PEDIATRIC DENTISTRY/Copyright © 1981 by The American Academyof Pedodontics/Vol. 3, No. 2 CAS Periodontal changesassociated with chronic idiopathic neutropenia KennethL. Kalkwarf, DD$, MS DennisP. Gutz, DDS,MS Abstract and respiratory tract. Severalcases 7-11,~,~.4~-57.4.s~have dis- cussed the response of the gingiva and periodontal This case report focuses on the periodontal status of a supporting apparatus during the neutrophil disorder. youngpatient with chronic idiopathic neutropenia. A four- year clinical courseis presented.Chronic idiopatin’c Severe gingivitis, oral ulcers and destruction of the neutropeniais a relatively rare blood dyscrasia alveolar crest have been consistently noted. characterized by a severe decrease in the numberof Suggested therapy for children exhibiting this circulating neutrophils. Youngindividuals exin’biting tins hematologic phenomena has included cysteine, ~ blood problemexperience rapid destruction of periodontal transfusions/ pyridoxine hydrochloride (vitamin B6), structures. A reviewof related literature reveals that the few ~.~ plasma transfusions, s transfusion with packed white discussions of dental symptomsavailable relate common cells, ~ non-specificu,~3 fever therapy/ splenectomy, findings: exaggeratedgingivitis, prematureexfoliation of the cortisone, 41,~.u testosterone, 5 and somatotropic hor- primary dentition and recurrent occurrence of aphthous- mone? Only antibiotic therapy has had predictable type lesions. While some mechanismsexplaining the lack of success in control of the systemic sequela.’s,~a.4~ Newer circulating neutrophils have been postulated, the exact methods of granulocyte transfusion appear to exhibit etiology of chronic idiopathic neutropeniais unknown. ~ Dentaltherapy for individuals exin’biting tins condition significant promise in future therapy regimes? shouMbe aimedat the reduction of local inflammationby The purpose of this paper is to present a case report careful and frequent removalof local irdtational factors. of a child exhibiting chronic neutropenia, discuss clas- Great care shouMbe employedduring dental therapy to sification of such neutropenias and review dental man- reducethe possibility of bacteremias. agement for children with such problems. Introduction MedicalHistory Neutropenia is characterized by a severe decrease in the number of circulating neutrophils in the peri- The patient, a slight, well nourished 31/~-year-old pheral blood vessels. Classically, neutropenia occurs in caucasian female, in no apparent distress, was referred adults, typically middle-aged women,and is a result of to the Periodontics Department, College of Dentistry, specific drug injection, radiation or severe infection; University of Nebraska Medical Center, for evaluation Several cases of infants and children exhibiting and treatment of her gingival condition. A medical severe neutropenias of questionable or idiopathic history revealed that initial problems were noted at origin have been reported in the literature. Due to age 21/~ weeks, when she developed an intense papular differences observed in onset, severity, symptomsand rash in the diaper area. A diagnosis of staphylococcal etiology, a number of names have been used to de- dermatitis was made and the patient was treated with scribe the clinical entities: infantile congenital agranu- ampicillin and oxacillin sodium. The rash cleared, but locytosis, ~ cyclic neutropenia, TM chronic benign recurred two weeks following discontinuance of the neutropenia, m~ reticular dysgenesia, 1~,~ congenital antibiotics. The patient also experienced numerous neutropenia,~*= infantile aneutrocytosis," chronic idio- early bouts of respiration difficulty and frequent ~ ~-s~ febrile episodes, with temperatures ranging from 101- pathic neutropenia and others. All children re- ° ported a history of persistent infections of the skin 103 F. Each illness was treated with antibiotic ther- apy. The clinical picture recurred with cessation of Accepted: December 2, 1980 each course of medication. PEDIATRICDENTISTRY 189 Vo|. 3, No. 2 The patient was evaluated at age three months and a diagnosis of hypogammaglobulinemia was made. She was treated with two injections of gammaglobu- lin. Multiple complete blood counts were completed during the course of her initial therapy. At no time was her granulocyte count above 3%. At age five months the patient was referred to the University of Nebraska Medical Center for a hema- tologic evaluation. Laboratory data obtained at that time was as follows: Hemaglobin: 11.5 Platelets: 552,000 WBC: 10,900 Urinalysis: normal Figure 1. Age three years, six months, mild plaque accumulation. A Differential: 76% Lymphocytes IgG: 630 granulomatous collar forms the gingival margin around each tooth. 