Payam Mirfendereski
Jeffrey, W. Goetz
Mary Ellen Martin
Takako, I. Tanaka
| Payam Mirfendereski | Jeffrey, W. Goetz | Mary Ellen Martin | Takako, I. Tanaka |
Payam Mirfendereski1, Jeffrey, W. Goetz1, Mary Ellen Martin2, Takako, I. Tanaka1
1Oral Medicine, University of Pennsylvania School of Dental Medicine; 2Hematology/Oncology, University of Pennsylvania
Introduction
Sclerodermatous cGVHD is a distinctive phenotype of cGVHD affecting the skin, subcutaneous tissue, and fascia. The 5-year cumulative incidence of sclerodermatous cGVHD has been estimated to be 15-20% among cGVHD patients. In the maxillofacial region, sclerodermatous cGVHD presents most commonly as jaw stiffness, loss of elasticity, and contractures in the perioral tissues secondary to fasciitis and sclerosis. Sclerodermatous cGVHD poses high morbidity with poor treatment options available. This case report aims to build on the currently limited literature on maxillofacial involvement of sclerodermatous cGVHD.
Methods
N/A
Results
A 38-year-old male with a history of Ph-positive B-cell acute lymphocytic leukemia s/p allogeneic stem cell transplantation (allo-SCT) presented with limited mouth opening and occasional jaw muscle pain. He reported weight loss over the preceding 6 months due to decreased oral intake. After undergoing cyclophosphamide/total body irradiation conditioning and allo-SCT from his HLA-matched sister 3 years prior, he had developed refractory cGVHD affecting his joints, fascia, and skin along with deep sclerosis of his torso and limbs. He had been treated with prednisone and ruxolitinib, both of which had been tapered off, and at the time of presentation was under management with belumosudil 200 mg twice daily and extracorporeal photopheresis twice weekly. Extraoral examination revealed poor head posture related to his neck and shoulder rigidity. TMJ examination revealed significantly limited maximum inter-incisal opening of 26 mm; however, there was no TMJ muscle tenderness on palpation. Oral examination revealed good hygiene without significant mucosal abnormality. The diagnosis was rendered as trismus secondary to sclerodermatous cGVHD. The plan for the patient comprised a dedicated TMJ MRI, a course of professional TMJ physical therapy, and conservative therapies including the use of an occlusal stabilization appliance. At his second visit, the patient received trigger point injections with 0.5% bupivacaine with 1:200,000 epinephrine in his masseters and temporalis insertions bilaterally. The patient reported benefit with conservative treatments and trigger point injections but has not initiated physical therapy to date due to concerns about the COVID-19 pandemic.
Conclusion
Sclerodermatous cGVHD affecting the maxillofacial region presents a significant impact on patients’ quality of life. As with other oral manifestations of cGVHD, oral health care providers should be involved in the diagnosis and appropriate management of sclerodermatous cGVHD within a team-based model.