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Successful Combination Therapy for Pulmonary Hypertension in Incontinentia Pigmenti: Case Report and Review of the Literature

Mattia Zanin, Enrico Bonaveglio, Martina Scanu, Annalisa Longobardo, Stefania Iannandrea, Elisa Catalano, Fortunato Ciliberto F, Sergio Grassitelli, Francesca Fusco, Mirela Bojan, Simona Quaglia

Pulmonary Arterial Hypertension (PAH) is a well-known but very rare and severe complication of Incontinentia Pigmenti (IP). Only few cases are reported in literature, most of them fatal. Recently, some reports described better outcome with a more aggressive treatment combining multiple pulmonary vasodilators and immunomodulatory drugs.

We present the case of a three months old female diagnosed with IP and severe PAH refractory to inhaled nitric oxyde, admitted in cardio-pediatric ICU for mixed cardiogenic and hemorrhagic shock and treated with a combination of high dose iNO, sildenafil, iloprost, TNF-alpha inhibitor and steroids. Following this treatment, PAP dropped from iso-systemic to half systemic, and PVRI halved as well. After an episode of septic shock and a hospital acquired pneumopathy from Pneumocystis jirovecii, the baby was discharged home in good conditions at the age of six months.