Lymphangioleiomyomatosis Clinical Presentation

Updated: Dec 22, 2019
  • Author: Joel Moss, MD, PhD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Presentation

History

Dyspnea and cough are common lymphangioleiomyomatosis (LAM) symptoms. [20] Less common symptoms (findings) are associated with pneumothorax and pneumoperitoneum, chylothorax and chylous ascites, and lymphedema.

Exacerbations of LAM have been reported to occur during pregnancy and menstruation, as well as with exogenous estrogen use. [21]

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Physical

The examination in lymphangioleiomyomatosis (LAM) is often normal.

Less common findings include the following:

  • Crackles

  • Wheezes

  • Pleural effusion

  • Pneumothorax

  • Ascites

Signs of tuberous sclerosis complex [22] may be present, and include the following:

  • Facial angiofibromas

  • Periungual fibromas

  • Hypomelanotic macules, ash-leaf spots

  • Shagreen patch - A cluster of hamartoma typically located on the lower back

  • Forehead plaque

  • Retinal hamartoma

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