An Atypical Case of Idiopathic Purpura Fulminans

Abstract

Idiopathic purpura fulminans (PF) is rare but has been reported in pediatric patients, commonly following infections. We present a case of a 5-year-old boy, heterozygous for factor V Leiden, with no history of recent infections, who presented with PF secondary to acquired protein S deficiency. Despite initial supportive treatment, the patient required surgical fasciotomy and extensive skin grafts. The protein S level normalized 4 months following the presentation. In this context, an autoimmune component with transient anti-protein S antibodies was believed to be involved. This case report highlights the course of idiopathic PF due to noninfectious acquired protein S deficiency.

Púrpura fulminante postinfecciosa: a propósito de un caso

“…2,[4][5][6][7][8][9] Las lesiones cutáneas aparecen en forma repentina y progresan con rapidez, pero en ausencia de signos clínicos de sepsis, a diferencia de la púrpura de causa infecciosa. Se acompaña de un estado de hipercoagulabilidad secundario a un déficit transitorio de proteína S y/o C. 2,9 La rápida progresión clínica se correlaciona con los hallazgos histológicos; se observa trombosis venosa extensa de la dermis con infarto hemorrágico de los tejidos adyacentes, presencia de microtrombos que causan dilatación capilar y congestión con glóbulos rojos en los estadios tempranos de la PF. 1,3 En estadios más avanzados, se observa necrosis fibrinoide con extravasación de glóbulos rojos en la dermis y fenómenos de vasculitis leucocitoclástica.…”

Postinfectious purpura fulminans: A case report

“…It usually affects the buttocks, thighs, and calves symmetrically. 2,[4][5][6][7][8][9] S k i n l e s i o n s a p p e a r s u d d e n l y a n d progress rapidly, but in the absence of clinical signs of sepsis, unlike infectious purpura. It is accompanied by a hypercoagulable state s e c o n d a r y t o a t r a n s i e n t p r o t e i n S o r C deficiency.…”

“…It is accompanied by a hypercoagulable state s e c o n d a r y t o a t r a n s i e n t p r o t e i n S o r C deficiency. 2,9 The rapid clinical progression correlates to…”