48 yo woman with a 32 year history of cutaneous lupus.

The patient is a 48 yo woman who presents with these words, "I am a monster!."  She has had cutaneous lupus since age 16.  Her major problems include disabling arthritis and cutaneous disease.  She has been married for 30 years and has three children.  Her eldest daghter has had two miscarriages.  Her second pregnancy was with twins. One died intrautero and the second, born at 5 months gestation is blind
secondary to retrolental fibroplasia.  The patient had a stroke 5 years ago from which she has had a complete recovery. She was under the care of a rheumatologist for 17 years and has been unsuccessfully treated with methotrexate, plaquenil, Enbrel, Remicade and Dapsone.  NSAIDs have been disappointing for her arthritis with the exception of Vioxx which is now not available. She takes prednisone 7.5/5 mg on alternate days.  She does not drink and has a 34 pack year smoking habit since age 14.  By history her rash is always present, but it is exacerbated in the summer.

Album of photos accessible at: Picasa VGRD.  Link will be disabled after two weeks.

The link is https://picasaweb.google.com/djelpern/VGRDJuly2011?authkey=Gv1sRgCKja_oTMrdLU4AE

Laboratory Data

CBC normal
BUN and Creatinine normal
ANA + 1:160 ? pattern
C3, C4 -- low,
Rheumatoid Factor + 252 (nl 0 - 20)
SSA + > 8
CRP normal

Pathology shows focal parakeratosis, an atrophic dermis, basal layar vacoulozation, numerous necrotic keratinocytes, mild sperficial perivascular interstitial and perifollicular infiltrate with numerous neutrophils.  Felt to be compatible with subacute lupus.

Most likely this patient has chronic cutaneous lupus

Plan:  I think we should be able to help her arthritis and skin disease. I would start by reinstituting Plaquenil (hydroxychloroquinine) and adding Imuran (azathioprine).  She will be referred to a rheumatologist for diagnostic and therapeutic suggestions.  The question of antiphospholipid disease needs to be pursued for the patient and her daughter.

What are your thoughts?  How long would you give the azathioprine?  Which biologics would you use? She has not done well with Enbrel (etanercept) or Remicade (infliximab).

Do you make a distinction between chronic cutaneous lupus and subacute lupus and is this important?  What are the psychosocial considerations of living with such a disorder for thirty years?  Should the presence of antiphosphid antibodies be looked for?

Lupus, subacute lupus, chronic cutaneous lupus, lupus anticoagulant, antiphospholipid antibody, disfigurement

Khaled El-hoshy MD, Dermatologist, Cairo, Egypt on July 7, 2011

My impression is Subacute cutaneous LE. I agree with the antimalarials. Would suggest oral steroids 15-20 mg/day for 3-4 weeks then taper as condition improves. Maybe switch to topical steroids class 1 or 2.

Robert Rudolph MD, Clinical Professor of Dermatology, University of Pennsylvania, Philadelphia, PA, USA on July 7, 2011

I'd use Plaquenil 200 mg BID, along with CellCept 500 mg QID - and might also add Accutane. Potent topical steroids should be used, and an occasional Kenalog 40 IM would be helpful.

Rick Sontheimer MD, Professor of Dermatology, Univ. of Utah, Salt Lake City, Utah, USA on July 8, 2011

I suspect that this woman's initial diagnosis was SCLE but due to the therapeutic refractoriness and persistence of her SCLE disease activity she has evolved superficial cutaneous atrophy in her lesions evident on the photographs that could raise the question of classification currently as generalized discoid LE (we have seen and informally published such transitions in prior chapters and reviews that we have written in the past).

However, her elevated Ro/SS-A autoantibody levels and her low complement levels would argue in favor of SCLE over generalized DLE. In addition, her refractory joint symptoms and elevated rheumatoid factor level would raise the possibility of an overlap with rheumatoid arthritis (ie, "Rupus"). SCLE has been more often reported to overlap with rheumatoid arthritis than has discoid LE. Also, this is the type of patient who can develop overlapping features with Sjogren's syndrome over time. Does she have a history of dry eyes or dry mouth?

For full commentary on the case click here.

Khalid Al Aboud MD, Dermatologist, King Faisal Hospital, Makkah, Saudi Arabia on July 8, 2011

Advice to stop smoking and avoid the sun. Use physical sunscreen if possible. I just want to add that unresponsiveness might be related to sub therapeutic dose of the drugs she was using. If the pregnancy is not a problem for her, you may try Thalidomide. It is effective for the treatment of severe cutaneous lupus.

Ambajogai Ovhal MD, Assistant Professor, Dept of Dermatology, Government Medical College, Latur, India on July 17, 2011

I think this description suits chronic cutaneous LE. I'm in favour of Methylprednisolone 1g and Azathioprine (100 mg) or cyclophosphamide 500mg pulse therapy for 3 days in a month followed by azathioprine in its therapeutic dose for 28 days. Continue pulse therapy for 6 such cycles. Meanwhile continue with hydroxychloroquine. We have to taper steroids by 5 mg at every pulse. Then after 6 months of good control without oral steroids we can think of azathioprine 50mg od for next 6-12 months along with Hydroxychloroquine. We can also consider methotrexate, cyclosporin in place of azathioprine. It does not matter whether the disease is chronic or subacute, its academic matter. We can rule out antiphospholipid titres for additional benefits. Along with this other measures should be very well practiced. Psychosocial assessment should be done in association with psychiatrist.

David Cook MD, Clinical Senior Lecturer, Dermatology, Concord Clinical School, Univesity of Sydney, NSW, Australia on July 17, 2011

Hydroxychloroquine is unlikely to be helpful in this case if the patient continues to smoke cigarettes. I would recommend a trial of thalidomide to control her cutaneous disease if all appropriate precautions are undertaken. She also needs a thrombophilia screen to exclude an associated thrombotic disease to account for the history of CVA at a young age. Her joint symptoms are not in keeping with a diagnosis of subacute LE and the raised Rheumatoid Factor in absence of a raised ANA suggest a cutaneous lupus and RA overlap. She may get improvement in the joint symptoms with thalidomide but my experience is that it will be more helpful for her cutaneous disease.

Nasser Altamimi MD, Dermatologist, Tarim, Yemen on July 20, 2011

Most likely diagnosis is chronic cutaneous lupus. Advice: 1-stop smoking and avoid the sun by physical sun screen. 2-Plaquenil + Imuran 3-Follow up by a rheumatologist

Abdullah Mancy MD, Dermatologist, AL-Ramadi Teaching Hospital, Ramadi, Iraq on July 24, 2011

There is what is called complement deficiency-associated lupus characterized by early onset, photosensitivity, less renal disease and Ro/La autoantibodies. Can this be applied to this patient? What about family history? We also know that TNF inhibitors may cause drug-induced lupus that has skin disease with malar rash, discoid lesion and photosensitivity. I think this patient may have complement deficiency-associated lupus which is aggravated by the administration of TNF inhibitors.