Rare Diseases Symptoms Automatic Extraction

Intraoral findings of Papillon-LeFevre syndrome.

[papillon-lefèvre syndrome]

Papillon-Lefevre syndrome (PLS) is a rare autosomal, recessive condition characterized by hyperkeratosis of palms and soles of the feet and elbows and by rapid formation of periodontitis and hypermobility, migration and exfoliation of the teeth of primary and permanent dentition. The purpose of this report was to describe the case of an 8-year-old boy who presented to the Department of Oral Diagnosis and Radiology of Faculty of Dentistry of Ondokuz Mayis University with a chief complaint of mobility and rapid loss of teeth. Hyperkeratosis of palms and soles were realized. His gingivae were hyperemic and edematous, and the teeth were mobile. Histopathological examination of the specimen taken from the thickened skin was reported to be consistent with PLS. All teeth with poor prognosis were extracted and extensive periodontal therapy was administered, and a special denture was constructed.

Diseases presenting "poor prognosis" symptom

  • adrenal incidentaloma
  • alexander disease
  • alpha-thalassemia
  • carcinoma of the gallbladder
  • cholangiocarcinoma
  • cushing syndrome
  • cutaneous mastocytosis
  • dedifferentiated liposarcoma
  • erdheim-chester disease
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • esophageal squamous cell carcinoma
  • hodgkin lymphoma, classical
  • junctional epidermolysis bullosa
  • kindler syndrome
  • liposarcoma
  • locked-in syndrome
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • megacystis-microcolon-intestinal hypoperistalsis syndrome
  • papillon-lefèvre syndrome
  • pleomorphic liposarcoma
  • primary effusion lymphoma
  • pyomyositis
  • sneddon syndrome
  • systemic capillary leak syndrome
  • von hippel-lindau disease
  • waldenström macroglobulinemia
  • well-differentiated liposarcoma
  • werner syndrome
  • wiskott-aldrich syndrome
  • zellweger syndrome

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