A 39-year-old black woman presented to the clinic with 2 months of swelling of her upper lip and cheeks with new dark spots on her face (Figure 180-1). An antinuclear antibody (ANA) was positive at a 1:80 dilution. A homogeneous nuclear pattern was present as commonly seen in systemic lupus erythematosus (SLE) and drug-induced lupus. The punch biopsy of a facial lesion was consistent with chronic cutaneous lupus erythematosus (discoid lupus). The remainder of her laboratory tests were normal. The patient's facial lesions did not respond to topical steroids and hence she was started on a short course of systemic steroids. The improvement was seen 3 weeks later (Figure 180-2). Hyperpigmentation remained but erythema, swelling, and pruritus were gone. The patient did not meet criteria for SLE and it is possible to have discoid lupus with a positive ANA. Treatment with hydroxychloroquine was discussed.
Erythema, swelling, and hyperpigmentation on the cheeks and lips of a 39-year-old black woman as the initial presentation of chronic cutaneous lupus erythematosus. (Courtesy of Richard P. Usatine, MD.)
Hyperpigmented malar rash 3 weeks later; the patient was treated with oral and topical steroids. The erythema and swelling are now gone and patient is feeling better. (Courtesy of Richard P. Usatine, MD.)
SLE is a chronic inflammatory disease that can affect many organs of the body including the skin, joints, kidneys, lungs, nervous system, and mucous membranes. Cutaneous lupus can occur in one of three forms: chronic cutaneous (discoid) lupus erythematosus, subacute cutaneous lupus erythematosus, and acute cutaneous lupus erythematosus.
- Chronic cutaneous lupus erythematosus = discoid lupus = DLE.
- Lupus profundus = lupus panniculitis.
- In the United States, the prevalence of SLE plus incomplete SLE (disease only partially meeting diagnostic requirements for SLE) is 40 to 50 cases per 100,000 persons.1 It is more common in women and patients with African ancestry.1 Worldwide, the highest SLE prevalences have been reported in Italy, Spain, Martinique, and the United Kingdom Afro-Caribbean population, but it is rarely reported among blacks who live in Africa.2
- Discoid lupus erythematosus (DLE) develops in up to 25% of patients with SLE, but may also occur in the absence of any other clinical feature of SLE.3 Patients with only DLE have a 5% to 10% risk of eventually developing SLE, which tends to follow a mild course.4 DLE lesions usually slowly expand with active inflammation at the periphery, and then to heal, leaving depressed central scars, atrophy, telangiectasias, and hypopigmentation.5 The female-to-male ratio of DLE is 2:1.
- One proposed mechanism for the etiology of SLE involves the development of autoantibodies that result from a defect in apoptosis. It has been determined that the specific defect ...