Systemic Lupus Erythematosus (SLE) is a devastating autoimmune disease that can affect nearly every organ system in the body. The skin is no exception, and in fact, may be involved in 70-80% of cases. An equal number of patients may experience abnormal sun or ultraviolet light sensitivity, called photosensitivity. There are also unique forms of lupus that involve predominantly the skin associated with photosensitivity with little or no blood or internal organ involvement. These include Discoid Lupus Erythematosus (DLE),
now called Chronic Cutaneous Lupus Erythematosus (CCLE), Subacute Cutaneous Lupus Erythematosus (SCLE), and the much less
Lupus Erythematosus which affects newborns. For this reason, I think it is important for dermatologists to be aware of these lupus variants and become comfortable with managing the diagnostic evaluation of such cases, if not the treatment, which is somewhat more complex.
All of the lupus variants are considered connective tissue diseases and therefore have features in common and sometimes overlap or coexist with other well-known connective tissue diseases: scleroderma, now called Progressive Systemic Sclerosis, Dermatomyositis, Rheumatoid Arthritis, and Sjogren’s Syndrome. Because all of these conditions have prominent skin and mucous membrane manifestations, the medical dermatologist should be able to diagnose and distinguish them from one another or emphasize their overlapping features.
The following cases show that the efficient use of time and testing by the dermatologist can help to find the correct diagnosis and render the appropriate treatment in a cost-effective manner in only one or two visits.
1. A middle-aged woman was referred to me by a rheumatologist with the chief complaint of bruising on her arms and a rash on her back. She was worried about autoimmune disease and consulted the rheumatologist to find out if she had lupus. In his thorough evaluation of her history and physical examination, he couldn’t any find anything to support the lupus diagnosis. She was very tanned and had superficial blood bruises on the backs of her arms, called purpura. On her upper back there were a few red raised hive-like bumps that were itchy.
I wanted to reassure her that she did not have lupus and therefore performed skin biopsies on the forearm and the back including one for immunofluoresence testing which can show lupus antibodies if present. I also ordered the blood tests for lupus including the anti-nuclear antibody (ANA) and several others that are associated with SLE. The forearm biopsy showed evidence of sun damage, thin skin, and blood leakage as expected. However, the back biopsies were consistent with lupus including the immunologic staining. The patient was right! Moreover, the ANA was positive. The only other positive lupus antibody was SS-A. People who have this antibody in their blood tend to have extreme sun sensitivity. On the basis of all the tests obtained on the first visit, I was able to tell her that she does indeed have Subacute Cutaneous Lupus Erythematosus (SCLE) without other organ involvement. The bruises were not due to the SCLE but rather the chronic excessive sun exposure in her hypersensitive state. I recommended the use of sunscreens, both creams and the oral supplement Heliocare, as well as sun avoidance. At her last follow-up visit, all of her skin lesions had resolved.
2. A 69-year-old woman was referred by her dermatologist for the treatment of generalized lichen planus (LP) of several years’ duration. The rash had been getting worse and spreading all over the body, including sun-protected areas. The rash consisted of red raised bumps and healing lesions which showed increased pigmentation. She did not complain of itching at all even though LP is considered to be one of the itchiest autoimmune skin diseases. Numerous skin biopsies had been performed over the years which showed an “interface dermatitis” with negative immunofluorescence and felt to be more consistent with the diagnosis of LP. The ANA blood test from years ago was negative.
I performed 3 skin biopsies including the immunofluorescence test for skin antibodies and re-ordered the ANA as well as other blood antibodies associated with lupus. All 3 skin specimens showed the changes consistent with lupus, but the lupus antibody was negative. The blood test helped to save the day: the ANA and SS-A antibody were positive indicating Subacute Cutaneous Lupus Erythematosus (SCLE). Because the rash was so extensive, I decided to treat her with the anti-malarial drug commonly used to treat lupus of the skin called Plaquenil or the generic hydroxychloroquine. After one month of treatment on a low dose along with limiting sun exposure, her skin was almost clear.
Comment: In 1984, I wrote an article for the Journal of the American Academy of Dermatology describing 6 cases of the Lupus erythematosus/Lichen planus Overlap Syndrome. Some of those patients turned out to have SCLE as the tests for detecting circulating antibodies against self became more sophisticated and accurate. This case may have been an example of the LE/LP overlap.
Lupus is much more common in young women than in men (about 9 to 1). But it must be remembered that elderly women can be affected as in this case, and the proper tests must be ordered and repeated if necessary to obtain the right diagnosis.
3. An elderly woman presented herself for a red itchy face. She had been previously
exposed to poison ivy and knew she was allergic. Her family doctor was treating her with prednisone and antibiotics to cover all the bases of allergic reaction, rosacea, and infection. She was concerned about the possibility of lupus because of bright redness of the cheeks extending over the bridge of the nose resembling the classic “butterfly rash” of SLE. She had no other symptoms of lupus except for photosensitivity which may have been exacerbated by the oral antibiotic.
In addition to the central facial rash, she had small individual red edematous papules, almost blisters, on the forehead. I took
biopsy specimens from these including immunofluorescence and ordered the lupus battery of blood antibodies. The skin biopsies showed allergic contact dermatitis with negative immunofluorescence. All of the blood tests were normal. She responded well to a long-acting steroid shot and a mild steroid cream to the face. All the other medications were discontinued.
There is a cliche used in medicine as in life: “Common things are common.” Her diagnosis was incompletely treated allergic contact dermatitis to poison ivy.
Other Interesting Case Studies:
Bullous Pemphigoid: Please Don’t Confuse It With Pemphigus Vulgaris