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Oral features of a family with benign familial neutropenia
Journal of the American Academy of Dermatology Volume 30, Number 5, Part 2
epidermis with alteration of the collagen bundles without any changes in elastic tissue. 6 These findings differ in patients with LFE. We report the first case of LFE in a young black man. The history of a similar disease in his late father should promptfamilial studies in future patients with LFE.
REFERENCES 1. Burket JM, Zelickson A, Padilla RS. Linear focal elastosis (elastotic striae). JAM ACAD DERMATOL 1989;20:633-6.
Porter et at. 2. Hagari Y, Mihara M, Shimao S. Linear focal elastosis: an ultrastructural study. Arch Dermatol 1991;127:1365-8. 3. White GM. Linear focal elastosis: A degenerative or regenerative process of striae distensae? J AM ACAD DERMATOL 1992;27:468. 4. Hashimoto K, DiBella RJ. Electron microscopic studies of normal and abnormal elastic fibers of skin. J Invest Dermatol 1967;48:405-23. 5. Lever WF, Schaumburg-Lever G. Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990;312. 6. Breathnach AS. Ultrastructure of epidermis and dermis in striae atrophicae. In: Moretti GR, Rebora A, eds. Striae distensae. Milan: Brocades, 1976:35.
Oral features of a family with benign familial neutropenia S. R. Porter, BSc, PhD, MBChB, FDS, RCS, RCSE,a J. Luker, PhD, FDS, RCSE,a C. Scully, MD, MDS, PhD, FDSRCPS, FFDRCSI, FDSRCS, FRCPath,a and A. Oakhill, MD, FRCp b Bristol, United Kingdom The oral features of three members of a family with familial benign neutropenia (a mother and two children) are detailed. Prepubertal periodontitis, oral ulceration, and angular stomatitis were the principal features. (J AM ACAD DERMATOL 1994;30:877-80.) Neutropenias are characterized by a transient or chronic decrease in the peripheral blood neutrophil count to 2 or more standard deviations below the normal mean. I Affected patients can have a spectrum of clinical manifestations ranging from health to recurrent, possibly life-threatening bacterial infections. 1 Mucosal ulceration and increased susceptibility to gingivitis and periodontitis are the main oral manifestations of neutropenias. Although numerous reports have been made of the oral aspects of cyclic neutropenia,2-9 little information is available about the oral features of chronic neutropenias. This report details the orofacial features of a family with benign familial neutropenia.
From the Centre for the Study of Oral Disease, University Department of Oral Medicine, Pathology, and Microbiology, Bristol Dental School and Hospital,' and the Institute for Child Health, Bristol Children's HospitaLb Reprint requests: S. R. Porler, Centre for the Study of Oral Disease, Bristol Dental School and Hospital, Lower Maudlin Street, Bristol, BSI 2LY, United Kingdom. Copyright ® 1994 by the American Academy or Dermatology, Inc. 0190-9622/94 $3.00 + 0
16/4/48767
CASE REPORTS
Case 1 A 3V2-year-old girl had severe gingivitis and stained upper incisors. Although she had developed normally she had a history of recurrent furuncles, otitis media, eczema, and superficial oral ulceration. The girl's mother was known to have benign familial neutropenia. Laboratory studies revealed a slightly low hemoglobin level (9.5 gmj dl) and a normal platelet count and white blood cell count (5 X 109jL), but she had neutropenia (0.15 X 109jL) with monocytosis (0.9 X 109 /L), lymphocytosis (3.3 X 109jL), and eosinophilia (0.9 X 109 jL). A bone marrow aspirate revealed normal erythropoiesis but little granulopoiesis beyond the metamyelocyte stage. Levels of serum IgA, IgG, and IgM were raised, probably as a result of chronic infection, and there were elevated titers of antibodies to herpes simplex virus, varicella zoster virus, and cytomegalovirus. The child had a normal female karyotype. A diagnosis of benign familial neutropenia was made and she was given an iron supplement and 1% hydrocortisone cream for the mild eczema, Examination revealed that the child was above the 50th percentile for height, weight, and normal intelligence. She had bilateral blepharitis (Fig. 1) but no lymphadenopathy. The patient had no oral mucosal lesions, but oral hy-
877
878 Porter et al.
Fig. 1. Case 1. Blepharitis and conjunctivitis at 9 years of age.
Journal of the American Academy of Dermatology May 1994
Fig. 2. Case 1. Gingivitis at 9 years of age.
