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MAC & Bronchiectasis | Last Active: 2 days ago | Replies (143)
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Replies to "I have heard via the Lung Matters Facebook page that some (1 or 2) people on..."
I must have read the same post. Someone commented (and my pulmonologist confirmed) that it may be have been due to Papillon–Lefèvre syndrome (PLS) is a very rare inherited disorder caused by mutations in the CTSC gene.
It is characterized by:
Thickened, scaly skin on the palms and soles (palmoplantar keratoderma)
Severe early-onset gum disease (periodontitis)
Premature loss of both baby and adult teeth
It usually begins in early childhood and is linked to a deficiency of the enzyme cathepsin C (DPP1).
When taking Brinsupri regular dental checks are imperative, every 3-6 months.