Condition Neutrophil Abnormality Oral Manifestation
Agranulocytosis Almost complete absence of granulocytes or PMNs. Oral lesions constitute an important phase of clinical aspect. These appear as necrotizing ulcerations of oral mucosa, tonsils and pharynx. Gingiva and palate are involved. Lesion appears as a ragged necrotic ulcer covered by a gray or even black membrane. Patients often manifest excessive salivation.
Cyclic neutropenia A periodic or cyclic diminution in circulating PMNs as a result of bone marrow arrest, accompanied by mild clinical manifestations, which spontaneously regress only to recur subsequently in rhythmic pattern. Patients exhibit gingivitis, sometimes stomatitis with ulceration, which corresponds to the period of neutropenia. In children the repeated insult of an infection often leads to considerable loss of supporting bone around the teeth.
Leukocyte adhesion deficiency These patients mostly children have multiple defects in neutrophil and mononuclear phagocyte adhesion dependent functions, including chemotaxis and CR mediated phagocytosis Recurrent infections with pyogenic bacteria including severe periodontal disease.
Hypergammaglobulinemia (Job’s Syndrome), rare complex autosomal recessive disorder. Defects in chemotaxis of PMNs. Recurrent infections with opportunistic organisms viz; Staphylococcu aureus and Candida albicans.
Chediak Higashi syndrome (rare disease) often fatal in early life as a result of lymphoma-like terminal phase, hemorrhage or infection with autosomal recessive mode of inheritance. Structural defect where in transformation of the azurophilic and specific granules into giant bodies called “megabodies” is characteristic. Functional neutrophil defects include decreased chemotaxis, degranulation and microbicidal activity. Ulceration of oral mucosa, severe gingivitis, glossitis, periodontal disease, severe bone loss and recurrent pyogenic infection. Mutation in vesicle trafficking regulator gene LYST.
Table 1: Oral manifestations associated with neutrophil abnormalities.