DESTIN, FLA. – Recognizing and treating discoid lupus early is imperative for preventing permanent scarring, according to Dr. Ruth Ann Vleugels.
When a patient presents with a "textbook case" of discoid lupus with the classic hyperpigmentation around the border with central atrophy and scarring that is characteristic of unchecked disease, it’s too late, she said at the annual Congress of Clinical Rheumatology.
Dr. Ruth Ann Vleugels
"When I see a case like this, I unfortunately can do nothing for them. There is no cream I can put on this, there is no laser I can give them ... all of this is permanent damage and scarring," she said, noting that even hair transplants in those with scalp scarring won’t work, because of the scar tissue.
For this reason, it is important to look for signs and symptoms – typically erythema and/or scaling, said Dr. Vleugels of Harvard Medical School, Boston.
Remember that erythema can look very different in different skin types, she advised, explaining that in light skin, the erythema will be red and obvious, but in darker skin it often looks violaceous and can be subtle.
"So we really, in our cutaneous lupus patients, need to pick up that activity in our darker skin patients to prevent scarring," added Dr. Vleugels, director of the autoimmune skin disease program at Brigham and Women’s Hospital, Boston.
Scaling is also an important feature that can help in diagnosing discoid lupus, and it may present with or without erythema.
Dr. Vleugels described one patient with scarring who clearly had existing disease, but who also had plaques with some erythema and scaling at the borders. While it was too late to do anything about the existing scars, the erythema and scaling represented disease activity that would lead to additional scarring without rapid treatment.
On the dorsal hands, discoid lupus tends to spare the knuckles, and on the scalp, the extensive scaling associated with discoid lupus can look like tinea capitis. Conchal bowl involvement is common, as is follicular plugging with excess keratin.
Another patient – a 19-year-old male with severe systemic lupus erythematosus who had been treated with cyclophosphamide and rituximab for renal disease, had impressive skin involvement, and at first glance, his skin disease may have appeared "a lost cause" under the circumstances. However, faint erythema was present throughout his scalp, and with aggressive treatment it was possible to bring back a lot of his hair.
"So in addition to maximizing the systemic regimen, something we do right away in these patients is give intralesional steroid injections to the scalp," Dr. Vleugels said, noting that the injections are very simple and easily learned.
She said she uses 10 mg/cc of intralesional triamcinolone diluted to 5 mg/cc with normal saline for scalp disease (and to a more cautious 3 mg/cc for facial disease), giving 0.1 cc per injection to avoid atrophy. The injections are given in the dermis, about 1 cm apart. The procedure can be completed in about 3 minutes in the office setting, and can be repeated every 4-5 weeks as needed.
Dr. Vleugels noted that she often gets referrals involving alopecia areata that has been confused with discoid lupus.
"Any patient with a circular area of hair loss, erythema, dyspigmentation, or scaling has discoid lupus," she said.
Both conditions can be treated with the intralesional injections, but the patient with signs and symptoms of discoid lupus requires a work-up for cutaneous lupus.
Scalp psoriasis can also be mistaken as discoid lupus because of the prominent scaling, but other characteristics of discoid lupus can help in making the diagnosis, she said, describing one such patient who also had the erythema and conchal bowl involvement that helped distinguish the two conditions.
Knowing the signs and symptoms of discoid lupus is important, because recent research suggests that a higher percentage of patients with cutaneous disease will go on to develop systemic disease than was previously believed, Dr. Vleugels said.
It was long held and taught that about 5%-10% of adult patients would go on to develop systemic disease, but 18% of more than 1,000 patients in a Swedish epidemiologic study who were followed for 3 years developed systemic disease (Br. J. Dermatol. 2011;164:1335-41), and 20% of those in a Mayo Clinic study who were followed for a mean of 8 years developed systemic disease.
"So this changes how we think about skin-only patients," she said, explaining that she follows adult patients a little longer than she used to, and screens them annually for systemic involvement.