Fig. 3. Case 1. Panoral radiograph at 6 years of age. Note the severe periodontal destruction. giene was poor; supragingival plaque deposits werepresent in most sites, in addition to severe acute marginal gingivitis (Fig. 2) and gingival recession. Three years later she had generalized severe acute and chronic gingivitis and several deep periodontal pockets. Panoral radiographs revealed severe periodontal bone loss around several teeth (Fig. 3). She had not had any specific therapy for neutropenia, but she was receiving frequent courses of antibiotics for recurrent blepharitis and conjunctivitis.
Case 2 A 4-year-old child, the brother of the patient in case 1, had recurrent oral ulcerations since birth. He had also had bacterial pneumonia at age 5 weeks, severe croup at age 10 months, facial cellulitis at age 3 years, and severe tonsillitis at age 4 years. One month later oral thrush developed. Approximately 4 months later he developed Pseudomonas cellulitis of the shaft of the penis that re-
sponded to oral ciprofloxacin therapy. He has had a persistently low neutrophil count that failed to improve with granulocyte colony-stimulating factor therapy. Examination revealed that his upper lip was swollen and everted, particularly on the left side, as a result of a large, superficial, irregular ulcer of the labial mucosa. Intraorally there was a second, smaller superficial ulcer on the lower labial mucosa (Fig. 4). He also had generalized supragingival deposits of plaque and generalized acute marginal gingivitis. Periodontitis was associated with the upper anterior teeth. A diagnosis of oral ulceration and prepubertal periodontitis resulting from benign familial neutropenia was made.
Case 3 The 33-year-old mother of cases 1 and 2 had recently had ulceration and swelling of the right angle of mouth. The patient was known to have benign familial neutropenia and had associated recurrent furunculosis until she
Journal of the American Academy of Dermatology Volume 30, Number 5, Part 2
Porter et
at.
879
Table I. Causes of neutropenia Decreased neutrophil production Primary immunodeficiencies Severe infantile genetic neutropenia (Kostmann's syndrome) Reticular dysgenesis (with congenital aleukocytosis) Neutropenia with agammaglobulinemia Transcobalamin II deficiency Neutropenia with dysgranulopoiesis Neutropenia with exocrine pancreatic dysfunction (Schwachman-Diamond syndrome) Neutropenia with disordered cellular immunity (cartilage-hair hypoplasia) Neutropenia in Chediak-Higashi syndrome Cyclic neutropenia Chronic neutropenia(s) Autosomal dominant Autosomal recessive Idiopathic Drug-induced bone marrow suppression Nutritional deficiency, e.g., vitamin B12, folate, copper Neutropenia caused by defective neutrophil movement Myelokithesis Lazy leukocyte syndrome Neutropenia caused by accelerated neutrophil destruction Autoimmune (isoimmune) neonatal neutropenia Autoimmune neutropenia, e.g., systemic lupus erythematosus, rheumatoid arthritis, Sjogren's syndrome, Felty's syndrome, Hodgkin's lymphoma, chronic active hepatitis, and pure white cell aplasia Neutropenia caused by marginalization of neutrophils, e.g., pseudoneutropenia caused by passive trapping of neutrophils in the spleen in sarcoidosis, lymphoma, tuberculosis, malaria, kala-azar, and Gaucher's disease
was approximately 16 years of age. The patient reported that her permanent teeth had rapidly become mobile and had been extracted by the time she was 14 years of age. There was no family history of early loss of teeth or parental consanguinity. Examination revealed scarring in the left infraorbital area and left calf as a result of previous skin infection. She had right angular stomatitis and was edentulous but had no oral mucosal lesions.
DISCUSSION
Neutropenia increases susceptibility to infections, particularly bacterial infections of the skin and mucosae, and the risk of infection is more likely
Fig. 4. Case 2. Labial ulcerations at 4 years of age.
