Pemphigus Vulgaris in Adolescence: case report

Department of Pediatric Dentistry

Luthern Medical Center

7/17/2009

Residents Name: Tyler Roberts
Article title: Pemphigus Vulgaris in adolescence: case report
Author: Fabio Ramoa Pires, DDS et al
Journal: Pediatric Dentistry
Volume #, pages: 22: 2, 159-162
Year: September 1999
Major topic: Pemphigus Vulgaris
Type of article: case report
Main purpose: review signs and symptoms of Pemphigus Vulgaris (PV) involving a rare case of adolescence.
Method of research: case review
Key pts and findings:
Pemphigus Vulgaris (PV), an autoimmune disease, is an intraepithelial blistering disease caused by auto antibodies against desmosomal antigens. Recently this antigen was cloned and found to be a member of the cadherin family of molecules.
In fifty percent of patients affected by PV, oral lesions will be the first sign of the disease. At some point ninety percent of patients will manifest these types of symptoms. Other forms of Pemphigus such as foliaceus, erythematosus, and vegetans almost exclusively affect the skin. PV most often affects middle aged adults in their fifties and sixties and tends to predominate in females. However, much like the case report found in this article it can and does show up in adolescents and even children.
This case report presents a 16 year old Brazilian female, diagnosed with PV. The patient reported that 5 months prior to her first visit to the hospital, she noticed painful oral ulcers. These oral lesions were soon followed by erythemetous and coalescent, irregularly shaped ulcers on her back. In addition, bilateral submandibular lymphadenopathy (swelling of the lymph nodes) was present. Her oral lesions were described as atrophic, erosive, desquamative lesions involving buccal mucosa, soft palate, togue, and buccal gingiva. Initially, the patient was started on 40 mg per day of prednisone, this however, was gradually reduced to 5 mg per day. The patient has since been in stable condition with candidiasis as her only side effect to the medication.

Conclusion: Dentist should be aware that oral lesions are frequently the first sign of complaints in patients with Pemphigus Vulgaris. No standard treatment protocol exist in adolescence and children due to its rarity.

Assessment of article: Interesting and insightful

Ewing’s sarcoma of the mandible: Radiographic features with emphasis on magnetic resonance appearance

LUTHERAN MEDICAL CENTER
Dental Residency Program
Literature Review Form

Resident: Boboia Date: 7/24/09
Article title: Ewing’s sarcoma of the mandible: Radiographic features with emphasis on magnetic resonance appearance
Author(s): Gorospe et al
Journal: Oral Surgery, Oral Medicine, Oral Path., Oral Radio., Endo
Volume #; Number; Page #s): 91: 6, 729-734
Month / Year: June 2001
Major topic: Ewings sarcoma / Imaging using MRI
Minor topic(s):
Type of Article: Case Presentation
Main Purpose: Review the radiologic features of an ES lesion emphasizing its appearance on MRI
Review of the Case: A 12 y/o female presents with facial swelling x 7 days. Once a diagnosis of ES with extension into the surrounding soft tissues was made, a chemotherapy protocol was commenced. Progress was evaluated using MRI after the second round of chemo. Due to progression of the tumor, radiotherapy was added to the treatment. After the 10th chemotherapy cycle the patient underwent an autologous bone marrow transplantation. The patient was discharged 15 days s/p transplantation. A new MRI revealed a decrease in tumor volume. All lab data normalized 40 days after transplantation. Clinical follow-up revealed the patient is alive and symptom free with no evidence of recurrence or distant metastases. Labs were normal 36 months after diagnosis.
Review of ES: : ES was first described in 1921. This lesion is thought to have neuroectodermal origin, exhibiting some histological similarities as these tumors. This is the second most common primary malignant bone tumor in children and adolescents. 80% of cases occur in the first 2 decades of life with a 2:1 male to female ratio. These lesions account for 4-15% of all primary bone tumors and 1% of all malignant tumors in children. 2/3 of all cases appear in the lower skeleton with a predisposition for long bones of the extremities and the pelvis; the involvement of the facial skeleton is very rare (3%). When the facial skeleton is involved, the mandible is the most commonly affected bone. ES is composed of small round cells with oval / round nuclei and scarce clear cytoplasm arranged in sheets. Differential diagnosis should consider osteosarcoma, lymphoma, neuroblastoma, and other neuroectodermal tumors. Swelling in the invoved area and pain are common symptoms for mandibular ES. Traditional treatment includes surgical excision and local irradiation. Recently chemotherapy and radiotherapy have dramatically improved long term survival rates. Surgery is justified only if tumor control and preservation of function are guaranteed. In this case surgery was not done due to the extension of the tumor into the surrounding tissues as well as pulmonary metastases.
Imaging: Plain radiographs are the first tool for determining presence of bone lesions; ES presents as a lytic, permeative, poorly defined lesion. CT scan provides better visualization yet similar information as that obtained from radiographs. It also provides more detailed information regarding the bone marrow extension of the primary tumor. MRI is the gold standard of imaging the extension of the primary tumor (it therefore remains the method of choice for tumor staging) as well as determining the effects of therapy.
Summary of conclusions: MRI is the diagnostic tool of choice in the local staging of ES and is an imaging technique of great value in monitoring the effects of chemotherapy
Assessment of article: Fair review of ES. MRI has routinely been used to stage and follow the progress of ES tumors for some time now (not exactly front page news).

