Necrotizing ulcerative stomatitis following bone marrow transplantation in a patient with chronic granulomatous disease: Case report.

Abstract

Background: Chronic granulomatous disease (CGD) is an inborn error of immunity characterized by life-threatening infections and inflammation. Allogeneic hematopoietic stem cell transplantation (alloHSCT) is potentially curative. Necrotizing periodontal diseases (NPDs) are rare and can be seen in immunocompromised/immunosuppressed individuals but have not been reported in patients with CGD. We report NPD in the form of necrotizing stomatitis (NS) in a patient transplanted for CGD.

Methods: A 12-year-old male presented with severe gingival pain. The patient's medical history was significant for X-linked CGD diagnosed at 4 years of age and a heterozygous RIPK1 mutation. The patient underwent alloHSCT from a mismatched unrelated donor. Intraoral examination on day +61 post-alloHSCT revealed multifocal gingival ulceration, which did not respond to a short course of prednisone and topical dexamethasone oral solution. The ulcers progressed to involve the lip mucosa and soft palate/tonsil (day +88).

Results: Biopsy from the soft palate/tonsillar area showed deep acute inflammation and abscesses consistent with the diagnosis of NS. The patient underwent deep gingival scaling on day +103 and received amoxicillin and metronidazole. The patient had complete resolution of the oral ulcers by day +125 but developed chronic graft-versus-host disease involving the oral mucosa on day +314 post-alloHSCT.

Conclusion: We report the first case of NS as a complication of alloHSCT in a patient transplanted for CGD which was successfully managed with antibiotic therapy, periodontal debridement, and discontinuation of potentially triggering agents. Our report demonstrates that NS/NPD may occur in post-alloHSCT setting.

Key points: Chronic granulomatous disease is an inherited multisystem autoinflammatory condition. Patients with chronic granulomatous disease may have oral manifestations including oral ulcerations and gingivitis/periodontitis. Necrotizing ulcerative stomatitis may arise in the post-hematopoietic stem cell transplantation setting.

Plain language summary: Chronic granulomatous disease (CGD) is a condition that impairs the immune system and can be managed with bone marrow transplantation. Necrotizing periodontal disease (NPD) are a group of rare conditions that affect the mouth and cause tissue death (necrosis) and present clinically as oral ulcers (mouth sores). In this article, we report NPD in a child who received bone marrow transplantation for his CGD. This report broadens the differential diagnosis of oral ulcers in the post-transplant setting.