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Characterization of orofacial features in sclerodermatous chronic graft-versus-host disease
Amal Bajonaid1, Praveen Kumar Guntaka2, Matthew Harper3,4
1College of Dentistry, Jazan University, Jazan, Saudi Arabia.
Insights
Sclerodermatous chronic graft-versus-host disease (cGVHD) can affect the face and mouth, causing fibrosis and limited opening. Management strategies can improve function and reduce symptoms after allogeneic hematopoietic cell transplantation (alloHCT).
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Chronic graft-versus-host disease (cGVHD) is a significant complication following allogeneic hematopoietic cell transplantation (alloHCT).
- Sclerodermatous cGVHD, particularly orofacial involvement, is poorly described but can be debilitating.
- Orofacial manifestations significantly impact patient morbidity.
Purpose of the Study:
- To characterize the orofacial features of sclerodermatous cGVHD.
- To describe the clinical presentation and management of orofacial sclerodermatous cGVHD.
- To evaluate treatment outcomes in a cohort of alloHCT patients.
Main Methods:
- Retrospective analysis of electronic medical records.
- Inclusion of patients who underwent alloHCT and developed orofacial sclerodermatous cGVHD between 1993 and 2017.
- Descriptive statistical analysis of patient data and clinical features.
Main Results:
- 39 patients with orofacial sclerodermatous cGVHD were identified.
- Common orofacial features included buccal mucosal fibrosis, limited mouth opening, perioral fibrosis, and gingival recession.
- Oral mucosal fibrosis was associated with chronic lichenoid inflammation and ulcerations; management included jaw stretching, surgery, and corticosteroid injections.
Conclusions:
- Orofacial sclerodermatous cGVHD presents with diverse manifestations affecting oral function.
- Fibrous banding, limited mouth opening, and perioral fibrosis are key features.
- Both surgical and non-surgical interventions show potential for improving function and reducing morbidity.
Background:
Chronic graft-versus-host disease (cGVHD) is a leading cause of morbidity and mortality following allogeneic hematopoietic cell transplantation (alloHCT). The sclerodermatous form of cGVHD can be particularly debilitating; however, orofacial sclerodermatous involvement remains poorly described.
Objective:
To characterize orofacial features of sclerodermatous cGVHD in a single center cohort of patients who underwent alloHCT.
Study Design:
Retrospective data were collected from electronic medical records and analyzed descriptively.
Results:
There were 39 patients who received alloHCT between 1993 and 2017 and developed orofacial sclerodermatous cGVHD. Concomitant cutaneous sclerodermatous cGVHD was common (n = 20, 51%). Orofacial sclerodermatous cGVHD features included fibrous bands of the buccal mucosa (n = 23, 59%), limited mouth opening (n = 19, 54%), perioral fibrosis (n = 8, 21%), and focal gingival recession (n = 4, 10%). Oral mucosal fibrosis was observed at the site of active or resolved chronic lichenoid inflammation in 30 patients, with all but two also presenting with a history of ulcerations. Management included jaw stretching exercises (n = 10; 6 stable/improved), surgery (n = 3; 2 improved), and intralesional corticosteroid injections (n = 2; 2 improved).
Conclusions:
Orofacial involvement with sclerodermatous cGVHD can present with multiple manifestations including fibrous banding, limited mouth opening, perioral fibrosis, and focal gingival recession. Surgical and non-surgical management strategies may improve clinical function and reduce morbidity.