Recurrent Apthous Stomatitis

Resident’s Name: Brian Schmid Date: 7/17/2009
Article title: Recurrent Apthous Stomatitis
Author(s): Jonathan Ship DMD, Elisa Chavez DDS et al
Journal: Quintessence International
Month, Year: 2000
Major topic: Review of recurrent apthous stomatitis
Type of Article: Review
Findings: RAS is the most common oral mucosal disease in humans with an incidence of 5-25% and much higher in selected populations (50-60% in med/dent students). Minor RAS consists of small (<10mm) painfululcers with a necrotic center and a gray-white pseudomembrane. They typically heal within 10-14 days without scaring and is typically located on nonkeratinized oral mucosa. Major RAS (aka Suttons Disease and periadenitis mucosa necrotica recurrens comprises 10-15% of RAS cases). These lesions are larger than 10mm and often scar. They can also last for months and be a major cause of dysphagia. Major RAS has a predilection for lips, tongue, soft palate and the palatal fauces. It is frequently found in HIV patients. Multiple small clusters of pin point ulcers characterize Herpetiform RAS.
The most common differential for RAS is herpes simplex, which differs from RAS in that the primary form causes fever and erythema and it typically occurs on attached tissue. Varicella Zoster virus can be differentiated by its unilateral patter of following the trigeminal nerve and a prodrom of pain and tingling/burning sensation. Herpangina lesions usually have correlated systemic symptoms like fever and will resolve in 1-2 weeks. Erythema multiforme lesions are accompanied by stargetoid skin lesions and occur on both attached and movable mucosa. Oral lichen planus can resemble RAS but lesions will typically also occur on the gingiva and hard palate, and is often not painful. Systemic lupus erythematosis, Crohns disease, Behcets disease, Reiters syndrome and HIV/AIDS can also present with RAS-like ulcers. While there is no hardline causation of hematological defects with RAS, a significant persentage of patients can be successfully treated with blood elements.
Chronic minor or major RAS can be a concern due to the myriad potential underlying systemic diseases and any chronic RAS patient should be referred for medical workup. Dermatology and internal medicine may be necessary for immunosupressant medications; Otolaryngology for infectious diseases such as pemphigoif and herpangina; GI for IBD and Crohns; Opthamology for Behcets or Reiters disease. Also, allergists, infectious disease and hematology may need to be consulted.
Anti-inflammatory topical gels and ointments are the first line of chemotherapeutic defense. They are most effective when applied early in the outbreak. The topical glucocorticoids of choice is fluocinonide, triamcinolone and clobetasol. These drugs mixed with Orabase can be an effective treatment. 5% amlexanox applied 4 times daily is also an effective and safe treatment. There are also effective topical rinses such as Sucralfate.

Key points/Summary: There are many possible treatments for RAS and even more potential causes. It is essential to cover your medical bases and account for any possible underlying causes.

Assessment of article: A super review of RAS related material.

Lesch-Nyhan syndrome: a treatment planning dilemma

Department of Pediatric Dentistry

Lutheran Medical Center

Date: 06/12/2009

Article title: Lesch-Nyhan syndrome: a treatment planning dilemma

Author(s): Joseph L Dicks

Journal: Pediatric Dentistry

 Volume (number): vol 4 num 2

 Month, Year: 1982

Major topic: Lesch-Nyhan syndrome

 Minor topics: 

Type of Article:  Case Review

Main Purpose: Review implications of Lesch-Nyhan syndrome and treatment of patient’s with this syndrome. 