when the neutrophil count falls below 0.5 X 109/ mm3. 10• 11 The main causes of neutropenia are outlined in Table 1. Drug-induced bone marrow suppression and autoimmune neutropenia are the most common causes. Primary chronic neutropenias are uncommon; there is some confusion in classification regarding these neutropenias, partly because their molecular basis is often unknown and because neutropenias are subclassified differently according to the presence or absence of severe systemic manifestations.10, 11 "Benign" familial neutropenia is an autosomal dominant disorder 12 that is possibly more common in blacks and Yemenite Jews 12-16 and is characterized by constant neutropenia with no anemia or thrombocytopenia but possible monocytosis and eosinophilia. 1 Affected patients have variable clinical features: patients with neutrophil counts greater than 0.5 X 109 jL often have no symptoms, but patients with lower counts can have recurrent or severe infections, particularly of the skin and respiratory tract. In contrast, gastrointestinal and genitourinary infections are uncommon and infections of the bone and central nervous system are rare. 17. 18 The bone marrow of affected patients is generally normocellular; however, although early neutrophil precursors are quantitatively and morphologically normal, there is a reduction in the number of more mature neutrophilic forms. The degree of reduction in peripheral blood neutrophils is usually proportional to the reduction in marrow precursors. In vitro neutrophil colony formation is usually but not always 5 normal, and pseudoneutropenia caused by
Journal of the American Academy of Dermatology May 1994
880 Porter et al. increased marginalization is unlikely. Patients with benign familial neutropenia have a normal bone marrow response to hydrocortisone. 19, 20 Patients with benign familial neutropenia generally have a good prognosis and only require prompt treatment of infections. However, patients with particularly severe neutropenias can benefit from intermittent high-dose corticosteroid therapy. I, 18 There are few reports of the oral manifestations of chronic neutropenia,21-26 and the oral aspects of familial benign neutropenia have rarely been detailedp-29 As with our three patients, it has previously been found that aphthous-like oral ulceration and increased susceptibility to gingivitis and periodontitis are the main oral problems; indeed, these problems can be the only manifestations of benign familial neutropenia. 28 In addition, although most patients have only intermittent episodes of systemic infections, oral ulceration can be a persistent problem. 28 In contrast, cyclic neutropenia tends to cause episodic rather than persistent oral ulceration, although it can still cause rapid periodontal destruction. 2-9 There are no specific oral hygiene measures for patients with neutropenias; however, all patients should be given frequent professional hygiene therapy and encouraged to maintain a scrupulous standard of oral hygiene. Few data are available concerning the periodontal microbiologic features associated with neutropenia; therefore, it is unclear whether topical or systemic antibiotic therapy is also indicated. REFERENCES 1. Webster ADB, Asherson G L. Primary neutrophil defects. In: Diagnosis and treatment of immunodeficiency disorders. Oxford: Blackwell Scientific Publications, 1980:298301. 2. Cohen W, Morris A. Periodontal manifestations of cyclic neutropenia. J Periodonto11961;32:159-68. 3. Cohen L. Recurrent oral ulceration and cutaneous infections associated with cyclical neutropenia. Dent Pract 1966;16:97-8. 4. Degnan E, Perlow A. Infected oral lesions of cyclic neutropenia. J Oral Med 1973;28:29-31. 5. Gates GF. Chronic neutropenia presenting with oral lesions. Oral Surg Oral Med Oral Pathol1969;27:563-7. 6. Rylander H, Ericsson I. Manifestations and treatment of periodontal disease in a patient suffering from cyclic neutropenia. J Clin Periodontol 1981;8:77-81. 7. PrichardJF, Ferguson DM, Windmiller J, et a1. Prepubertal periodontitis affecting the deciduous and permanent
8. 9. 10.
11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29.
