Griscelli Syndrome and Periodontal Therapy Approach: a Case Report

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Griscelli Syndrome (GS), a rare autosomal recessive genetic
disorder characterized with pigmentary dilution of the skin,
malfunctions of humoral and cellular immunity, was first described
by Claude Griscelli in 1978. In this report, periodontal therapy and
follow-up of a 10 year-old girl with GS type 2, who applied to our
clinic with complaints of gingival bleeding and redness, was
presented. The patient had silvery-grey coloured hair and eyebrow,
well-rounded nose radix and tip, and hemiparesthesia at the right
part of her body. Intraoral examination revealed pigmentation of
the lips, heavy accumulation of microbial dental plaque, hyperemia
and edema at gingiva. She was diagnosed with plaque-induced
gingivitis, and initial periodontal treatment including oral hygiene
instructions, scaling and polishing was performed under
recommended antibiotic prophylaxis. She was followed up with 1
month intervals. After 6 months, she attended to the clinic with
clinical signs of acute necrotizing ulcerative gingivitis and was
treated with diluted hydrogen peroxide and chlorhexidine
digluconate (0.2%) rinse. Due to the hyperactivity of T lymphocytes
and macrophages, intensive host response to dental plaque in
patients with GS can be seen. In conclusion, importance of oral
hygiene should be emphasized in establishing high level of oral
health and, maintenance of patients with GS should be carried out
with short intervals.


Griscelli syndrome type 2; nonsurgical periodontal debridement; acute necrotizing ulcerative gingivitis

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Griscelli C, Durandy A, Guy-Grand D, Daguillard F, Herzog C, Prunieras M: A syndrome associating partial albinisim and immunodeficiency. Am J Med 1978;65:691-702.

Pastural E, Barrat FJ, Dufourcq-Lagelouse R, Certain S, Sanal O, Jabado N, Seger R, Griscelli C, Fischer A, de Saint Basile G: Griscelli disease maps to chromosome 15q21 and is associated with mutations in the myosin-Va gene. Nat Genet 1997;16:289-292.

Wilson SM, Yip R, Swing DA, O’Sullivan TN, Zhang Y, Novak EK, Swank RT, Russell LB, Copeland NG, Jenkins NA: A mutation in Rab27a causes the vesicle transport defects observed in ashen mice. Proc Natl Acad Sci USA 2000;25:173-176.

Menasche G, Pastural E, Feldmann J, Certain S, Ersoy F, Dupuis S, Wulffraat N, Bianchi D, Fischer A, Le Deist F, de Saint Basile G: Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome. Nat Genet 2000;25:173-176.

Menasche G, Ho CH, Sanal O, Feldmann J, Tezcan I, Ersoy F, Houdusse A, Fischer A, de Saint Basile G: Griscelli syndrome restricted to hypopigmentation results from a melanophilin defect (GS3) or a MYO5A F-exon deletion (GS1). J Clin Invest 2003;112:450-456.

Kurugöl Z, Ozkınay F, Vardar F, Karaçalı S, Kütükçüler N, Deveci R, Ozkınay C: Griscelli syndrome: Report of a case and review of the literature. Pediatr Int 2001;43:298-301.

Kirzioglu Z, Altun AC: Griscelli syndrome: A case report of Reye’s syndrome and atopic dermatitis history. J Indian Soc Pedod Prev Dent 2008;26:118-120

Tezcan I, Sanal O, Ersoy F, Uçkan D, Kılıç S, Metin A, Cetin M, Akın R, Oner C, Tuncer A: Successful bone marrow transplantation in a case of Griscelli disease which presented in accelerated phase with neurological involvements. Bone Marrow Transplant 1999;24:931-933.

Arico M, Zecca M, Santoro N, Caselli D, Maccario R, Danesino C, de Saint Basile G, Locatelli F: Successful treatment of Griscelli syndrome with unrelated donor allogeneic hematopoietic stem cell transplantation. Bone Marrow Transplant 2002;29:995-998.