Overview of method of research:   Case Review, Review of Literature

Findings:  

Self mutilative behavior, especially associated with the mouth makes these patients a particular challenge for dentists.

Key points/Summary :

Cause is an inheritable error of purine metabolism, in males only

Large amounts of uric acid build up, leading to renal failure if untreated

Presents as mental retardation and self mutilative behavior, which does not improve with treatment

All have cerebral palsy with sever spasticity.

Usually mutilative behavior starts with the eruption of teeth and all perioral structures are soon destroyed

Often these patients are relaxed when restrained.

There is not much in the dental literature about this syndrome (at in 1982)

In some cases, offending teeth are extracted under LA and restraint

Extremely destructive cases will have all teeth extracted to limit mutilative risks

Sometimes periods of mutilative remission will fool clinicians into delaying treatment of dangerous teeth.  

Although radical, usually full mouth extraction is the only choice in mutilative cases. 

In mild cases, a soft splint has been used with some success

3 catagories of the syndrome

cat 1: no oral self-mutilative or biting behavior, most rare form

cat 2: some oral self-mutilative tendencies, but can be controlled with restraint

cat 3: severe, attempt to make mouth guard, if not, full mouth extraction under GA is best.

Assessment of article:  I’ve never seen a patient with this syndrome, but it is very interesting and would be heart-breaking to treat. 

The spectrum of dental manifestations in vitamin D-resistant rickets: implications for management

Department of Pediatric Dentistry

Lutheran Medical Center

 

Resident’s Name:  Chad Abby                                    Date: 3/20/2009

Article title:  The Spectrum of Dental Manifestations in Vitamin D-resistant Rickets:  Implications for Management

Author(s):  W. Kim Seow, S.C. Latham

Journal:  Pediatric Dentistry

 Volume (number): Vol. 8, No. 3

Month, Year:  1986

Major topic:  Rickets

 Minor topic(s):  Implications for management in vitamin D-resistant rickets (VDRR)

Type of Article:  literature review

Main Purpose:  To provide guidelines in the prevention of dental abscesses in the VDRR patient and to determine whether varying degrees of dental manifestations appear

Methods:  Thirteen patients were used in this study and they were divided into 3 main grades depending on their dental manifestations and the need for treatment and prevention of dental abscesses.

Findings:  Grade 1 is comprised of patients who show minimal or no dental manifestations of VDRR.  These patients only require routine dental care and preventative measures.  Grade 2 patients are those who show moderate dental manifestation of the disease, only a few teeth are involved, and the prophylactic procedures required moderate.  Grade 3 is comprised of patients who show severe dental manifestations of the disease and require extensive treatment and aggressive prophylactic measures to prevent the development of dental abscesses.  Besides prophylactic coverage to prevent occlusal wear, routine preventative care for dental caries is extremely important as minimal caries can lead to pulp exposures.

Key points/Summary :  The most common form of rickets in developed countries is an inherited form of rickets known as vitamin D-resistant rickets (VDRR), usually inherited in an X-linked dominant manner.  Low levels of serum phosphate lead to defective calcification with signs and symptoms of rickets appearing at about 8-10 months of age.  These signs include lateral bowing of the legs, frontal bossing, enlargement of the costochondral junctions, scoliosis and lordosis.  Medical management of VDRR consists of phosphate replacement together with vitamin D given usually in the form of calcitriol.  Dental manifestations are quite characteristic with multiple “spontaneous” dental abscesses usually caused by the large pulp chambers the teeth usually posses.  Histological studies often show abnormal dentin calcification.  The dental manifestations seen in patients with VDRR appear in a spectrum of severity, ranging from the very severe, with involvement of nearly the entire dentition, to the very mild with normal appearance of the teeth.  Effective strategies for the prevention of dental abscesses are available and should be employed to suit each individual patient’s needs. 

Assessment of article:  A child with grade 3 VDRR would be very interesting and stressful to treat.

