The “Flolan Rash”: An Impressively Asymptomatic and Non-Inflammatory Eruption Following Long-Term Epoprostenol Therapy
November 2022 | Volume 21 | Issue 11 | 1249 | Copyright © November 2022
Published online October 19, 2022
Dana M. Hutchison MD MSca, Margit Juhasz MD MScb, Janellen Smith MDb, Bonnie A. Lee MDb
aUniversity of California, Irvine, School of Medicine, Irvine, CA
bUniversity of California, Irvine, Department of Dermatology, Irvine, CA
Abstract
Epoprostenol (Flolan) is a last-resort intravenous medication for the treatment of severe pulmonary arterial hypertension (PAH). Cutaneous adverse events of Flolan are well-known by pulmonologists, though lacking in dermatologic literature. We report a near erythrodermic appearing, yet asymptomatic eruption lasting 10 years in a woman with end-stage PAH treated with long-term intravenous epoprostenol. Non-pruritic, blanching, erythematous papules coalescing to plaques surrounded by a hypopigmented halo encompassed her entire torso, as well as bilateral upper and lower extremities. Additional findings included bright red palms and soles associated with pain and tingling while walking. Laboratory workup revealed thrombocytopenia and a slightly elevated erythrocyte sedimentation rate (ESR); connective tissue disease markers were negative. Skin biopsies were, surprisingly, largely unremarkable without an inflammatory infiltrate. The patient was trialed on topical clobetasol ointment without effect. Her striking, yet asymptomatic and non-inflammatory eruption was thought due to long-term use of epoprostenol, a last-resort synthetic prostacyclin used to treat severe PAH. As her cutaneous findings were not bothersome, her dose of Flolan was not lowered and her lower extremity pain was treated with gabapentin. With this case, we aim to increase awareness of the impressive “Flolan rash”, a persistent erythematous eruption well-known by pulmonologists, yet scarcely described in dermatologic literature.
Significant Finding: We report a striking, yet asymptomatic and non-inflammatory skin eruption lasting 10 years presumed due to long-term use of epoprostenol for end-stage pulmonary arterial hypertension.
Meaning: Cutaneous adverse events of intravenous epoprostenol are well-known by pulmonologists, though lacking in dermatologic or primary care literature. The extensive body surface involvement, and near erythroderma, associated with Flolan necessitates awareness by patients, dermatologists, and other healthcare providers outside of the field of pulmonology.
J Drugs Dermatol. 2022;21(11):1249-1251. doi:10.36849/JDD.6821
INTRODUCTION
Epoprostenol (Flolan) is a last-resort intravenous (IV) medication for the treatment of severe pulmonary arterial hypertension (PAH). Cutaneous adverse events of Flolan are well-known by pulmonologists, though lacking in dermatologic literature.1 We report an extensive near erythrodermic appearing asymptomatic eruption following long-term use of epoprostenol. This characteristic and striking eruption associated with Flolan necessitates awareness by healthcare providers outside of the field of pulmonology in order to avoid unnecessary procedures.
CASE
A 57-year-old woman with a past medical history of end-stage PAH treated with continuous IV epoprostenol (maximum dose 89 ng/kg/min) for 10 years presented to the emergency department for a self-reported tender nodule of the shin and burning sensation of her right lower extremity. On exam, no palpable nodule was appreciated. However, she had an extensive, yet asymptomatic confluent and blanchable erythematous eruption surrounded by a hypopigmented halo, encompassing her entire torso and all extremities. She further had brightly erythematous palms and soles associated with painful tingling while walking
