Condition Neutrophil Abnormality Oral Manifestation
Specific granule deficiency (SGD) is a rare disease, which is probably autosomal recessive. It represents a failure to pack whole group of proteins (both specific and azurophilic granule protein) into granules. Deficiency of these components results in depressed respiratory burst activity, diminished ability to respond to chemotaxis and poor phagocytosis. Intraphagolysosomal killing is predictably sluggish. Severe periodontitis and ulceration.
Papillon-Lefevre syndrome is a rare autosomal recessive disorder. Reduced PMN motility and reduced PMN bactericidal activity have been reported. There is rapid generalized destruction of alveolar bone and early loss of deciduous and permanent dentition. After the loss of deciduous teeth, tissues heal rapidly and without sequel until the eruption of permanent dentition, when the process begins anew. Recently associated in affected individuals with a mutation in the Cathepsin C gene.
Table 2: Oral manifestations associated with neutrophil abnormalities.