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Scholars Journal of Medical Case Reports | Volume-12 | Issue-06
Lymphangioleiomyomatosis Associated with Tuberous Sclerosis of Bourneville: Report of a Case and Review of the Literature
Z.Hanyn, H.Benjelloun, S.Alioua, N.Zaghba, K.Channoun, M.Abatourab, H.harraz, N. Yassine
Published: June 12, 2024 |
54
31
DOI: 10.36347/sjmcr.2024.v12i06.037
Pages: 1115-1120
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Abstract
Lymphangioleiomyomatosis (LAM) is a disease mainly affecting women, most often between the ages of 20 and 40, often associated with tuberous sclerosis complex (TSC). We report the case of a 31-year-old patient, treated for schizophrenia, who presented with acute respiratory failure. Chest imaging revealed the presence of multiple thin-walled cystic formations with well-defined contours, of diffuse distribution, extended to both pulmonary fields. The diagnosis of definite LAM was made based on the diagnostic criteria for LAM according to the ERS 2010 international guidelines associated with definite TSC was made based on the presence of two major criteria also according to the consensus conference on the diagnosis of TSC from 2012.The development was marked by the death of the patient the day following her hospitalization. Through this observation, we wish to shed light on a relatively rare disease whose diagnosis remains well codified and easy.