dentition in a patient with cyclic neutropenia: a case report and discussion. J Periodontol 1984;55:114-22; Scully C, McFadyen EE, Campbell A. Orofacial manifestations in cyclic neutropenia. Br J Oral Surg 1985;70:9610l. Levine S. Chronic familial neutropenia, with marked periodontallesions: report of a case. Oral Surg Oral Med Oral Pathol1959;12:31O-4. Lichtman MA. Classification and clinical manifestations of neutrophil disorders. In: Williams WJ, Bentler E, Erslev AJ,et aI., eds. Haematology. New York: McGraw Hill, 1990:802-6. Dale DC. Neutropenia. In: Williams W J, Bentler E, Erslev AJ, et aI., eds. Haematology. New York: McGraw Hill, 1990:807-16. Shoenfeld Y, WeingergerA, Avishar R, et a1. Familial 1eukopenia among Yemenite Jews. Isr J Med Sci 1978; 14:1271-4. Shaper AG, Lewis P. Genetic neutropenia in people of African origin. Lancet 1971;2:1021-3. Rippey J1. Leukopenia in West Indians and Africans. Lancet 1967;2:44. Davis LR. Leukopenia in West Indians and Africans. Lancet 1967;2:213. Mintz M, Sachs 1. Normal granulocyte colony forming cells in the bone marrow of Yemenite Jews with genetic neutropenia. Blood 1973;41:745-52. Pincus SH, Boxer LA, Stossel TP. Chronic neutropenia in childhood. Am J Med 1976;61:849-61. Dale DC, DuPont Guerry IV, Wewerka JR, et a1. Chronic neutropenia. Medicine (Baltimore) 1979;58:128-44. Senn JS, Messner HA, Stanley ER. Analysis of interacting cell populations in cultures of marrow from patients with neutropenia. Blood 1974;44:33-40. Shoenfeld Y, Modan M, Berliner S, et a1. The mechanism of benign hereditary neutropenia. Arch Intern Med 1982; 142:797-9. Andrews RG, Benjamin S, Shore N, et ai. Chronic benign neutropenia of childhood with associated oral manifestations. Oral Surg Oral Med Oral Patho11965;20:719-25. Gates GF. Chronic neutropenia presenting with oral lesions. Oral Surg Oral Med Oral Pathol1969;27:563-7. Kyle RA, Linman JW. Gingivitis and chronic idiopathic neutropenia: report of two cases. Mayo Clin Proc 1970; 45:494-504. Mishkin DJ, Akers JO, Darby CP. Congenitalneutropenia: report of a case and biorationale for dental management. Oral Surg Oral Med Oral PathoI1976;42:738-45. Reichart PA, Dornow H. Gingivo-periodontal manifestations in chronic benign neutropenia. J Clin Periodontol 1978;5:74-80. Baehni PC, Payot P, Tsai C-C, Cimasoni G. Periodontal status associated with chronic neutropenia. J Clin PeriodontoI1983;10:222-30. Deasey MJ, Vogel RI, Macedo-Sobrinho B, et a1. Familial benign chronic neutropenia associated with periodontal disease. J Periodontol 1980;51 :206-10. Vann WF, Oldenburg TR. Atypical hereditary neutropenia: case reports oftwo siblings. J Dent Child 1976;43:265-9. Stabholz A, Soskolne V, Machtei E, et ai. Effect of benign familial neutropenia on the periodontium of Yemenite Jews. J Periodontal 1990;61:51-4.
epidermis with alteration of the collagen bundles without any changes in elastic tissue. 6 These findings differ in patients with LFE. We report the first case of LFE in a young black man. The history of a similar disease in his late father should promptfamilial studies in future patients with LFE.
REFERENCES 1. Burket JM, Zelickson A, Padilla RS. Linear focal elastosis (elastotic striae). JAM ACAD DERMATOL 1989;20:633-6.
Porter et at. 2. Hagari Y, Mihara M, Shimao S. Linear focal elastosis: an ultrastructural study. Arch Dermatol 1991;127:1365-8. 3. White GM. Linear focal elastosis: A degenerative or regenerative process of striae distensae? J AM ACAD DERMATOL 1992;27:468. 4. Hashimoto K, DiBella RJ. Electron microscopic studies of normal and abnormal elastic fibers of skin. J Invest Dermatol 1967;48:405-23. 5. Lever WF, Schaumburg-Lever G. Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990;312. 6. Breathnach AS. Ultrastructure of epidermis and dermis in striae atrophicae. In: Moretti GR, Rebora A, eds. Striae distensae. Milan: Brocades, 1976:35.
Oral features of a family with benign familial neutropenia S. R. Porter, BSc, PhD, MBChB, FDS, RCS, RCSE,a J. Luker, PhD, FDS, RCSE,a C. Scully, MD, MDS, PhD, FDSRCPS, FFDRCSI, FDSRCS, FRCPath,a and A. Oakhill, MD, FRCp b Bristol, United Kingdom The oral features of three members of a family with familial benign neutropenia (a mother and two children) are detailed. Prepubertal periodontitis, oral ulceration, and angular stomatitis were the principal features. (J AM ACAD DERMATOL 1994;30:877-80.) Neutropenias are characterized by a transient or chronic decrease in the peripheral blood neutrophil count to 2 or more standard deviations below the normal mean. I Affected patients can have a spectrum of clinical manifestations ranging from health to recurrent, possibly life-threatening bacterial infections. 1 Mucosal ulceration and increased susceptibility to gingivitis and periodontitis are the main oral manifestations of neutropenias. Although numerous reports have been made of the oral aspects of cyclic neutropenia,2-9 little information is available about the oral features of chronic neutropenias. This report details the orofacial features of a family with benign familial neutropenia.
