Eman, Ghormallah Alamodi
Andres Davila
Katherine France
| Eman, Ghormallah Alamodi | Andres Davila | Katherine France |
Eman Ghormallah Alamodi, Andres Davila, Katherine France
Oral Medicine, University of Pennsylvania Dental Medicine
Introduction
Pityriasis rubra pilaris (PRP) is a rare inflammatory dermatologic condition. Only four cases to date have reported on oral mucositis associated with PRP. Herein, we present an additional case of oral lesions associated with PRP.
Methods
A 13-year-old female presented to the University of Pennsylvania School of Dental Medicine with a chief complaint of 4-month history of asymptomatic, generalized gingival erythema. She denied recent changes to diet, medications, and oral hygiene products. Pertinent medical history included PRP, holocarboxylase synthetase deficiency, and an associated history of B complex deficiency, and malnutrition. The patient took multiple medications and supplements, reported no known drug allergies, and both family and social histories were non-contributory. Review of systems was positive for dry and pigmented lesions around the umbilicus and at her hairline. Intra-oral examination revealed a papillary, pedunculated, erythematous gingival lesion in the mandibular midline and a 1cm well-demarcated band of erythema extending along the buccal attached gingiva to the molars with a 2-3cm thick band of moderate homogenous erythema on the palatal gingiva. Her tongue exhibited prominent areas of depapilation and mild erythema. Differential diagnosis included granulomatous inflammation, plasma cell gingivitis, and autoimmune-associated mucositis.
Results
Biopsy specimens evaluated with hematoxylin and eosin staining and direct immunofluorescence were consistent with chronic mucositis. The linear gingival erythema extended in size over follow-up but remained asymptomatic. Given the patient’s medical history, non-specific clinical and histologic findings, similarity to other reported cases, and lack of established connection with other medical conditions, PRP-associated mucositis was suspected. The patient is planned for management of PRP with ixekizumab, and her oral lesions will be evaluated for response.
Conclusion
Despite its name, pityriasis rubra pilaris commonly affects glabrous as well as follicular skin. The heterogenous presentation and clinical course and the pathogenesis of the condition all support the development of oral lesions in these patients. The clinical appearance and diagnostic findings above suggest an additional case of oral mucositis secondary to PRP.