Dermatology Online Journal

Persistent periorbital edema as a sole manifestation of cutaneous lupus erythematosus: Report of two cases
H Ghaninejad¹, S Kavusi¹, F Safar ¹, M Asgari², and ZS Naraghi²
Dermatology Online Journal 12 (2): 14

Department of Dermatology¹ and Department of Pathology², Razi Hospital, Tehran University of Medical Science, Tehran, Iran. sukavusi @yahoo.com

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Abstract

In lupus erythematosus, dramatic periorbital edema and erythema without any evidence of other significant cutaneous or systemic involvement is unusual. We describe two patients with severe periorbital edema and erythema as the sole manifestation of cutaneous lupus erythematosus.

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Clinical synopses

Case 1

A 23-year-old white man had a 1-year history of asymptomatic, bilateral swelling of his eyelids and cheeks; the swelling extended over his nasal bridge and forehead. Initially the swelling occurred intermittently, but after 1 year it was permanent. His past medical and family history was unremarkable.

Physical examination showed prominent swelling on a background of mild erythema involving the eyelids, cheeks, forehead, and nose. There were no associated symptoms, including fever, pain, pruritus, or stinging. He was otherwise well and had no evidence of systemic involvement. Laboratory investigations, including urinalysis, complete blood count, sedimentation rate, liver function tests, and renal function tests were within normal limits. Antinuclear antibody and anti-Ro (SSA) were negative. Chest X-ray and skull Roentgenogram were normal. The biopsy specimen showed compact hyperkeratosis, diffuse but marked basal cell hydropic degeneration, and superficial and deep perivascular, as well as periadnexal lymphocytic infiltrate (Fig. 2c). Direct immunofluorescence of the lesion revealed strong band-like granular deposition of C3 and IgG along the basement membrane zone. Direct immunofluorescence on normal skin of buttock was negative.

Chloroquine was started at a dose of 150 mg/bid. After 2 months, no improvement was achieved; prednisolone 60 mg/day was added for 1 month and tapered over the next 4 weeks. After 2 more months the patient showed mild improvement.

He was seen again 1 year later for acne vulgaris. His facial swelling had recurred. The new biopsy specimen showed normal epidermis with dermal fibrosis and presence of interstitial mucin. Direct immunofluorescence showed a band like granular deposit of IgG and C3 along the dermoepidermal junction. He was treated with chloroquine 150 mg/bid for another 6 months. Despite reducing the amount of mucin in dermis, his facial swelling did not improve.

Case 2

A 36-year-old man had a 2-year history of swelling, erythema and edema on the left side of his face. For the previous 6 months, he had developed also a severe left eye proptosis with prominent periorbital swelling.

Physical examination showed marked facial asymmetry with pronounced nonpitting swelling of his left upper eyelid and severe proptosis of the left eye. Malar erythema was present (Fig. 1). His facial erythema was aggravated by sun exposure. There was a family history of thyroid disease, but he had none of the symptoms of thyrotoxicosis. His medical history was otherwise unremarkable. A computed tomographic scan of the orbits revealed left-side proptosis and enlargement of external ocular muscles in left orbit with retrobulbar fat infiltration. Routine lab tests (CBCdiff, ESR, RFT, and LFT) and his serum creatine kinase were within the normal range. He had negative antinuclear antibodies on two different occasions, but a strongly positive anti-ds DNA antibodies (radioimmunoassay, 95 IU m-¹; normal < 35). Anti-Ro (SSA) was negative.

Figure 1a	Figure 1b
Figure 1 (a): Malar erythema, swelling of the left upper eyelid, and left eye proptosis. (b) (Oblique view)

Because of left eye proptosis, a thyroid study was obtained, which revealed normal thyroid function tests (TSH= 2.3 µIU/ml and T₄ = 10.6 µg/dl), a negative thyroglobulin antibody (= 15; normal < 60) and a markedly elevated thyroid peroxidase (TPO= 255 IU; normal < 30).

These values, CT scan findings and proptosis were compatible with a diagnosis of autoimmune thyroiditis with ophthalmopathy.

