Frequently Asked Questions about Cutaneous Lupus

What is Cutaneous Lupus?

Cutaneous lupus erythematosus, the skin-related form of lupus, affects people with systemic lupus erythematosus (SLE), but can also occur on its own.  Cutaneous lupus erythematosus is an autoimmune disorder that affects millions of people worldwide.  Approximately 85% of people with SLE will have a cutaneous manifestation of the disease at some point during their disease (1). About 20% of systemic lupus patients originally present with skin findings, which is the second most common initial symptom (2) and would be critical to early diagnosis.

Cutaneous lupus erythematosus has been classified into three major subtypes: acute cutaneous lupus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus, with discoid lupus being the most common subtype (3). A butterfly-shaped (malar) rash on the cheeks and nose that is triggered by sunlight exposure is indicative of acute cutaneous lupus erythematosus. Subacute cutaneous lupus erythematosus is characterized by either ring-shaped red patches with scaly borders and lighter centers, or scaly red bumps in sun-exposed areas. Discoid lupus lesions are composed of red scaly plaques with both light and dark pigmentation, scarring, and skin thinning.

What causes Cutaneous Lupus?

While the overall picture of the development of cutaneous lupus is still being elucidated, it is thought that a complex network of multiple factors that cause systemic lupus is behind the etiology of cutaneous lupus. These factors include environmental triggers including medications, viruses, and sunlight, and abnormalities in various genes related to the immune system (4).  In particular, sun exposure is an important activator of cutaneous lupus. Ultraviolet light radiation can cause skin manifestations in cutaneous lupus patients by promoting cell death. These dead cells, in turn, become targets for autoantibodies. Antibodies are normally produced by the immune system to recognize foreign proteins in the body, which could be due to an infection. On the other hand, autoantibodies are abnormal antibodies since they react to the body’s own proteins and cause inflammation and damage to the body. In addition, ultraviolet light radiation also promotes the release of mediators called cytokines, which can ultimately activate and recruit inflammatory cells (4). An individual’s genetic makeup also likely predisposes him or her to cutaneous lupus. For instance, various genes important in recognition of foreign proteins (i.e. major histocompatibility complex genes) have been identified with higher frequency in cutaneous lupus patients (5). However, it is still unclear how these genes exactly lead to the formation of cutaneous lupus lesions. In summary, genetic, environmental, and immunological factors can contribute to the development of autoimmune reactions resulting in cutaneous lupus, and approaches to mitigating these causative factors are actively being sought (see below: Are Treatments Available?).

Who Gets Cutaneous Lupus?

Similar to systemic lupus, cutaneous lupus appears in approximately twice as many females as males (6). The highest peak of onset occurs in individuals in their 3rd and 4th decades of life (7). While African Americans appear to be more likely to develop discoid lupus than Caucasians, subacute cutaneous lupus patients show the opposite trend, with 85% of them being Caucasian (7). Although a specific genetic makeup can be a risk factor for cutaneous lupus development, the vast majority of cutaneous lupus patients do not appear to have relatives with systemic lupus (8).

Are Treatments Available?

There is no cure for cutaneous lupus; it is, rather, a chronic disease that can be controlled by a variety of medications and lifestyle changes such as limiting sun exposure through use of sunscreens and sun-protective clothing.  The following are examples of treatments currently being used for cutaneous lupus:

Sun avoidance: Sun protection is a very important proactive measure to prevent skin lupus flares caused by UVB radiation.  Physical sunscreens (clothes, wide-brimmed hats) as well as chemical sunscreens (lotions) are effective at blocking harmful ultraviolet radiation that can precipitate lupus-related rashes and other skin manifestations.

Steroids: These anti-inflammatory agents can be applied directly on the skin (topical), injected into the skin lesions (intralesional), or taken by mouth (oral). Prednisone is a commonly used oral corticosteroid that is reserved for severe disease and can quickly mitigate cutaneous lupus lesions.  Its mechanism of action involves decreasing antibody production and inflammatory cells.

Anti-malarials: This group of drugs, which include hydroxychloroquine, chloroquine, and quinacrine was made to initially treat malaria, but has also anti-inflammatory properties. The mechanism of action of these drugs is unknown. These drugs are often used as the first oral medications in cutaneous lupus because their side effect profile is less serious as some of the other oral medications.

Mycophenolate mofetil, azathioprine, methotrexate: These immunosuppressive medications are reserved for severe disease, and are long-term options for cutaneous lupus patients attempting to wean themselves off of oral steroids. They are thought to affect DNA synthesis, which would decrease production of inflammatory cells.

Thalidomide: Another long-term option for cutaneous lupus patients with severe disease, thalidomide is an inhibitor of a cytokine called TNF-α. Impeding the activity of this cytokine will decrease the severity of the immune response.

References

1. Rothfield N, Sontheimer RD, Bernstein M. Lupus erythematosus: systemic and cutaneous manifestations.  Clin Dermatol 2006; 24:348-362.

2. Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, et al. Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party of Systemic Lupus Erythematosus. Medicine (Baltimore) 1993; 72:113-24.

3.  Gilliam JN, Sontheimer RD. Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol 1981; 4(4):471-5.

4. Lin, JH, Dutz JP, Sontheimer RD, Werth VP. Pathophysiology of Cutaneous Lupus Erythematosus. Clinic Rev Allerg Immunol 2007; 33:85-106.

5.  Fowler JF, Callen JP, Stelzer GT, Cotter PK. Human histocompatibility antigen associations in patients with chronic cutaneous lupus erythematosus. J Am Acad Dermatol 1985; 12:73-7.

6. Durosaro O, Davis MDP, Reed KB, Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005. Arch Dermatol 2009; 145:249-53.

7.  Lee HJ, Sinha AA. Cutaneous lupus erythematosus: understanding of clinical features, genetic basis, and pathobiology of disease guides therapeutic strategies. Autoimmunity 2006; 38:433-44.

8.  Tebbe B, Orfanos CE. Epidemiology and socioeconomic impact of skin disease in lupus erythematosus. Lupus 1997; 6:96-104.