Rheumatologists at ‘forefront’ of diagnosing scleroderma skin disease
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Rheumatologists are likely to be at the “forefront” of diagnosing scleroderma skin disorders and other dermatological conditions, a speaker said at Congress of Clinical Rheumatology East.
“Dermatologists, and you in rheumatology, are really on the forefront of diagnosing systemic sclerosis because those cutaneous features are really so key,” Avery H. LaChance, MD, MPH, director of the Connective Tissue Diseases Clinic at Brigham and Women’s Hospital, and assistant professor of dermatology at Harvard Medical School, told attendees.
In addition to scleroderma, LaChance addressed morphea and eosinophilic fasciitis in the second part of her popular “Dermatology for the Rheumatologist” talks at CCR East.
Puffy fingers may be the primary presenting complaint for patients with SSc, she said. In addition, late onset and/or asymmetric Raynaud’s phenomenon should raise suspicion for SSc.
“Raynaud’s, nailfold capillary change and sclerodactyly are really the hallmark features of SSc,” she said.
Regarding treatment, rheumatologists should consider mycophenolate mofetil as a first line to treat not only cutaneous fibrosis, but also so-called “salt-and pepper dyspigmentation,” which can be observed in some scleroderma patients with darker skin tones.
For patients with microstomia — which LaChance suggested is a major source of body image dissatisfaction in this patient population — hyaluronidase injections may be considered.
For scleroderma patients with severe Raynaud's disease of the hands and/or feet, LaChance offered some “controversial” advice.
“I am going to be a little bit controversial here and suggest that we really should be thinking about Botox injections for some of our patients with Raynaud’s,” she said.
Regarding morphea — which she described as a chronic, autoimmune, fibrosing condition of the skin and subcutaneous tissue — LaChance said there is no clear profile of laboratory abnormalities that mark this patient population.
“I really think of morphea as a clinical diagnosis,” she said.
Women are more likely than men to have morphea, which can occur in both pediatric and adult populations.
“It is more common in our white population,” LaChance said.
Morphea may have an antigenic trigger in genetically predisposed hosts, according to LaChance. She added that the immunology of morphea is “biphasic,” with a primary inflammatory response followed by fibrosis.
There are multiple subtypes of morphea, ranging from generalized to pansclerotic. LaChance encouraged rheumatologists to familiarize themselves with the myriad presentations, as this will guide therapeutic decision-making.
Regarding treatment, methotrexate with or without systemic corticosteroids is the first-line option for all subtypes beyond plaque morphea.
“Mycophenolate mofetil has recently been shown to be an effective alternative for patients who do not respond to or cannot tolerate methotrexate,” LaChance added.
The last condition LaChance covered was eosinophilic fasciitis, which she described as a rare fibrosing disorder characterized by erythema, edema and induration of the bilateral upper and lower extremities.
Women are more commonly impacted, particularly those aged 30 to 60 years.
“We do not know what causes this,” LaChance said, adding that it may be brought on by physical stress. “It is thought to be on the severe end of the morphea spectrum.”
Importantly, eosinophilic fasciitis is marked by the absence of Raynaud’s, nailfold capillary change and microstomia, she said. Rather, a groove sign is present, along with mobility of the skin over distal dorsal fingers and the “cobble stoning” of proximal extremities.
“You are going to be on the front line for diagnosing these patients,” Lachance said.
MRI can be used in the diagnosis of eosinophilic fasciitis and is associated with lower morbidity than fascial biopsy.
“We think of MRI as confirmation of the diagnosis,” LaChance said.
Combination therapy with immune suppression and systemic steroids with methotrexate or mycophenolate mofetil is optimal for these patients, according to LaChance.
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LaChance AH. CPPD: Dermatology for the Rheumatologist 2: Fibrosis and Sclerosis. Presented at: The Congress of Clinical Rheumatology East; May 12-15, 2022; Destin, Fla.
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