Epstein Pearls


Article Author:
Laura Diaz de Ortiz


Article Editor:
Magda Mendez


Editors In Chief:
David Wood
Andrew Wilt
Hajira Basit


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
6/8/2019 1:38:46 PM

Introduction

Some transient benign oral mucosal conditions are often observed in newborns. These lesions exhibit a wide range of developmental abnormalities and morphologic variations. The identification and differentiation of the lesions remain crucial to guide clinicians in prioritizing the management of these problems as well as in the education to anxious parents.

In 1880, a Prague pediatrician Alois Epstein first described Epstein disease as the presence of small nodules in the oral cavity of newborns.[1][2][3][4]

Multiple investigators reported a high rate of occurrence of palatal mucosal cysts in fetuses and infants. However, it was not until 1967 when Alfred Fromm studied and classified them according to their location and composition as Epstein pearls, Bohn nodules, and dental lamina cysts based on his comprehensive study of 1367 newborn infants. This was one of the first and largest studies done on oral cysts. Fromm concluded that these lesions were commonly found among infants and there were distinct types based on their histology and clinical presentation.

Some authors use these terms interchangeably. Epstein pearls have been labeled as epithelial debris of the tooth follicle, gingival glands of Serres, and as abortive enamel organs on the Palatine area. On the other hand, Bohn’s nodules are those found along the buccal and lingual aspects of the dental ridges. Dental lamina cysts are usually found on the crest of the alveolar mucosa. Currently, Palatal cysts of the newborn are the preferred term instead of Epstein’s pearls, Bohn’s nodules, or gingival cysts.[5][6][7][8]

Etiology

Keratin entrapment within the soft and hard palates causes Epstein pearls.

Epidemiology

Epstein pearls are observed in nearly 60% to 85% of newborn infants. Among the different racial groups, Japanese newborns are most commonly affected (up to 92%), followed by Caucasians and African-Americans. No gender predilection has been observed through the years.[9]

In one study, Epstein pearls were more common in infants born to multigravida mothers, also with those with higher birth weight. One study done in Turkey found that Epstein's pearls were less frequent in post-term babies in comparison with pre-term and term ones. A greater rate seen in term babies was reported. (Moosavi et al.).

Pathophysiology

Near the end of the 8 weeks in utero, the palate begins its development. Each maxillary process generates a lateral palatine process within the mouth. These processes are horizontal and shelf like, growing from the lateral aspect of the mouth toward the midline and downward. Between the 10 to the 11 weeks in utero, the lateral palatine processes meet and fuse with each side and with the much smaller premaxillary process and the nasal septum; palatal fusions normally are completed by the end of the 4 months of gestation.

In this stage, there is a theory that states that epithelium entrapped between the palatal shelves and the nasal process formed cysts called Epstein pearls. Another theory expressed that these cysts may come from epithelial remnants that have arisen from the formation of the minor salivary glands of the palate.

Histopathology

Epstein pearls are keratin-filled cysts with stratified squamous epithelium lining. Located on the mid-palatal raphe at the junction of the hard and soft palates.

History and Physical

They are small, opaque whitish-yellow lesions adjacent to the mid palatine raphe with no mucous glands in it Lesions are firm in consistency, size range from less than a millimeter to several millimeters in diameter. Size does not progress over time. They can be palpated during sucking by the examiner.

  • Epstein pearls can be seen in cluster groups of 2 to 6 cysts, although they can present as an isolated lesion. Their distribution varied from mouth to mouth, barely being identical even in twins.
  • Occasionally these cysts show communication with the mucosal surface.
  • Cleft children appear to have cysts in the identical distribution pattern as non-cleft children except for those with a cleft palate which are located at the margins of the palatal shelves instead of the midline.
  • Parents of newborn infants sometimes worriedly visit a dental surgeon or pediatrician, complaining of the presence of these abnormal structures in the mouth of their infants. 
  • The clinician should explain that Epstein pearls are benign and asymptomatic, with no interference with feedings or tooth eruption.
  • Epstein pearls resemble the equivalent of milia (white papules produced by retention of sebum and keratin in the hair follicles), which are frequently seen on the faces of neonates.

Evaluation

Epstein pearls are a clinical diagnosis. No laboratory or imagining is needed.

Treatment / Management

No treatment or removal is required. Parental apprehension should be alleviated by reassurance.

Differential Diagnosis

After a lesion is found in the oral cavity, it is important to formulate a differential diagnosis since this will help lead any additional evaluation of the condition and managing the patient.