From the Centre for the Study of Oral Disease, University Department of Oral Medicine, Pathology, and Microbiology, Bristol Dental School and Hospital,' and the Institute for Child Health, Bristol Children's HospitaLb Reprint requests: S. R. Porler, Centre for the Study of Oral Disease, Bristol Dental School and Hospital, Lower Maudlin Street, Bristol, BSI 2LY, United Kingdom. Copyright ® 1994 by the American Academy or Dermatology, Inc. 0190-9622/94 $3.00 + 0
16/4/48767
CASE REPORTS
Case 1 A 3V2-year-old girl had severe gingivitis and stained upper incisors. Although she had developed normally she had a history of recurrent furuncles, otitis media, eczema, and superficial oral ulceration. The girl's mother was known to have benign familial neutropenia. Laboratory studies revealed a slightly low hemoglobin level (9.5 gmj dl) and a normal platelet count and white blood cell count (5 X 109jL), but she had neutropenia (0.15 X 109jL) with monocytosis (0.9 X 109 /L), lymphocytosis (3.3 X 109jL), and eosinophilia (0.9 X 109 jL). A bone marrow aspirate revealed normal erythropoiesis but little granulopoiesis beyond the metamyelocyte stage. Levels of serum IgA, IgG, and IgM were raised, probably as a result of chronic infection, and there were elevated titers of antibodies to herpes simplex virus, varicella zoster virus, and cytomegalovirus. The child had a normal female karyotype. A diagnosis of benign familial neutropenia was made and she was given an iron supplement and 1% hydrocortisone cream for the mild eczema, Examination revealed that the child was above the 50th percentile for height, weight, and normal intelligence. She had bilateral blepharitis (Fig. 1) but no lymphadenopathy. The patient had no oral mucosal lesions, but oral hy-
877
878 Porter et al.
Fig. 1. Case 1. Blepharitis and conjunctivitis at 9 years of age.
Journal of the American Academy of Dermatology May 1994
Fig. 2. Case 1. Gingivitis at 9 years of age.
Fig. 3. Case 1. Panoral radiograph at 6 years of age. Note the severe periodontal destruction. giene was poor; supragingival plaque deposits werepresent in most sites, in addition to severe acute marginal gingivitis (Fig. 2) and gingival recession. Three years later she had generalized severe acute and chronic gingivitis and several deep periodontal pockets. Panoral radiographs revealed severe periodontal bone loss around several teeth (Fig. 3). She had not had any specific therapy for neutropenia, but she was receiving frequent courses of antibiotics for recurrent blepharitis and conjunctivitis.
Case 2 A 4-year-old child, the brother of the patient in case 1, had recurrent oral ulcerations since birth. He had also had bacterial pneumonia at age 5 weeks, severe croup at age 10 months, facial cellulitis at age 3 years, and severe tonsillitis at age 4 years. One month later oral thrush developed. Approximately 4 months later he developed Pseudomonas cellulitis of the shaft of the penis that re-
sponded to oral ciprofloxacin therapy. He has had a persistently low neutrophil count that failed to improve with granulocyte colony-stimulating factor therapy. Examination revealed that his upper lip was swollen and everted, particularly on the left side, as a result of a large, superficial, irregular ulcer of the labial mucosa. Intraorally there was a second, smaller superficial ulcer on the lower labial mucosa (Fig. 4). He also had generalized supragingival deposits of plaque and generalized acute marginal gingivitis. Periodontitis was associated with the upper anterior teeth. A diagnosis of oral ulceration and prepubertal periodontitis resulting from benign familial neutropenia was made.
Case 3 The 33-year-old mother of cases 1 and 2 had recently had ulceration and swelling of the right angle of mouth. The patient was known to have benign familial neutropenia and had associated recurrent furunculosis until she
Journal of the American Academy of Dermatology Volume 30, Number 5, Part 2
Porter et
at.