Prevalence of dental abscess in a population of children with vitamin D-Resistant rickets

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: Derek Banks Date: June 5, 2009
Article title: Prevalence of dental abscess in a population of children with vitamin D-Resistant rickets
Author(s): A McWhorter, N Seale
Journal: Pediatric Dentistry
Volume (number): 13:2
Month, Year: 1991
Major topic: Metabolic Disorders
Minor topic(s): Vitamin D-Resistant Rickets (VDRR)
Type of Article: Retrospective study
Main Purpose: Evaluate prevalence and contributing factors to abscess in children with VDRR
Overview of method of research: Examining dental records, taking oral histories from parents, guardians, or family dentists, and examining patients
Findings: Out of 25 patients with VDRR, 6 had experienced a dental abscess. Of these six, however, there were 42 total abscesses altogether. 34 of the 42 abscessed without gross caries or previous treatment and the remaining 8 abscessed after receiving treatment and were all posterior teeth. 57% were anterior and 43% posterior. Mandibular and maxillary teeth were involved equally. Sequence of teeth effected loosely followed eruption pattern. No direct correlation could be made between radiographic abnormalities (e.g. pulp chambers extending to the DEJ) and abscess. Most abscesses occurred in patients taking higher dosages of vitamin D, indicating a correlation between the severity of the disease and susceptibility for dental abscess (or possibly a link between vitamin D supplement and susceptibility for abscess?). None of the children in this study had only one abscess. Number of abscesses in ranged from 2-11 in patients who had abscesses. Presence of abscess, therefore, may be a good predictor for future abscess. The authors suggest prophylactic treatment (e.g. pulp/ssc) of primary molars may be indicated in patients with VDRR and a previous abscess.
Key points/Summary : Only 25% of patients with VDRR in this study had abscesses. Of the 25%, all had multiple abscesses. Presence of abscess may be correlated with severity of the disease.
Assessment of article: A larger sample size would be nice.

Rickets

Laura Sabnani 6/4/2009

Rickets

A disease characterized by a softening of the bones causing deformities and fractures due to a lack of Vitamin D, calcium, or phosphate
Mostly caused by Vitamin D Deficiency but can also be caused by severe vomiting and or diarrhea
Patients at risk include breast-fed infants who are not exposed to sunlight or patient’s mothers not exposed to sunlight and individuals who are not drinking fortified milk.
Pts with red hair, vegetarians, patients who are lactose intolerant, have renal disorders, genetics (x-linked)
Calcium and phosphate absorption from the gut requires Vitamin D
If the blood levels become too low of these minerals, calcium and phosphate may be released from the bones
Pts can have bone pain, muscle weakness, increased tendency for fractures, bowed legs, growth disturbances, hypocalcemia, tetany, craniotabes (soft skull)
May be diagnosed by blood test showing low levels of calcium and serum phosphorus. Serum alkaline phosphatase may be high
Arterial blood gases may show metabolic acidosis
X-ray may show altered shape of bones
Bone biopsy rare but can confirm
Treatment increase in dietary HGH, phosphates and Vitamin D
Exposure to sunlight, cod liver oil, halibut-liver oil, and viosterolSupplementation may also be used Vitamin D3 (most readily absorbed form)

Hypophosphatasia

Hypophosphatasia Fact Sheet
Dan Boboia, DDS


What is it?

A rare inborn error of metabolism characterized by a wide spectrum of defects in mineralized tissues. Patients can have a low serum alkaline phosphatase level, excessive excretion of phosphoethanolamine in the urine, and abnormally wide osteoid arms. Clinical manifestations are highly variable and range from almost a total lack of skeletal formation to the premature loss of permanent anterior teeth.

Epidemiology:

Inherited as autosomal recessive trait with a prevalence of 1:100,000 live births

Etiology:

Caused by a defect in the gene encoding tissue nonspecific alkaline phosphatase (TNSALP)

Enzyme is present in most tissues and in blood serum; it cleaves phosphate from three other specific proteins. These proteins are intermediates in diverse and unrelated biochemical pathways.

