Cutaneous involvement ‘frequent’ in patients with catastrophic antiphospholipid syndrome

About half of the patients with catastrophic antiphospholipid syndrome have cutaneous involvement, which can help predict outcomes in this condition, according to a study.

“[Catastrophic antiphospholipid syndrome (CAPS)] is a rare but life-threatening condition, with a frequent delay in diagnosis,” Anastasia Dupré, MD, of the internal medicine service at Assistance Publique–Hôpitaux de Paris, Centre Hospitalo-Universitaire Cochin, in France, told Healio. “We thought it was important to study cutaneous involvement in CAPS, as it can help for the diagnosis in a patient with multisystemic failure.”

Dupré added that there was a lack of literature regarding the histologic features and evolution of these lesions.

In the retrospective cohort study, the researchers aimed to describe the clinical and pathological features of cutaneous involvement during CAPS, along with the outcomes of these complications. Data were accrued from the French multicenter APS/systemic lupus erythematosus register through December 2020.

“It is, to our knowledge, the biggest cohort of patients with cutaneous CAPS,” Dupré said. “We describe a new feature of skin involvement: distal inflammatory edema.”

The group collected data for 120 patients with at least one CAPS episode overall. The analysis included the 65 patients (median age, 31 years; age range, 12-69 years; 66% female) with cutaneous involvement.

Among 60 patients with available data, 35% demonstrated catastrophic APS as the first APS manifestation.

Regarding the lesion types, recent-onset or newly worsened livedo racemosa were observed in 45% of the cohort; 42% demonstrated necrotic and/or ulcerated lesions; 29% had subungual splinter hemorrhages; 23% had apparent distal inflammatory edema, which was marked by reddened and warm hands, feet or face; and 14% had vascular purpura. Patients may have demonstrated more than one of these outcomes.

The researchers also conducted 16 biopsies during CAPS episodes. Results of these analyses showed that 94% demonstrated microthrombi of dermal capillaries.

“The sensitivity of cutaneous biopsy was excellent, showing micro-thrombi in all patients but one, which can help for the diagnosis,” Dupré said.

Lesions healed without sequelae in 91% of 64 patients with such data available.

Further analysis showed that 37% of patients with cutaneous involvement experienced more frequent histologically proven CAPS, whereas just 24% of those without cutaneous involvement experienced this outcome. Mortality rates were 5% among CAPS patients with cutaneous involvement and 9% for those without.

“Cutaneous involvement in CAPS is frequent — about 50% of patients — and may have many different clinical aspects,” Dupré concluded. “You have to think about CAPS in a patient with APS in case of new onset or worsening of livedo racemosa, necrotic or ulcerated lesions, distal inflammatory edema, subungual splinter hemorrhages or vascular purpura.”