Looking beyond the ordinary: Genital lupus

Authors


Introduction

Genital lupus occurs infrequently, and the amount of literature regarding this condition currently is negligible. To our knowledge, no cases of genital lupus in a patient with systemic lupus erythematosus (SLE) have been reported. Although the actual prevalence of genital lupus may be low, physician and/or patient discomfort (e.g., in terms of discussing a genital lesion) as well as time constraints may contribute to underdiagnosis of this disorder.

We hope to increase physician awareness of this unusual condition by reporting the case of a 27-year-old African American woman who had SLE manifesting as arthralgias, lupus nephritis, and cutaneous discoid lesions, especially in the vulvar region.

Case Report

The patient, a 27-year-old African American woman had a 4-year history of SLE manifesting as discoid skin lesions on the face and joint pain. The patient presented with new discoid plaques on her lower back. After further discussion, she disclosed the presence of a nonpruritic genital lesion. This lesion had been present for at least 1 year and had been progressively increasing in size. The patient had poor body image and low self-esteem due to the genital lesion.

The genital lesion was located predominantly on the left labium majora, and appeared as a depressed, hypopigmented, salmon-pink plaque (Figure 1). Loss of pubic hair at the site of the plaque was observed.

Figure 1.

Clinical appearance of discoid genital plaque in a patient with systemic lupus erythematosus.

At the time of presentation, she was receiving prednisone (40 mg/day), as prescribed by her primary care physician. She had previously been receiving hydroxychloroquine, but this treatment had been discontinued for more than a year.

The serologic tests were positive for antinuclear antibodies (1:640 titer; homogenous pattern), double-stranded DNA antibodies (1:160 titer), and anti-Smith antibodies. The complement C3 level was 64 mg/dl (normal 86–184 mg/dl). Renal biopsy specimens showed focal sclerosing and membranous glomerulonephritis. Biopsy samples of the skin lesion showed acanthosis hyperkeratosis and dermal chronic inflammatory infiltrate (perivascular and interstitial).

Discussion

Photosensitivity and malar rash are 2 of the diagnostic criteria for SLE; however, in patients with lupus, physicians may limit their search for cutaneous manifestations to sun-exposed areas. Vulvar involvement is very rare in SLE. Although vulvar lesions in discoid lupus have been previously reported (1), to our knowledge, this is the first report of discoid genital lupus in a patient with SLE.

The literature on genital lupus lesions is very limited (2). This may be partly attributable to low prevalence, physician discomfort in searching for this manifestation, time constraints, or patient discomfort. Unless specifically asked by the physician, patients seldom disclose the existence of such a lesion resulting in lack of treatment. Lack of appropriate treatment could lead to devastating effects on the patient's sexual life, body image, and overall self-esteem. Untreated patients may be especially prone to scarring and disfigurement, and such sequela are preventable by early diagnosis and treatment. Increased physician awareness of this unusual manifestation of SLE is important.

Ancillary