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Correspondence: Eric T. Stoopler, DMD, FDSRCS, FDSRCPS, University of Pennsylvania School of Dental Medicine, 240 South 40th Street, Philadelphia, PA 19104.
Department of Oral and Maxillofacial Surgery and Pharmacology, University of Pennsylvania School of Dental Medicine, PhiladelphiaDepartment of Otorhinolaryngology: Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia
A 14-year-old girl with dyskeratosis congenita (DC) was evaluated for widespread symptomatic oral lesions that developed shortly after undergoing allogeneic hematopoietic stem cell transplant (allo-HSCT) at the age of 1 year. Physical examination revealed generalized reticulate pigmentation of the skin and nail dystrophy consistent with the DC phenotype (Figures 1 and 2) Intraoral examination revealed generalized Wickham striae and erythema of the bilateral buccal mucosa with a linear pseudomembranous ulcer on the right buccal mucosa (Figure 3). Histopathologic analysis of the lesion revealed lichenoid mucositis without evidence of dysplasia and cumulative findings were consistent with oral chronic graft-versus-host disease (cGVHD). The patient was prescribed fluocinonide gel 0.05% to oral lesions twice daily and dexamethasone solution 0.5 mg/5 mL, 10-mL swish and spit twice daily for symptom management, and nystatin rinse 100,000 U, 5-mL swish and spit three times daily for antifungal prophylaxis.
Figure 3Oral lesion in a patient with dyskeratosis congenita demonstrating a linear pseudomembranous ulcer with surrounding striae on the right buccal mucosa.
This condition is associated with several telomerase-shortening genes with multiple inheritance patterns, including X-linked (DKC1), autosomal dominant (TERT, TINF2), or autosomal recessive (NOP10, NHP2, TCAB1).
Oral leukoplakia has been observed in up to 80% of patients with DC, with a reported 1000-fold increased risk of malignant transformation to oral SCC compared with the general population.
Patients with oral cGVHD or DC must undergo continuous surveillance because of the increased risk of oral and systemic malignancies associated with both conditions.
Reference
Fernández García M.S.
Teruya-Feldstein J.
The diagnosis and treatment of dyskeratosis congenita: a review.
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