Vitamin D-dependent Rickets
Overview: a disease of childhood where growing bones fail to mineralize.
Etiology: as opposed to nutritional Rickets (results from inadequate sunlight exposure or inadequate intake of dietary vitamin D, calcium, or phosphorus), vitamin D-dependent rickets is secondary to a gene defect that codes for the receptor for vitamin D in the gut. A metabolite of vitamin D is a hormone that is responsible for regulation of calcium release and uptake from bones.
Diagnosis: is through 1) blood tests: serum calcium (low), serum phosphorus (low) and 2) through bone biopsy (rarely performed, but will confirm Rickets diagnosis).
Accounts of “spontaneous” dental abscesses resulting in pulp infection through abnormally mineralized dentin.
- Thinning of inferior mandibular cortex
- Reduction in density, number, and thickness of trabeculae in cancellous bone
- Thinning/missing lamina dura
- Delayed tooth eruption
- Short stature
- Soft, pliable bones
- Bowed legs, increased incidence of greenstick fractures
- Widening/fraying of epiphyses of long bones
- Fronto-parietal bossing of skull
Special dental considerations/needs: Early dental treatment/prevention is necessary to prevent spontaneous pulpal necrosis in primary and permanent teeth.
Seow WK, et al. Pedatric Dentistry Vol 8, No 3 (1996): 245-250
Nield, LS, et al. Am Fam Physician. 2006 Aug 15;74(4):619-626
Stevens-Johnson Syndrome (Erythema Multiforme Major)
Overview: SJS is a life threatening condition of the skin in which cell death causes the epidermis to separate from the dermis. SJS is the more severe muco-cuteaneous form of erythema multiforme and is considered a dematological emergency.
Etiology: SJS is thought to arise from a disorder of the immune system and can be due to a viral infection (HSV, mumps, influenza) OR a drug-induced reaction from the use of antibiotics and sulfa drugs.
Diagnosis: Usually a diagnosis of exclusion (differential diagnoses usually include mucous membrane pemphigoid, pemphigus, and lichen planus). A biopsy is necessary to make a definitive diagnosis. Patients with Lupus and HIV are at higher risk. Overall incidence is relatively low (6:1,000,000). More common in adults than children, women:men (2:1).
Oral Manifestations and Clinical Features:
Begins with fever, sore throat, and fatigue. Conjunctivitis occurs in 30% of children with SJS. The rash that develops on the skin is widespread and appears as red, round macules, vesicles, bullae, and ulcers that have a characteristic “target” or “iris” appearance. Lesions in the mouth are the most common sign of the syndrome and present as erythematous and edematous blisters that rupture leaving painful ulcers/erosions. These erosions are covered by yellowish white pseudomembranes or hemorrhagic crusts.
Special dental considerations/needs: Defer treatment until lesions have completely resolved.
Kempton, et al. Misdiagnosis of Erythema Multiforme: A Literature Review and Case Report, Pediatric Dentistry July/Aug 2012
Stitt, VJ. Stevens-Johnson Sydrome: A Review of the Literature. J Natl Med Assoc. Jane 1988; 80(1):104-108.