Concomitant Incontinentia pigmenti and antiphospholipid antibody syndrome leading to diagnostic dilemma in a case of bad obstetric history: First report of the association

Abstract

Antiphospholipid Antibody syndrome (APS) is an autoimmune disease characterized by recurrent thrombotic events, pregnancy morbidity, and persistence of Antiphospholipid antibodies (APLA). Incontinentia pigmenti is a rare X-linked dominant disorder, lethal in the majority of affected males in utero and variably expressed in females. Herein we present a case of a cutaneously asymptomatic lady with bad obstetric history who was detected to have APLA syndrome during Antenatal Care, with a dermatological referral to the dermatologist of her offspring leading to detection of Incontinentia pigmenti in the otherwise healthy offspring. We present this case to highlight the diagnostic difficulties in the coexistence of these two conditions in the same patient, particularly when the autosomal dominant gene is not expressed in females.