Figure 2a	Figure 2b
Figure 2 (a) Epidermal atrophy, focal liquefaction degeneration of the basal layer with Civatte bodies, pigment incontinence, and superficial perivascular lymphocyte infiltration (hematoxylin & eosin × 100). (b) Eyelid skin biopsy specimen shows strongly positive staining with Alcian blue, indicating interstitial mucin deposition (Alcian blue × 100).

Figure 2c
Marked basal cell hydropic degeneration, dermal edema, and dense perivascular and perifollicular lymphocytic infiltrate (hematoxylin & eosin × 100).

A skin biopsy specimen from the left upper eyelid revealed epidermal atrophy and focal vacuolization of the basal layer with occasional Civatte bodies. The dermis showed pigment incontinence and superficial perivascular lymphocyte infiltration with a few plasma cells (Fig. 2a). Strong staining with alcian blue indicated a marked dermal mucin deposition (Fig. 2b). Direct immunofluorescence showed diffuse granular deposition of IgG, IgM, C3 and weak deposition of IgA along the dermoepidermal junction with deposition of immunoreactions on cytoid bodies. The patient was treated with chloroquine (150mg/bid) and sun protection. After 3 months, he showed marked improvement in his facial erythema and edema with marked regression of his unilateral proptosis.

Comment

Systemic lupus erythematosus (SLE)-related eyelid edema was first described by Tuffanelli and Dubios [1]. They studied 520 patients over a 13-year period. They reported an overall 4.8 percent in incidence of periorbital edema in SLE. It has been reported also in cases of discoid lupus erythematosus and lupus erythematosus profundus [2, 3]. The presence of increased dermal mucin can result in periorbital edema in lupus erythematosus (LE) [4]. In our cases, the disease was limited to the skin but the lesions lacked the appearance of discoid lupus erythematosus, including atrophy, scarring, and follicular plugging.

Cyran and et al. reported two similar cases and used the term chronic cutaneous lupus erythematosus to refer to the particular subset of LE into which their patients fall [5].

Our first patient is unusual in that periorbital edema was associated marked fibrosis along with mucin deposition in dermis. The presence of dermal mucin, dermal edema and loose areolar connective tissue in the periorbital area probably account for the swelling in our patients. Although it is well known that dermal mucin is often found in the skin lesions of SLE and DLE, it rarely produces clinically apparent features.

Association of autoimmune thyroiditis with LE was demonstrated in second patient. LE coexists with other diseases of presumed autoimmune nature, including autoimmune thyroiditis [6]. A high prevalence of antithyroid antibodies has been found in patients with SLE [7, 8]. Miller et al. reported more than 45 percent of these patients had either subclinical or biochemical primary hypothyroidism, which is much higher than the prevalence in the normal population [9]. Although periorbital edema frequently occurs in autoimmune thyroid disease, the characteristic histopathologic and immunofluorescence findings in our patient were consistent with LE as the cause of his clinical findings.

Our report suggests that every patient with persistent periorbital edema should undergo histologic and immunofluorescent evaluation, and LE should be included in the differential diagnosis.

Acknowledgments: The authors declare that they have no competing interests. Written consent was obtained from the patient or their relative for publication of study.

References

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4. Williams WL, Ramos-Caro FA. Acute periorbital mucinosis in discoid lupus erythematosus. J Am Acad Dermatol. 1999;41:871-3.

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6. Mihailova D, Grigorova R, Vassileva B, Mladenova G, Ivanova N, Stephanov S, Lissitchky K, Dimova E. Autoimmune thyroid disorders in juvenile chronic arthritis and systemic lupus erythematosus. Adv Exp Med Biol 1999; 455: 55-60.

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8. Weetman AP, Walport MJ. The association of autoimmune thyroiditis with systemic lupus erythematosus. Br J Rheumatol 1987; 26: 359-61.

9. Miller FW, Moore GF, Weintraub BD, Steinberg AD. Prevalence of thyroid disease and abnormal thyroid function test results in patients with systemic lupus erythematosus. Arthritis Rheum 1987; 30: 1124-31.

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