Birt-Hogg-Dube syndrome

ICD-10: Q87MeSH: D058249ORPHA: 122

Overview

human autosomal dominant genetic disorder that can cause susceptibility to kidney cancer, renal and pulmonary cysts

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Birt-Hogg-Dube syndrome, sourced from HPO and Orphanet clinical annotations.

Skin tagsFibrofolliculomaSpontaneous pneumothoraxMultiple pulmonary cystsRepeated pneumothoracesRenal cystMultiple lipomasPneumothoraxNeoplasmMedullary thyroid carcinomaParathyroid adenomaRenal cell carcinomaAngiofibromasCutaneous melanomaThyroid noduleSalivary gland oncocytomaTrichodiscomaEpidermoid cystCollagenomaThyroid cystOral mucosal papule

Classification & Codes

ICD-10 Code

Q87

MeSH Code

D058249

Orphanet Code

ORPHA:122
Birt-Hogg-Dube syndrome
ICD-10Q87
MeSHD058249
OrphanetORPHA:122
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO