Cutaneous lupus erythematosus (CLE) is present in 25% of patients with systemic lupus at the time of diagnosis, but it can also occur in up to 85% of cases at some point in their disease course,, said during the virtual annual meeting of the Society for Pediatric Dermatology.
“CLE can also occur without any systemic disease,” said Dr. Wu, associate professor of pediatrics at the University of North Carolina at Chapel Hill. “It’s been shown that the risk of developing systemic lupus differs according to the type of skin involvement, meaning that cutaneous lupus can be classified into acute, subacute, chronic, and intermittent forms.”
Malar rash is the prototypical acute cutaneous lesion and is associated with active systemic lupus erythematosus (SLE) and anti–double stranded DNA antibody positivity, while discoid lupus erythematosus is the most common chronic lesion. “A small percentage of patients with discoid lupus can develop systemic lupus, particularly when the lesions are more disseminated,” said Dr. Wu, who specializes in pediatric rheumatology as well as allergy and immunology.
In the American College of Rheumatology’s 1997 classification system, mucocutaneous manifestations constitute 4 out of the 11 criteria that clinicians use to make a diagnosis of SLE: malar rash, discoid-lupus rash, photosensitivity, and oral or nasal mucocutaneous ulcerations. Dr. Wu recommends performing an oral exam on suspect cases, “because the oral ulcers that we see in systemic lupus tend to be painless, so oftentimes patients don’t realize they have them.”
Five other organ-specific manifestations of SLE include nonerosive arthritis, nephritis, encephalopathy, pleuritis or pericarditis, and cytopenia. The two other criteria are positive immunoserology and a positive antinuclear antibody test. “If you have any individuals present with one of these [mucocutaneous manifestations criteria], you want to think about getting a CBC to look for cytopenia or a urinalysis to look for evidence of nephritis, and potentially some additional blood studies, depending on your level of suspicion for systemic lupus,” Dr. Wu said.
Other rarer CLE manifestations include lupus pernio or chilblains, lupus panniculitis, livedo reticularis, bullous LE, urticarial vasculitis, neutrophilic dermatoses, and alopecia.
Common treatments for cutaneous manifestations associated pediatric SLE include hydroxychloroquine, low dose corticosteroids, topical steroids, methotrexate, and leflunomide. Other options for increasing severity of systemic disease include lenalidomide/thalidomide, azathioprine, calcineurin inhibitors, belimumab (Benlysta), high-dose corticosteroids, mycophenolate mofetil (CellCept), rituximab (Rituxan), and cyclophosphamide. Cutaneous manifestations of pediatric SLE can often be refractory to treatments.
In 2017, Dr. Wu and associates published aof 10 adolescents who received lenalidomide for refractory CLE. One of the subjects was a 21-year-old male with a significant malar rash despite being on hydroxychloroquine, azathioprine, and prednisone 40 mg daily. “One month after being on lenalidomide he had a pretty impressive response,” Dr. Wu said. “It’s not quite clear how lenalidomide works in cutaneous lupus. Currently it’s only approved for use in myelodysplastic syndromes, multiple myeloma, as well as certain lymphomas. It’s thought to modulate different parts of the immune system, which collectively result in the cytotoxicity against tumor cells.”
Lenalidomide is supplied in capsule sizes ranging from 2.5 mg to 25 mg and is given once daily. “For a smaller child, I would think about starting 5 mg once a day,” Dr. Wu said. “For an adult-sized adolescent, you could start at 10 mg once a day and then titrate up based on response. Side effects that you need to worry about are cytopenia and GI symptoms. The venous and arterial thromboembolism risk has been seen in patients with multiple myeloma, and it is unclear if this risk is applicable to all indications.” Use of the medication requires enrollment into a safety monitoring program.
She reported having no financial disclosures.