50% of CAPS patients present with cutaneous involvements, including distal inflammatory edema: JAMA

A new study found that in patients with Catastrophic antiphospholipid syndrome (CAPS), nearly half of them presented with cutaneous involvement having a wide spectrum of clinical presentations like distal inflammatory edema. The study results were published in the journal JAMA Dermatology.  

Antiphospholipid syndrome (APS) presents with a rare, severe complication called Catastrophic antiphospholipid syndrome (CAPS) characterized by diffuse small-vessel thromboses leading to multiorgan failure. Despite several clinical presentations, cutaneous involvement has not been thoroughly described. Hence researchers conducted a study to describe the cutaneous involvement, its clinical and pathological features, and outcomes in these patients and to compare them without cutaneous involvement. 

A retrospective cohort study was conducted by including patients from the French multicenter APS/systemic lupus erythematosus register (ClinicalTrials.gov: NCT02782039) by December 2020. All patients meeting the revised international classification criteria for CAPS were included, and patients with cutaneous manifestations were analyzed more specifically. Patients with CAPS having cutaneous involvement, clinical and pathological data, as well as course and outcome, were gathered from patients and compared to those in the register who did not have cutaneous involvement. 

Results: 

• Among 120 patients with at least 1 CAPS episode, there were 65 (54%) with skin involvement with a median [range] age, of 31 [12-69] years).
• Among the total 43 [66%] were women.
• Among 21 of 60 (35%) patients with available data, Catastrophic antiphospholipid syndrome was the first APS manifestation.
• The main lesions were recent-onset or newly worsened livedo racemosa (n = 29, 45%), necrotic and/or ulcerated lesions (n = 27, 42%), subungual splinter hemorrhages (n = 19, 29%), apparent distal inflammatory edema (reddened and warm hands, feet, or face) (n = 15, 23%), and/or vascular purpura (n = 9, 14%).
• Microthrombi of dermal capillaries were seen in 15 out of 16 biopsies performed during CAPS episodes (94%).
• In more than 90% (58 of 64) of patients, these lesions healed without sequelae. 
• Patients with cutaneous involvement showed a trend toward more frequent histologically proven CAPS (37% vs 24%, P = .16) than those without such involvement, while mortality did not differ significantly between the groups (respectively, 5% vs 9%, P = .47). 

Thus, the present study reported a wide variety of skin involvement during CAPS and their healing without major sequelae in most patients. 

Further reading: Dupré A, Morel N, Yelnik C, et al. Cutaneous Involvement in Catastrophic Antiphospholipid Syndrome in a Multicenter Cohort of 65 Patients. JAMA Dermatol. Published online December 07, 2022. doi: 10.1001/jamadermatol.2022.5221