Association Found Between Degos-Like Lesions and Systemic Sclerosis, Other Manifestations of Vasculopathy

Article

New research explored associations between lesions resulting from Degos and diffuse cutaneous systemic sclerosis.

Lorinda Chung, MD, MS

Lorinda Chung, MD, MS

An association was identified between Degos-like lesions and diffuse cutaneous systemic sclerosis (SSc) as well as other cutaneous manifestations of vasculopathy, according to new findings.

The main aim of this research was to examine the clinical associations of the manifestations that are associated with Degos-like lesions in a substantial cohort of patients diagnosed with SSc.

Degos is a condition resulting in lesions on the skin, the pathogensis of which is related to endothelial dysfunction and to immune dysregulation.

The study was authored by Lorinda Chung, MD, MS, from Stanford University’s School of Medicine at the Palo Alto VA Health Care System.

“Degos-like lesions are cutaneous manifestations of a small-vessel vasculopathy that appear as atrophic, porcelain-white papules with red, telangiectatic borders,” Chung and colleagues wrote. “No study has adequately examined Degos-like lesions in patients with systemic sclerosis (SSc).”

Background

The investigators conducted a retrospective, cohort study of SSc patients that had been to the Stanford Rheumatologic Dermatology Clinic in the period between January of 1998 and December of 2018.

Participants recruited by the investigators for the study had to have been 18 years or older and had to meet the 2013 SSc of the American College of Rheumatology and European League Against Rheumatism classification criteria.

The research team gathered data on the participants’ demographic characteristics, on their clinical characteristics such as systemic and cutaneous SSc manifestations, on their autoantibody status, and on the existence of Degos-like lesions on the participants.

The investigators’ cohort that they assembled ended up with 506 total participants with SSc, with 88.3% being female and the mean age at the participants’ first non–Raynaud disease symptoms being about 46.

Study Results

The research team concluded that there were 27 participants (or 5.3%) with reported Degos-like lesions, and about 89% of those 27 showed lesions which affected their fingers.

For the participants with Degos-like lesions, there was a greater likelihood of their having diffuse cutaneous SSc versus the participants without lesions (15 [55.6%] compared to 181 [37.8%]; P=.04).

Furthermore, the research team found an association between Degos-like lesions and acro-osteolysis (10 [37.0%] compared to 62 [12.9%]; P<.01), calcinosis (15 [55.6%] compared to 115 [24.0%]; P<.01), and digital ulcers (15 [55.6%] compared to 173 [36.1%]; P=.04).

They also noted that although lesions were found not be associated with manifestations in the internal organs—including interstitial lung disease, scleroderma renal crisis, or pulmonary arterial hypertension—they did find P<.10 for an association with participants’ gastric antral vascular ectasia.

“Results of this study suggest an association of Degos-like lesions with diffuse cutaneous SSc and other cutaneous manifestations of vasculopathy, including acro-osteolysis, calcinosis, and digital ulcers,” they wrote. “A prospective longitudinal study is warranted to examine the onset of Degos-like lesions and to elucidate whether these lesions play a role in SSc.”

References:

Song P, Li S, Lewis MA, Fiorentino DF, Chung L. Clinical Associations of Degos-Like Lesions in Patients With Systemic Sclerosis. JAMA Dermatol. Published online February 08, 2023. doi:10.1001/jamadermatol.2022.6330

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