879
Table I. Causes of neutropenia Decreased neutrophil production Primary immunodeficiencies Severe infantile genetic neutropenia (Kostmann's syndrome) Reticular dysgenesis (with congenital aleukocytosis) Neutropenia with agammaglobulinemia Transcobalamin II deficiency Neutropenia with dysgranulopoiesis Neutropenia with exocrine pancreatic dysfunction (Schwachman-Diamond syndrome) Neutropenia with disordered cellular immunity (cartilage-hair hypoplasia) Neutropenia in Chediak-Higashi syndrome Cyclic neutropenia Chronic neutropenia(s) Autosomal dominant Autosomal recessive Idiopathic Drug-induced bone marrow suppression Nutritional deficiency, e.g., vitamin B12, folate, copper Neutropenia caused by defective neutrophil movement Myelokithesis Lazy leukocyte syndrome Neutropenia caused by accelerated neutrophil destruction Autoimmune (isoimmune) neonatal neutropenia Autoimmune neutropenia, e.g., systemic lupus erythematosus, rheumatoid arthritis, Sjogren's syndrome, Felty's syndrome, Hodgkin's lymphoma, chronic active hepatitis, and pure white cell aplasia Neutropenia caused by marginalization of neutrophils, e.g., pseudoneutropenia caused by passive trapping of neutrophils in the spleen in sarcoidosis, lymphoma, tuberculosis, malaria, kala-azar, and Gaucher's disease
was approximately 16 years of age. The patient reported that her permanent teeth had rapidly become mobile and had been extracted by the time she was 14 years of age. There was no family history of early loss of teeth or parental consanguinity. Examination revealed scarring in the left infraorbital area and left calf as a result of previous skin infection. She had right angular stomatitis and was edentulous but had no oral mucosal lesions.
DISCUSSION
Neutropenia increases susceptibility to infections, particularly bacterial infections of the skin and mucosae, and the risk of infection is more likely
Fig. 4. Case 2. Labial ulcerations at 4 years of age.
when the neutrophil count falls below 0.5 X 109/ mm3. 10• 11 The main causes of neutropenia are outlined in Table 1. Drug-induced bone marrow suppression and autoimmune neutropenia are the most common causes. Primary chronic neutropenias are uncommon; there is some confusion in classification regarding these neutropenias, partly because their molecular basis is often unknown and because neutropenias are subclassified differently according to the presence or absence of severe systemic manifestations.10, 11 "Benign" familial neutropenia is an autosomal dominant disorder 12 that is possibly more common in blacks and Yemenite Jews 12-16 and is characterized by constant neutropenia with no anemia or thrombocytopenia but possible monocytosis and eosinophilia. 1 Affected patients have variable clinical features: patients with neutrophil counts greater than 0.5 X 109 jL often have no symptoms, but patients with lower counts can have recurrent or severe infections, particularly of the skin and respiratory tract. In contrast, gastrointestinal and genitourinary infections are uncommon and infections of the bone and central nervous system are rare. 17. 18 The bone marrow of affected patients is generally normocellular; however, although early neutrophil precursors are quantitatively and morphologically normal, there is a reduction in the number of more mature neutrophilic forms. The degree of reduction in peripheral blood neutrophils is usually proportional to the reduction in marrow precursors. In vitro neutrophil colony formation is usually but not always 5 normal, and pseudoneutropenia caused by
Journal of the American Academy of Dermatology May 1994
880 Porter et al. increased marginalization is unlikely. Patients with benign familial neutropenia have a normal bone marrow response to hydrocortisone. 19, 20 Patients with benign familial neutropenia generally have a good prognosis and only require prompt treatment of infections. However, patients with particularly severe neutropenias can benefit from intermittent high-dose corticosteroid therapy. I, 18 There are few reports of the oral manifestations of chronic neutropenia,21-26 and the oral aspects of familial benign neutropenia have rarely been detailedp-29 As with our three patients, it has previously been found that aphthous-like oral ulceration and increased susceptibility to gingivitis and periodontitis are the main oral problems; indeed, these problems can be the only manifestations of benign familial neutropenia. 28 In addition, although most patients have only intermittent episodes of systemic infections, oral ulceration can be a persistent problem. 28 In contrast, cyclic neutropenia tends to cause episodic rather than persistent oral ulceration, although it can still cause rapid periodontal destruction. 2-9 There are no specific oral hygiene measures for patients with neutropenias; however, all patients should be given frequent professional hygiene therapy and encouraged to maintain a scrupulous standard of oral hygiene. Few data are available concerning the periodontal microbiologic features associated with neutropenia; therefore, it is unclear whether topical or systemic antibiotic therapy is also indicated. REFERENCES 1. Webster ADB, Asherson G L. Primary neutrophil defects. In: Diagnosis and treatment of immunodeficiency disorders. Oxford: Blackwell Scientific Publications, 1980:298301. 2. Cohen W, Morris A. Periodontal manifestations of cyclic neutropenia. J Periodonto11961;32:159-68. 3. Cohen L. Recurrent oral ulceration and cutaneous infections associated with cyclical neutropenia. Dent Pract 1966;16:97-8. 4. Degnan E, Perlow A. Infected oral lesions of cyclic neutropenia. J Oral Med 1973;28:29-31. 5. Gates GF. Chronic neutropenia presenting with oral lesions. Oral Surg Oral Med Oral Pathol1969;27:563-7. 6. Rylander H, Ericsson I. Manifestations and treatment of periodontal disease in a patient suffering from cyclic neutropenia. J Clin Periodontol 1981;8:77-81. 7. PrichardJF, Ferguson DM, Windmiller J, et a1. Prepubertal periodontitis affecting the deciduous and permanent
8. 9. 10.