20% Monocytes IgA: 45 Exaggerated gingival recession is evident, particularly in the ante- 3% Eosinophils IgM: 97 rior region. l%Basophils 9% Neutrophils A repeat blood count was comparable to the first. A bone marrow examination was reported to be consist- ent with congenital neutropenia. A diagnosis of con- genital neutropenia, specifically of the congenital infantile agranulocytosis form, was made. 3y,6m Following the diagnosis, the patient experienced numerous dermatologic infections that were treated with antibiotic therapy. At age two, severe otitis re- quired lancing of the typanic membranes. Ampicillin therapy was initiated at that time and continued in multiple two week courses. 4y, 6m Family History Outside of a family history of allergic disorders, no significant familiar characteristics could be identified. Hematologic evaluations of the patient's parents were within normal limits. Dental Findings Intital periodontal examination revealed swollen, edematous gingiva. A distinct granulomatous collar was evident at the cervical region of the teeth. It was crimson red in color and bled easily when manipulated 6y, 9m (Figure 1). No evidence of perioral or other lesions within the oral cavity or pharyngeal region was Figure 2: Serial bitewing radiographs. present. Gingival recession had occurred in the ante- The importance of plaque control was stressed to rior regions, resulting in exposed root surfaces. The the mother. She was instructed in disclosing and ginival tissue was very tender and the patient resisted cleaning her daughter's mouth with an extra soft attempts at gentle probing. No clinical evidence of ac- nylon brush, being careful to remove all traces of vis- tive carious lesions was detected. Moderate accumula- ible plaque. An emollient was prescribed to provide tions of plaque were present. The mother noted that pallative relief to local regions devoid of epithelium. A the patient frequently complained of a "sore mouth" thorough prophylaxis was performed and the patient and resisted attempts at toothbrushing. Radiographic was placed on a frequent maintenance regimen. The examination revealed exaggerated alveolar bone loss, patient's mother was told to call if any change in oral particularly in the anterior regions of the mouth (Fig- symptoms was noticed. ures 2 and 3). No radiographic evidence of active car- The patient was seen at one to three month inter- ies was found. vals over the next four years. At maintenance appoint- CHRONIC IDIOPATHIC NEUTROPENIA 190 Kalkwarf and Gutz Figure 4: Age four years, moderate plaque, gingival edema and swelling. Gingival architecture in the anterior regions is becoming 5y, 0 m quite distorted. Granulomatous collar is not as evident. 6y, Om Figure 5: Age four years, six months, moderate plaque accumula- tion. Recession is more pronounced in the anterior region. Drifting 6y ,9m of the mandibular anterior teeth is becoming noticeable. Figure 3: Serial anterior radiographs. ments, plaque control was evaluated and discussed with the patient and her parents. Gingival contour, color and consistency were evaluated. Gentle debride- ment and polishing were accomplished as required. Kodachromes were obtained at six-month intervals for documentation. Bitewing and anterior radiographs were obtained yearly to evaluate osseous changes (Fig- ures 2 and 3). During the four years of observation, the patient's plaque control varied from fair to poor. From conver- sations with her mother it was determined that gin- Figure 6: Age five years, mild plaque accumulation. Recession and gival inflammation periodically progressed to a point staining are more prominent. The mandibular incisors are extremely mobile and exhibit evidence of drifting. where use of even a soft toothbrush was very uncom- fortable. During these periods, the parents would not insist that cleaning be performed and massive manent dentition (Figures 5, 6, and 7). Clinical evi- amounts of bacterial plaque would accumulate, result- dence of active caries was not identified at any exami- nation. Soft tissue lesions, appearing to be aphthous- ing in increased gingival swelling and edema (Figure type ulcerations were occasionally present in the oral 4). The granulomatous collar remained present at each observation period, being more evident during some and perioral regions (Figure 9). Radiographic evalua- periods than others (Figures 1, 5 and 8). tions revealed progressing destruction of the alveolar Progressive gingival recession eventually resulted in bone about the primary dentition. This was especially advanced root exposure throughout the primary den- evident about the anterior teeth and in the furcation tition. This was especially evident in the anterior re- regions of the primary molars (Figures 2 and 3). gions of the mouth