Subacute Cutaneous Lupus Erythematosus (SCLE)
- Author: Jeffrey P Callen, MD; Chief Editor: William D James, MD more...
Background
Subacute cutaneous lupus erythematosus (SCLE) is a nonscarring, non–atrophy-producing, photosensitive dermatosis. SCLE may occur in patients with systemic lupus erythematosus (SLE), Sjögren syndrome, or deficiency of the second component of complement (C2d), or it may be drug induced. Some patients also have the lesions of discoid lupus erythematosus (DLE), and some may develop small vessel vasculitis. (See the image below.) (See Etiology.)
Early lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption. Patients with SCLE frequently fulfill 4 or more of the criteria used to classify SLE. Serologic abnormalities are common. Therapy with sunscreens, topical corticosteroids, and antimalarial agents is usually effective. (See Workup and Treatment.)
SCLE lesions heal without scarring or atrophy, but they may result in dyspigmentation. Severe systemic disease is unusual, but when it occurs, the patient may develop life-altering sequelae. (See Prognosis.)
Etiology
Subacute cutaneous lupus erythematosus (SCLE) occurs in genetically predisposed individuals, most often in patients with human leukocyte antigen B8 (HLA-B8), human leukocyte antigen DR3 (HLA-DR3), human leukocyte antigen DRw52 (HLA-DRw52), and human leukocyte antigen DQ1 (HLA-DQ1). A strong association exists with anti-Ro (SS-A) autoantibodies. The reaction is believed to be related to ultraviolet (UV) light modulation of autoantigens, epidermal cytokines, and adhesion molecules, with resultant keratinocyte apoptosis.
The disease usually manifests following UV light exposure, but other triggers or inciting factors also must be operative as not all patients have disease that follows photoexposure. Exacerbation of disease or induction of lesions is more common following UV-B exposure, but it might also occur in patients exposed to UV-A as well. Some patients exhibit sensitivity to only UV-A or to UV-A and UV-B.[1]
In perhaps as many as 20% of patients with SCLE, drugs may exacerbate or induce their disease; the most frequently implicated drug is hydrochlorothiazide, but other antihypertensive agents along with a list of over 100 different agents has been reported in relation to the awakening or exacerbation in individual patients.[2]
Many pharmaceutical companies combine an antihypertensive agent with hydrochlorothiazide, and this should be assessed carefully. Implicated agents also include calcium channel blockers, angiotensin-converting enzyme (ACE) inhibitors, terbinafine, and tumor necrosis factor antagonists.[3, 4, 5, 6] Therefore, a careful drug history should be part of the initial evaluation of patients with SCLE.[7]
Patients with a deficiency of the second component of complement (C2d) often manifest SCLE lesions as part of the SLE-like disease.
Epidemiology
Worldwide, SLE prevalence ranges from 17-48 cases per 100,000 persons. The highest prevalence of SLE occurs in patients aged 40-60 years. The male-to-female ratio of SLE is approximately 1:10. The male-to-female ratio of cutaneous lupus erythematosus (CLE) is approximately 1:2-3.[8] Of patients with CLE, 10-50% have SCLE.
SCLE is more common in whites (>85%). The male-to-female ratio of SCLE is 1:4. The condition typically occurs in patients aged 15-70 years, with the mean age being approximately 43 years.
Prognosis
Subacute cutaneous lupus erythematosus (SCLE) uncomplicated by severe SLE has a good prognosis. Some patients may manifest spontaneous remission; however, most have chronically active disease or a course punctuated by intermittent exacerbations. Exacerbation in the spring or summer is not uncommon.
Approximately one half of patients with SCLE have 4 or more of the criteria for classification as SLE, but in these patients, the disease is generally less severe, although in individual patients the full range of severity and end organ dysfunction is possible. By definition, skin lesions heal without scarring or atrophy but may leave residual dyspigmentation.
Patient Education
Instruct patients about sun-avoidance techniques, the use of sun-protective clothing, and the appropriate use of sunscreens. Discuss the expectations for prognosis. Make patients aware of the symptoms of SLE.
Because SCLE is exacerbated by sunlight or other UV light exposure, advise patients to take precautions. One precaution is to discourage exposure to sunlight between the hours of 10 am and 4 pm. While this is beneficial to some patients, many are so exquisitely photosensitive that this alteration does not help. In addition, advise patients to avoid artificial light sources, such as tanning beds.
For patient education information, see the Arthritis Center, as well as Lupus (Systemic Lupus Erythematosus).
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