Abstract
Papillion Lefevre syndrome (PLS) is a rare disease associated with skin lesions, severe destruction of periodontium and in some cases, calcification of dura. Skin lesions consist of hyperkeratosis and icthyosis of localized areas on palms, soles, knees and elbows, multiple functional neutrophil defects, including myeloperoxidase deficiency as well as defective chemotaxis and phagocytosis.
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