11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29.
dentition in a patient with cyclic neutropenia: a case report and discussion. J Periodontol 1984;55:114-22; Scully C, McFadyen EE, Campbell A. Orofacial manifestations in cyclic neutropenia. Br J Oral Surg 1985;70:9610l. Levine S. Chronic familial neutropenia, with marked periodontallesions: report of a case. Oral Surg Oral Med Oral Pathol1959;12:31O-4. Lichtman MA. Classification and clinical manifestations of neutrophil disorders. In: Williams WJ, Bentler E, Erslev AJ,et aI., eds. Haematology. New York: McGraw Hill, 1990:802-6. Dale DC. Neutropenia. In: Williams W J, Bentler E, Erslev AJ, et aI., eds. Haematology. New York: McGraw Hill, 1990:807-16. Shoenfeld Y, WeingergerA, Avishar R, et a1. Familial 1eukopenia among Yemenite Jews. Isr J Med Sci 1978; 14:1271-4. Shaper AG, Lewis P. Genetic neutropenia in people of African origin. Lancet 1971;2:1021-3. Rippey J1. Leukopenia in West Indians and Africans. Lancet 1967;2:44. Davis LR. Leukopenia in West Indians and Africans. Lancet 1967;2:213. Mintz M, Sachs 1. Normal granulocyte colony forming cells in the bone marrow of Yemenite Jews with genetic neutropenia. Blood 1973;41:745-52. Pincus SH, Boxer LA, Stossel TP. Chronic neutropenia in childhood. Am J Med 1976;61:849-61. Dale DC, DuPont Guerry IV, Wewerka JR, et a1. Chronic neutropenia. Medicine (Baltimore) 1979;58:128-44. Senn JS, Messner HA, Stanley ER. Analysis of interacting cell populations in cultures of marrow from patients with neutropenia. Blood 1974;44:33-40. Shoenfeld Y, Modan M, Berliner S, et a1. The mechanism of benign hereditary neutropenia. Arch Intern Med 1982; 142:797-9. Andrews RG, Benjamin S, Shore N, et ai. Chronic benign neutropenia of childhood with associated oral manifestations. Oral Surg Oral Med Oral Patho11965;20:719-25. Gates GF. Chronic neutropenia presenting with oral lesions. Oral Surg Oral Med Oral Pathol1969;27:563-7. Kyle RA, Linman JW. Gingivitis and chronic idiopathic neutropenia: report of two cases. Mayo Clin Proc 1970; 45:494-504. Mishkin DJ, Akers JO, Darby CP. Congenitalneutropenia: report of a case and biorationale for dental management. Oral Surg Oral Med Oral PathoI1976;42:738-45. Reichart PA, Dornow H. Gingivo-periodontal manifestations in chronic benign neutropenia. J Clin Periodontol 1978;5:74-80. Baehni PC, Payot P, Tsai C-C, Cimasoni G. Periodontal status associated with chronic neutropenia. J Clin PeriodontoI1983;10:222-30. Deasey MJ, Vogel RI, Macedo-Sobrinho B, et a1. Familial benign chronic neutropenia associated with periodontal disease. J Periodontol 1980;51 :206-10. Vann WF, Oldenburg TR. Atypical hereditary neutropenia: case reports oftwo siblings. J Dent Child 1976;43:265-9. Stabholz A, Soskolne V, Machtei E, et ai. Effect of benign familial neutropenia on the periodontium of Yemenite Jews. J Periodontal 1990;61:51-4.