title: Orthodontic treatment considerations for a patient with erythropoietic protoporphyria.
abstract: Erythropoietic protoporphyria (EPP) is an inherited blood disorder in which formation of the heme group of hemoglobin is defective. Specifically, a deficiency of the enzyme ferrochelatase leads to the accumulation of protoporphyrin, resulting in often painful photosensitivity of the skin and tissues. The prevalence of EPP is estimated at 1:75,000 to 1:200,000. Photosensitivity is exhibited upon exposure to light with specific wavelengths through the creation of reactive oxygen products (oxidants), activation of the complement system, and mast cell degranulation. The aim of this article is to report the orthodontic treatment of an 11-year-old boy with EPP, a Class III skeletal relationship, and an anterior crossbite. Orthodontic treatment established normal overbite and overjet. Short-term periodontal and dental tissue responses to treatment were noted. Extra care was needed when collecting photographic and radiographic records for this patient and during some treatment procedures to avoid causing a photosensitive reaction of the skin or oral mucosa.
authors.affiliation: Associate clinical professor, Department of Orthodontics, Faculty of Dentistry, University of British Columbia, Vancouver, British Columbia, Canada; private practice, Langley, British Columbia, Canada. Electronic address: richard@transformationorthodontics.com.
authors.forename: Richard G
authors.initials: RG
authors.lastname: Standerwick
authors.forename: Edwin H K
authors.initials: EH
authors.lastname: Yen
authors.forename: Benjamin
authors.initials: B
authors.lastname: Pliska
mesh_terms: D002508:Cephalometry / Q000379:methods; D002648:Child; D006801:Humans; D008297:Male; D008310:Malocclusion / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D009971:Orthodontics, Corrective / Q000379:methods*; D010787:Photosensitivity Disorders / Q000209:etiology*; D046351:Protoporphyria, Erythropoietic / Q000150:complications*; D011862:Radiography, Panoramic / Q000379:methods