Lupus & antiphospholipid syndrome

Skin involvement in the catastrophic antiphospholipid syndrome: A review from catastrophic antiphospholipid syndrome (CAPS) registry

LupusPublished Cross-sectional study

Authors' conclusion

The presence of skin involvement in patients with CAPS is frequent. The most frequent manifestations were livedo reticularis and skin necrosis. Skin involvement does not seem to be associated to mortality.

Study context

Population
Patients with CAPS in the CAPS Registry (European Forum on aPL), April 1992 to December 2024377 episodes
Exposure
CAPS episodes with vs without skin involvement
  • Skin involvementn = 377
Outcome
Prevalence of skin involvement in CAPS
Methodological detailsregistry-based, Female 49.4%; mean age 39.4 (SD ± 17.9) years
  • Registry-based
  • Female 49.4%; mean age 39.4 (SD ± 17.9) years

Main finding

As reported in the abstract

Cutaneous involvement (prevalence)

Skin involvement

47%

377

Observational data: associations, not effects.

Further findings

Most frequent skin manifestations

Livedo reticularis
17.6%
Skin necrosis
13%
Ischemic ulcers
10.4%
Skin ischemic
9.7%
Skin purpura
7.1%
Gangrene
5%
Splinter haemorrhages
2.6%
RP
1.5%

Most frequently affected organs in CAPS episodes with skin involvement

Kidneys
72%
Lungs
61%
Central nervous system
54.5%
Heart
54.4%

Thrombocytopenia and hemolysis features

More common in patients with skin involvement
P < 0.05 for all

Predominant pathological finding

Thrombotic microangiopathy, episodes
82 (68.3%)

Mortality, episodes with vs without skin involvement

No difference: 29.3% vs 32.1%
P = 0.42

Original publication

Skin involvement in the catastrophic antiphospholipid syndrome: A review from CAPS registry. Lupus. Published 15 June 2026. doi:10.1177/09612033261461987

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