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A case of severe allergic eosinophilic asthma with nasal polyposis in a patient affected by Birt-Hogg-Dubé syndrome successfully treated with benralizumab


C. C. De Pace cosimo.de.pace26@gmail.com*, G. Scioscia*, D. Lacedonia, P. Fuso, G. Lepore, M. G. Palumbo, M. P. Foschino-Barbaro

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Doi
10.23822/EurAnnACI.1764-1489.222

Summary
Birt-Hogg-Dubé (BHD) syndrome is a rare genetic pathology characterized by cutaneousfibrofolliculomas, pulmonary cysts and kidney tumours. Severe asthma is themost serious form of asthma which does not respond to standard treatments. Wepresent the case of a 68 years old male patient who had frequent respiratory tract infections,shortness of breath and decline in lung function, nasal polyposis and hypertrophyof the nasal turbinates, for this reason he was treated as a severe asthmatic patient forseveral years with ICS + LABA and with high doses of OCS. When we tried to reduceOCS, the patient's symptoms worsened, so we requested a HRTC scan that showedthe presence of several cysts spread ubiquitously. The patient had a family history ofpneumothorax, for this reason we requested a genetic test that resulted in a heterozygouspoint mutation on exon 12 (c.1429 C > T) of FLCN gene. Despite the diagnosis ofBHD syndrome, the patient's clinical condition kept on, suggesting an underlying severeasthma and the blood tests we requested pointed out an high percentage of eosinophils.For this reason, we opted for the administration of benralizumab which resultedin an excellent asthma control and in an increased quality of life.

Key words
Birt-Hogg-Dubé; severe asthma; pneumothorax; benralizumab; eosinophilic asthma.

Cite this article as:
De Pace CC, Scioscia G, Lacedonia D, Fuso P, Lepore G, Palumbo MG, Foschino-Barbaro MP. A case of severe allergic eosinophilic asthma with nasal polyposis in a patient affected by Birt-Hogg-Dubé syndrome successfully treated with benralizumab. Eur Ann Allergy Clin Immunol 2021;53(6):277-9. doi:10.23822/EurAnnACI.1764-1489.222.

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