Bourneville-Pringle Disease Treated with Electrocauterization and Topical Tacrolimus 0.1%: A 1 Year Observation of Severity and Recurrence

Intan Nurmawati Putri, Yoga Hadi, Holy Ametati, Diah Adriani Malik, Meira D. K. A.

2019

Abstract

Bourneville-Pringle Disease (BPD) also known as tuberous sclerosis complex (TSC) is a rare autosomal dominant genodermatosis, which incidence varies from 1/12.000 to 1/14.000 live births. The genes implied to be mutated in this disease are tuberous sclerosis 1 (TSC1) and tuberous sclerosis 2 (TSC2). Diagnosis is made with two or more significant features or one major plus two or more minor features of the disorder. Management of BPD is supportive and symptomatic. Prognosis depends on systemic involvement. We reported an 11-year-old boy with various size firm tumors on his face, hypomelanotic macule on his back and slightly elevated skin-colored patch on his right lateral thigh. History of neurological manifestation and systemic complaint were adverse. Histopathological examination was following angiofibroma in BPD. Electrocauterization with general anesthesia combined with topical tacrolimus, 0.1% were performed for the angiofibromas in this patient. The prognosis was qua ad vitam dubia ad bonam, quo ad sanationam, and quo ad cosmeticam dubia ad malam. In this case, diagnose BPD was based on anamnesis, clinical features (three significant symptoms; angiofibroma, ash leaf macule, shagreen patch), and histopathological examination. The aim of angiofibroma therapy is for cosmetic purpose. Electrocauterization and topical tacrolimus 0.1% yielded a satisfactory result for the angiofibroma. Angiofibromas never worsen. There was an improvement in his FASI (Facial Angiofibroma Severity Index) score from severe to mild. Inhibitor mammalian of target rapamycin (mTOR) pathway might be considered. A proper therapy will improve a patient's quality of life.

Paper Citation

in Harvard Style

Putri I., Hadi Y., Ametati H., Malik D. and D. K. A. M. (2019). Bourneville-Pringle Disease Treated with Electrocauterization and Topical Tacrolimus 0.1%: A 1 Year Observation of Severity and Recurrence.In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease - Volume 1: ICTROMI, ISBN 978-989-758-469-5, pages 460-464. DOI: 10.5220/0009991504600464

in Bibtex Style

@conference{ictromi19,
author={Intan Nurmawati Putri and Yoga Hadi and Holy Ametati and Diah Adriani Malik and Meira D. K. A.},
title={Bourneville-Pringle Disease Treated with Electrocauterization and Topical Tacrolimus 0.1%: A 1 Year Observation of Severity and Recurrence},
booktitle={Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease - Volume 1: ICTROMI,},
year={2019},
pages={460-464},
publisher={SciTePress},
organization={INSTICC},
doi={10.5220/0009991504600464},
isbn={978-989-758-469-5},
}

in EndNote Style

TY - CONF

JO - Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease - Volume 1: ICTROMI,
TI - Bourneville-Pringle Disease Treated with Electrocauterization and Topical Tacrolimus 0.1%: A 1 Year Observation of Severity and Recurrence
SN - 978-989-758-469-5
AU - Putri I.
AU - Hadi Y.
AU - Ametati H.
AU - Malik D.
AU - D. K. A. M.
PY - 2019
SP - 460
EP - 464
DO - 10.5220/0009991504600464