Some of the differential diagnosis for Epstein pearls include:

  • Bohn nodules which are mucous gland cysts, frequently located on the buccal or lingual aspects of the alveolar ridges and rarely on the palate. Histologically consist of mucous glands and ducts. They are numerous, grayish white and firm in consistency.
  • Dental lamina cysts are remnants of dental lamina epithelium found on the crest of the alveolar mucosa. They may be greater in size, more transparent, solitary lesion and fluctuant.
  • Natal and Neonatal teeth are rare and usually located in the lower incisors region. They represent the early eruption of normal primary teeth. These are associated with developmental anomalies and recognized syndromes including Rubinstein-Taybi, Hallerman-Streiff, Ellis-van Creveld, Pierre-Robin, pachyonychia congenita, short rib-polydactyly type II, steatocystoma multiplex, cyclopia, Pallister-Hall, and many others. Therefore if the patient has any other dysmorphic features, genetic evaluation is indicated.
  • Congenital epulis is pedunculated, soft nodule from 1 mm to several cms in diameter located on gingival margin.
  • A congenital ranula is a translucent, firm papule or nodule found on the anterior floor of mouth, lateral to lingual frenulum.
  • Alveolar lymphangioma is bluish fluid-filled lesions on the alveolar ridges. No seen in the palate. Predominant in black neonates. These lesions can be isolated or multiples.

Prognosis

Most of these cysts involute or spontaneously rupture eliminating their keratin contents into the oral cavity within the first few weeks to months of postnatal life.The lesions are hardly ever seen after three months of age. However, it has been suggested that part of the cystic epithelium may remain inactive even in the adult gingiva.

Enhancing Healthcare Team Outcomes

Epstein pearls may be encountered by many specialists including nurse practitioners. The key is to reassure the parent that the lesions are harmless and will disappear with time. No attempt should be made to remove them as this may cause more harm than good.


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Epstein Pearls - Questions

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Epstein pearls usually are what color?

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Epstein pearls usually are found in what part of the body?



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Examination of the mouth of an infant reveals Epstein pearls along the gum line. The infant may have which of the following?



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While assessing the oral cavity in an infant, Epstein pearls are noted along the gum line. What disease should be ruled out?



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Which statement is most accurate regarding Epstein pearls?

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Which ethnic group is mostly affected by Epstein pearls?



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Who first described the Epstein pearls?



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What are the histologic findings and location of the Epstein pearls?



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What is the treatment for Epstein pearls?



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Which of the following is not part of the differential diagnosis of Epstein pearls?



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A four-day-old baby born full term at 41 weeks by induced vaginal delivery is seen for the first time in the outpatient pediatric clinic. Vital signs are within normal limits. Physical exam is remarkable for two millimeters opaque whitish lesion located on midpalate area. Which of the following is the next best step in the management of this patient?



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A 2-month-old boy is brought to the clinic for the 1st set of vaccines. Oral examination shows six small white-yellowish cysts on the midpalate area. The patient is smiling and coos. At what gestational age do these lesions most often form in the uterus?



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A one-month-old boy is brought for the first time to the clinic for evaluation. He was adopted from Japan. Mother expresses concern about multiples whitish lesions noted on the roof of the mouth. On physical exam, four whitish cysts are seen in the mid-palatine region. Mother inquires if they will be any issues with tooth eruption. Which of the following best answers her question?



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A 15-day old girl is born full term by C-section due arrest of labor. During the initial physical exam, five pearly lesions are seen in the palatine area. Rest of the exam is unremarkable. Epstein pearls are suspected. Which of the following best describes the histological composition of these lesions?



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Epstein Pearls - References

References

Zeman J,Zeman L, Short view on origins of paediatric health care in Prague. Casopis lekaru ceskych. Summer 2018;     [PubMed]
Haveri FT,Inamadar AC, A cross-sectional prospective study of cutaneous lesions in newborn. ISRN dermatology. 2014;     [PubMed]
Singh RK,Kumar R,Pandey RK,Singh K, Dental lamina cysts in a newborn infant. BMJ case reports. 2012 Oct 9;     [PubMed]
Gupta P,Faridi MM,Batra M, Physiological skin manifestations in twins: association with maternal and neonatal factors. Pediatric dermatology. 2011 Jul-Aug;     [PubMed]
de Carvalho JF,Pereira RM,Shoenfeld Y, Pearls in autoimmunity. Auto- immunity highlights. 2011 May;     [PubMed]
Gokdemir G,Erdogan HK,Köşlü A,Baksu B, Cutaneous lesions in Turkish neonates born in a teaching hospital. Indian journal of dermatology, venereology and leprology. 2009 Nov-Dec;     [PubMed]
Sachdeva M,Kaur S,Nagpal M,Dewan SP, Cutaneous lesions in new born. Indian journal of dermatology, venereology and leprology. 2002 Nov-Dec;     [PubMed]
Moosavi Z,Hosseini T, One-year survey of cutaneous lesions in 1000 consecutive Iranian newborns. Pediatric dermatology. 2006 Jan-Feb;     [PubMed]
Hayes PA, Hamartomas, eruption cyst, natal tooth and Epstein pearls in a newborn. ASDC journal of dentistry for children. 2000 Sep-Oct;     [PubMed]

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