkers1 in 1979. Patients were reported to be clinically homogeneous, frequently having a mild systemic disease marked by musculoskeletal complaints and fullfilling in 50% of cases the American Rheumatism Association's (At¬ lanta, Ga) criteria for diagnosis of SLE. In these patients, SLE seemed to have a rather benign course and was rarely com¬ plicated by renal or central nervous system manifestations. A strong association with circulating anti-SSA/Ro and antiSjögren's syndrome B or La antigen (anti-SSB/La) antibodies and the HLA-DR3 phenotype were other features reported. The concept of SCLE has persisted, even though the spectrum of the associated clinical manifestations has widened to include severe renal and neurologic manifestations, and the frequency of anti-SSA/Ro and anti-SSB/La antibodies and HLA-DR3 was reported in recent series to be lower than expected.5 The significance of antibodies in the pathogenesis of SCLE is widely debated. Recently, Furukawa et al6 demonstrated that antibodies to SSA/Ro and SSB/La bind to UV-irradiated human keratinocytes and speculated that the binding is an important inducer of antibody-dependent cytotoxicity in pho¬ tosensitive cutaneous lupus.

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The treatment of SCLE is, in general, satisfactory using an approach that includes photoprotection and administration of antimalarial drugs such as hydroxychloroquine sulfate (200 to 400 mg/d). If hydroxychloroquine sulfate is not an effective therapy, quinacrine (100 mg once or twice daily) can be added to therapy, or the patient's therapy can be switched to chloroquine. Other systemic agents occasionally employed in the treatment of patients who fail to respond to the above-

mentioned measures include low-to-intermediate doses of systemic corticosteroids associated with antimalarial drugs, dapsone (100 to 200 mg/d), thalidomide, gold salts, retinoids, and a-2 recombinant interferon. Some ß-blockers have been reported to be associated with lupuslike syndrome and keratoconjunctivitis sicca syndrome.7 We were not able to find any reference concerning penbutolol. It is not possible to exclude the possibility that the drug played a role in inducing our patient's disease, although lacrimal secretion was persistently low 1 year after penbutolol suspenReferences

1. Sontheimer RD, Thomas JR, Gillian JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115:1409-1415. 2. Sontheimer RD, Maddison PJ, Reichlin M, et al. Serologic and HLA associations in subacute cutaneous lupus erythematosus: a clinical subset of lupus erythematosus. Ann Intern Med. 1982;97:664-671. 3. Herrero C, Bielsa I, Font J, et al. Subacute cutaneous lupus erythematosus: clinicopathologic findings in 13 cases. J Am Acad Dermatol. 1988;19:1057-1062. 4. Jerdan MS, Hood AF, Moore GW, Callen JP. Histopathologic comparison of the subsets of lupus erythematosus. Arch Dermatol.

1990;126:52-55. 5. Callen JP, Klein J. Subacute cutaneous lupus erythematosus: clinical, serologic, immunogenetic, and therapeutic considerations in 72 patients. Arthritis Rheum. 1988;31:1007-1013. 6. Furukawa F, Kashihara-Sawami M, Lyons MB, Norris DA.

Binding of antibodies to the extractable nuclear antigens SS-A/Ro and SS-B/La is induced on the surface of human keratinocytes by ultraviolet light (UVL): implications for the pathogenesis of photosensitive cutaneous lupus. J Invest Dermatol. 1990;94:77-85. 7. Hess E. Drug-related lupus. N Engl J Med. 1988;318:1460-1462.

Figurate erythema, photosensitivity, and conjunctival irritation of recent onset. Subacute cutaneous lupus erythematosus (SCLE) and keratoconjunctivitis sicca.

Figurate Erythema, Photosensitivity, and Conjunctival Irritation of Recent Onset Luigi Naldi, MD; Marzia Bronzoni, MD; Luca Cavalieri d'Oro, MD; Franc...
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