Signs and Symptoms / Diagnosis:

Classified according to age when symptoms first appear

1) Perinatal - occurs in utero; most infants are stillborns or die shortly after birth; death is usually due to respiratory failure; marked hypocalcification of skeletal structures is often observed

2) Infantile – patients may appear normal up to 6 months of age-after this they may show failure to grow; less severe yet similar skeletal deformities as the lethal form; infants feed poorly, present with rib deformities (predisposing to pnemonia), skull deformities such as wide or obliterated fontenelles / cranial sutures (predisposing to increased intracranial pressure), blue sclera and pathologic lid retraction; generalized osteopenia and ossification defects with a bowing of the long bones; fatal in about half of cases

3) Childhood - child may have rickets, small stature, waddling gate, improperly formed periodontal ligament due to partial or complete lack of cementum formation; premature loss of primary anterior teeth (without evidence of a significant inflammatory response) displaying cementum aphasia; deciduous incisors usually effected first; Skull has radiographic appearance of “beaten copper” showing uniformly spaced, poorly defined, small radiolucencies

4) Adult – typically mild; associated with premature loss of anterior maxillary and mandibular teeth, history of early primary tooth loss, enamel hypoplasia, proptosis, elevated intracranial pressure / brain damage, pseudogout, and stress fractures involving the metatarsal bones of the feet



Treatment and Prognosis:

Treatment of symptoms is all that can be done because the lack of alkaline phosphatase cannot be corrected. Attempts to treat this condition by alkaline phosphatase infusions have been unsuccessful because the enzyme function intracellularly rather than in the extracellular environment. Fractures are treated with orthopedic surgery, prosthetic appliances are indicated to replace missing teeth, but satisfactory results are not always attainable because of the hypoplastic alveolar bone.

Phenylketonuria and dentistry: review of the literature

Department of Pediatric Dentistry
St Joseph Hospital

Resident’s Name: Craig Elice Date: June 5, 2009
Article title: Phenylketonuria and dentistry: review of the literature
Author(s): Walker, JD, Cral JJ, McDonnell
Journal: J Dent. Child
Month, Year: July-August 1982
What is it? PKU is an autosomal recessive disorder noted by a defect in phenylalanine hydroxylase. This enzyme converts phenylalanine into tyrosine, which when defective accumulates in body fluids and causes brain damage and mental retardation.
Epidemiology: affects 1 in 15,000-18,000 births, showing a greater frequency in Irish and Scottish, and generally Caucasians.
Signs and Symptoms: Untreated PKU patients classically show moderate to severe mental retardation with schizoid behavior, hyperkinetic movements, microcephaly and are prone to seizures. The children are blonder then unaffected siblings, have blue eyes, a musty odor, and a tendency to have eczema.
Diagnosis: Early detection of neonates is performed by a bacterial inhibition assay to detect elevated phenylalanine in the serum. A diagnosis is made by having two serum phenylalanine levels greater than 20mg percent at least 24 hours apart while the infant is on a regular diet, Tyrosine levels of less than 5 mg percent, and a pattern of urinary metabolites.
Treatment: Dietary therapy should begin as early as possible. Products like Lofenalac or other low phenylalanine products are given to infants. The diet should be maintained to a minimum of 6 years of age when the brain is almost fully developed. However, continuation of the diet to a later age shows improved behavior and reduction in the severity of the eczema. No special considerations are necessary when treating the oral health of patients diagnosed with PKU

Precocious Puberty

Precocious Puberty

Brian Schmid DMD

Definition:
Early onset of puberty which results in normal development and growth. May be a variation of normal development or a disease-state secondary to abnormal endogenous hormone production or exogenous exposure.

Etiology:
Causes are variable and disparately supported. They include: endocrine disorders, familial associations, genital abnormalities, neurophysiologic conditions and hormone releasing tumors. Idiopathic central precocious puberty can be partial, complete or transient; it is often due to damage to the inhibitory system of the brain or a hypothalamic hamartoma releasing increased amounts of GnRH
Peripheral precocious puberty is caused by the presence of sex steroids from other sources such as gonadal/adrenal tumors, congenital hyperplasia etc.

Symptoms:
Early pubic, breast or genital development, induction of early bone maturation resulting in a reduced final adult height. Pubic hair or genital enlargement before 9.5 years, breast development in boys, pubic hair before 8 or breast development in girls with onset before 7 years, menstruation in girls before 10. Studies have shown that development in both sexes, particularly females is trending younger which has complicated the diagnostic criteria of precocious puberty.

Treatment:
It is often left untreated. Anastrazole, an aromatase inhibitor, can be used to interfere with the production of estrogens. Histrelin is a GnRH inhibitor.

Oral findings:
Often associated with McCune Albright Syndrome which can include polyostotic fibrous dysplasia and hypodontia. Also some crossover with Hyperpituitarism whose oral/dental findings include frontal bossing, prognathism, intradental separation, macroglossia, oligodontia and macrodontia.