Tuberous sclerosis associated lymphangioleiomyomatosis: A case report

Lymphangioleiomyomatosis (LAM) is a multisystem disorder that primarily affects the lung. Tuberous sclerosis complex (TSC) is characterized by multiple benign tumours of the skin, brain, eyes, heart, lung, liver, and kidney. LAM can be either sporadic (sporadic-LAM) or in association with Tuberous Sclerosis (TSC-LAM). Many clinical, radiologic, and pathologic features are shared between TSC and sporadic variants. We present a case admitted at The Indus Hospital Karachi with pneumothorax and multiple manifestations of TSC-LAM. Image A: Facial angiofibromas and hypopigmented ash leaf lesions seen.

Automated summary

Lymphangioleiomyomatosis (LAM) is a multisystem disorder primarily affecting the lung, while Tuberous sclerosis complex (TSC) is characterized by multiple benign tumors in various organs. LAM can occur either sporadically or in association with TSC. There are several shared features between TSC and sporadic LAM. We present a case of TSC-LAM with pneumothorax and multiple manifestations.