# Antiphospholipid syndrome presenting as extensive skin ulcers on unilateral lower extremity: a case report

## TL;DR

This case report describes a rare presentation of antiphospholipid syndrome with severe skin ulcers on one leg, without vasculitis.

## Contribution

The report highlights a rare nonthrombotic manifestation of APS with extensive skin ulcers and inflammatory lipoatrophy.

## Key findings

- APS can present with extensive skin ulcers without cutaneous vasculitis.
- The case involved severe inflammatory lipoatrophy and positive anti-β2-glycoprotein I and lupus anticoagulant.
- This case emphasizes the importance of recognizing nonstandard APS manifestations.

## Abstract

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent arterial and venous thrombosis, habitual fetal miscarriages, often accompanied by mild to moderate thrombocytopenia, and persistent moderate-to-high titer positivity for antiphospholipid antibodies (aPLs). However, patients with antiphospholipid antibodies may also present with several nonthrombotic clinical manifestations, such as thrombocytopenia, cardiac valve disease, nephropathy, skin ulcers, or cognitive dysfunction, which are collectively referred to as nonstandard manifestations of APS. Of these, for APS with predominantly cutaneous ulcers, previous reports have focused on APS with combined cutaneous vasculitis, and its medical treatment, rather than cutaneous ulcers with predominantly fatty inflammatory lesions, and the associated surgical treatment. Here, we admitted a relatively rare case of primary APS with extensive skin ulceration of the right lower extremity, without cutaneous vasculitis, in the presence of extensive and severe inflammatory lipoatrophy, carrying anti-β2-glycoprotein I and lupus anticoagulant, which is reported as follows, with a view to raising awareness of this disease.