Periodontitis As A Manifestation Of Systemic Diseases

Many systemic conditions assoc iated with or predisposing to periodontal attachment loss have defective numbers of neutrophils or defective neutrophil function «is a common finding. Ihis underscores the importance of the neutrophil in the protection ol the periodontium against infection. Severe periodontitis has been observed in individuals with primary neutrophil disorders including agranulocytosis, neutropenia. Chediak-Higashi syndrome. and lazy leukocyte syndrome In addition, more frequent and severe periodontitis has been observed In

Lazy Leukocyte Syndrome

Fig. 27-2 Dentition ot a 17 year-old boy with Papillon-Lefevre syndrome The missing teeth wt-ie exfoliated.

Down Syndrome Periodontitis

Fig. 27-3 Palms (A) and knees (B) of the paiient in Fig. 27 1. Note the hyperkeralotic scaly lesions.

individuals who exhibit secondary neutrophil impairment as seen in Down syndrome, Pa pi llon-l.efevre syndrome, and inflammatory bowel disease. The remainder <>l this chapter discusses some of these entities.

Papillon-Lefevre Syndrome

Papillon-Lefevre syndrome is characterized by hyperkera-totic skin lesions, severe destruction of the periodontium, and in some cases, calcification of the dura.' ' The cutaneous and periodontal changes usually appear together before the age of 4 years. I he skin lesions consist of hyperkeratosis and ichthyosis of localized areas on palms, soles, knees, and elbows digs. 27-2 and 27-3).

Periodontal involvement consists of early inflammatory changes that lead to bone loss and exfoliation of teeth. Primary teeth are lost by 5 or 6 years of age. The permanent dentition then erupts normally but within a few years the permanent teeth are lost owing to destructive periodontal disease. By the age of 15 years, patients are usually edentulous except for the third molars. These also are lost a few years after they erupt. Iboth extraction sites heal uneventfully.'* I he microscopic changes reported include marked chronic inflammation of the lateral wall of the pocket with a predominantly plasma cell infiltrate, considerable osteoclastic activity and apparent lack of osteoblastic activity, and an extremely thin cement urn/4 Bacterial flora studies of plaque in a case of Papillon-Lefevre syndrome revealed a similarity to bacterial flora in chronic periodontitis 1 Spirochete-rich zones in the apical portion of the pockets, as well as spirochete adherence to the cementurn and microcolony formation of Mycoplasma spp., have been reported in Papillon-Lefevre syndrome.20 < iram-negative cocci and rods appear at the apical border of plaque.4 No significant alterations have been found in peripheral blood lymphocytes and polymorphonuclear neutrophils (PMNs).tl

Papillon-Lefevre syndrome is inherited and appears to follow an autosomal recessive pattern. Parents are not affected, and both must carry the autosomal genes for the syndrome to appear in the offspring. It may occur in siblings; males and females are equally affected. The estimated frequency is 1 to 4 cases per I million individuals. Rare cases of adult onset of this syndrome, albeit with mild periodontal lesions, have also been described.3

Fig. 27-2 Dentition ot a 17 year-old boy with Papillon-Lefevre syndrome The missing teeth wt-ie exfoliated.

Fig. 27-3 Palms (A) and knees (B) of the paiient in Fig. 27 1. Note the hyperkeralotic scaly lesions.

Down Syndrome Periodontal Disease
Fig. 27-4 A 14-year-old patient with Down syndrome and severe periodontal destruction.

Down Syndrome

Down syndrome (mongolism, trisomy 21) is a congenital disease caused by a chromosomal abnormality and characterized by mental deficiency and growth retardation. I he prevalence of periodontal disease in Down syn-chome is high (occurring in almost 100% of patients younger than 30 years). Although plaque, calculus, and local irritants (e.g., diastemataf crowding of teeth, high frenum attachments, and malocclusion) are present and oral hygiene is poor, the severity of periodontal destruction exceeds that explainable by local factors alone.5,7,36 Periodontal disease in Down syndrome is characterized by formation of deep periodontal pockets associated with substantial plaque accumulation and moderate gingivitis ilig. 27-4). These findings are usually generalized,

Atariruti/inx Ulcerative Periodontitis. Refractory Petiihionlitis, and Periodontitis as a Manifestation of Systeniii Diseases ■ ( 11.AIM I K 27 407

although they tend to he more severe in the lower anterior region; marked recession is also sometimes seen in this region, apparently associated with high trenum attachment. The disease progresses rapidly. Acute necrotizing lesions are a frequent finding.

Two factors have been proposed to explain the high prevalence and increased severity of periodontal destruction associated with Down syndrome: a reduced resistance to infections because of poor circulation, especially in areas ol terminal vascularization such as the gingival tissue/*'10 and a delect in I-cell maturation and polymorphonuclear leukocyte chemotaxis.1 Increased numbers of P. intermedia have been reported in the mouths of children with Down syndrome.1"

Neutropenia

Destructive generalized periodontal lesions have been described in children with neutropenia, lor further information, see Chapter 14.

Chediak-Higashi Syndrome

Chediak-Higashi sy ndrome is rare and is charac terized by recurrent bacterial infections including rapidly destructive periodontitis. It is discussed in Chapters 10 and 14.

Hypophosphatasia

Hypophosphatasia is a rare familial skeletal disease characterized by rickets, poor cranial bone formation, crane-ostenosis, and premature loss of primary teeth, particularly the incisors. Patients have a low level of serum alkaline phosphatase, and phosphoethanolaminc is present in serum and urine.

Teeth are lost with no clinical evidence of gingival inflammation and show reduced cementum formation.-' In patients with minimal bone abnormalities, premature loss of deciduous teeth may be the only symptom of hypophosphatasia. In adolescents, this disease resembles localized juvenile periodontitis.H

Leukocyte Adhesion Deficiency

Cases of leukocyte adhesion deficiency (LAD) are rare and begin during or immediately alter eruption of the primary teeth. Extremely acute inflammation and proliferation of the gingival tissues with rapid destruction of bone are found. Profound defects in peripheral blood neutrophils and monocytes and an absence of neutrophils in the gingival tissues have been noted in patients with I \I)>S M; these patients also have frequent respiratory tract infections and sometimes otitis media. All primary teeth are affected, but the permanent dentition may not be affected,-'

Essentials of Human Physiology

Essentials of Human Physiology

This ebook provides an introductory explanation of the workings of the human body, with an effort to draw connections between the body systems and explain their interdependencies. A framework for the book is homeostasis and how the body maintains balance within each system. This is intended as a first introduction to physiology for a college-level course.

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