An atypical presentation of antiphospholipid antibody syndrome

Abstract

Cutaneous manifestations in antiphospholipid antibody syndrome (APS) though common, are extremely diverse and it is important to know which dermatological finding should prompt consideration of antiphospholipid syndrome. The cutaneous manifestations of APS vary from livedo reticularis to cutaneous necrosis, and systemic involvement is invariably an accomplice in APS. Cutaneous ulcers with sharp margins can be seen in APS and they are usually seen on the legs. This case had an atypical presentation, as the initial presentation was painful necrotic ulcers over the legs, which resembled pyoderma gangrenosum and she had no systemic manifestations. There was no history of any arterial or venous thrombosis or any abortions. Antiphospholipid syndrome can be tricky to diagnose when cutaneous lesions are atypical. Nonetheless, it is very important to pin down this syndrome early due to its systemic complications.

Anti-phospholipid antibody syndrome with skin as a primary involved organ: an interesting case report

“…5 Though many a times cutaneous features are mostly associated with systemic findings, but isolated involvement of skin has also been reported even in female patients. 6 APLA syndrome is divided into primary and secondary APLA syndrome the former one is isolated while later one is associated with systemic diseases the most common among it is SLE. 7 Our present clinically presented with rapidly developing cutaneous oedema with ulcers which later on developed to involve dorsum of hand also and though these kind of rapid evolution of disease is seen in catastrophic APLA syndrome commonly our patient didn't show multiorgan involvement or systemic thrombosis with thrombocytopenia may be due to early intervention.…”

“…The treatment of choice for APLA syndrome includes corticosteroids, heparin, oral warfarin and aspirin. 6 As our patient also has widespread cutaneous oedema with ulcers, surgical intervention was done to remove necrotic tissue and release oedema was done. 7 Also had it not been treated on time, there were chances of widespread cutaneous necrosis which is a very rare phenomena reported in very few case reports.…”

Pyoderma Gangrenosum Mimicking Antiphospholipid Antibody Syndrome in a Child With Juvenile Systemic Lupus Erythematosus

“…The cutaneous manifestations of APS vary from livedo reticularis to cutaneous necrosis. Ulcers resembling PG have been described seldom in APS and may cause confusion in diagnosis [18,19]. In addition, less than one third of patients with APS show histologic evidence of coagulopathy [20].…”