Dariers Disease with Oral Manifestations : A Clinical Rarity : Case Report

Dr. Saurav Shankar Das; Dr. Nandini N. Krishnamurthy; Dr. Grace Mary Joseph; Dr. Shijal Narayan; Dr. Riolla Sanchia Dsouza1

1

Publication Date: 2024/07/04

Abstract: Darier disease (DD), also known as keratosis folicularis and White disease , is an inherited autosomal dominant genodermatosis. DD often develops in childhood, persists through adolescence and causes small papules predominantly in seborrheic areas such as the face , chest and back. The oral lesions are asymptomatic and comprise multiple white papules in the buccal mucosa and the soft and hard palate , giving a cobblestone appearance. Histologically , DD is characterized by corps ronds and grains in addition to suprabasal cleavage. There are no currently validated curative treatments available for DD, with the majority of cases treated symptomatically. This is a case report of DD with varied manifestations intraorally and extraorally. Diagnosis was established on the basis of clinical , histopathological and dermatological findings. Patient reported to the OPD with a chief complaint of loose teeth. A holistic treatment approach was carried out based on clinical and histological findings. Functional and esthetic demands were met with satisfactory results.

Keywords: Dariers Disease , Extraction , Complete Denture ,Biopsy.

DOI: https://doi.org/10.38124/ijisrt/IJISRT24JUN1954

PDF: https://ijirst.demo4.arinfotech.co/assets/upload/files/IJISRT24JUN1954.pdf

REFERENCES

  1. Schmieder SJ, Sathe NC, Rosario-Collazo JA. Darier Disease. 2023 Nov 30. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan
  2. Suryawanshi H, Dhobley A, Sharma A, Kumar P. Darier disease: A rare genodermatosis. J Oral Maxillofac Pathol. 2017 May-Aug;21(2):321.
  3. Alsharif SH, Alesa D, Baabdullah A. Type 1 Segmental Darier Disease: Case Report and Discussion of the Treatment Options. Case Rep Dermatol. 2020 Sep 11;12(3):159-167.
  4. Savaş S, Koku Aksu AE, Sarıkaya E, Leblebici C, Gürel MS. Darier's disease: Clinical and demographic features of nine cases. TURKDERM-Turkish Archives of Dermatology and Venereology. 2018;52(2):51-5
  5. Cardoso CL, Freitas P, Taveira LA, Consolaro A. Darier disease: case report with oral manifestations. Med Oral Patol Oral Cir Bucal. 2006 Aug 1;11(5):E404-6.
  6. Sakuntabhai A, Ruiz-Perez V, Carter S, Jacobsen N, Burge S, Monk S, et al. Mutations in ATP2A2, encoding a Ca2+ pump, cause Darier disease. Nat Genet. 1999 Mar;21(3):271-7.
  7. Ashok Kumar P, Paulraj S, Dutta S. Debilitating Darier's Disease and Its Impact on the Quality of Life. Cureus. 2020 May 15;12(5):e8133.
  8. McNish A, Fitz-Henley M, Ho JD. Keratosis follicularis (Darier disease). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumordariersdisease.html. Accessed June 29th, 2024.
  9. Manoja KG, Siriwardena BS, Jayasooriya PR, Siriwardane DJ, Tilakaratne WM. A Rare Clinical Presentation of Intraoral Darier's Disease. Case Rep Pathol. 2011;2011:181728.10.Sehgal VN, Srivastava G. Darier's (Darier-White) disease/keratosis follicularis. Int J Dermatol. 2005 Mar;44(3):184-92.