Pachyonychia congenita comprises a heterogeneous group of autosomal dominantly inherited conditions manifesting with characteristic nail thickening, palmoplantar keratoderma, follicular keratosis and oral manifestations like mucosal leukokeratosis. Less frequently epidermal cysts, hair-shaft abnormalities, natal teeth and laryngeal involvement may be seen. It is distributed almost evenly throug...

Dental lamina cyst or alveolar cyst is a commonly diagnosed developmental anomaly in the mouth of newborns. They are quite often mistaken as natal teeth if present in the lower anterior region. Although the prevalence is high, they are rarely seen because of the transient nature of the lesions. They are self limiting and disappear spontaneously a few weeks or months after birth. Hence no treatm...

Pachyonychia congenita (PC) is a rare autosomal dominant genetic skin disorder due to a mutation in any one of the five keratin genes, KRT6A, KRT6B, KRT6C, KRT16, or KRT17. The main features are palmoplantar keratoderma, plantar pain, and nail dystrophy. Cysts of various types, follicular hyperkeratosis, oral leukokeratosis, hyperhidrosis, and natal teeth may also be present. Four unrelated Ind...

ABSTRACT Because of multisystemic impairment in patients with Ellis-van Creveld syndrome, multidisciplinary care may be demanded since birth to assure breastfeeding. This report presents a case an infant that was facing breastfeeding difficulties because maxillary neonatal teeth. A 3 months old male syndrome referred Pediatric Dentistry Department two upper teeth causing difficulties. Clinical ...

BACKGROUND Riga-Fede disease (RFD) is a benign inflammatory disorder characterised by the appearance of a traumatic ulceration of the oral mucosa. Early detection of RFD and its adequate management are very important. CASE REPORT The authors present a an unusual case of RFD with concomitant Staphylococcus aureus meningitis. A 36-day-old female infant was referred to the emergency room of the ...

A review of the literature reveals a need to clarify the pathologic physiology of congenital polychlorinated biphenyl (PCB) poisoning, which is characterized by intrauterine growth retardation, brown staining of the skin and mucous membranes, as in Addison's disease, natal teeth, widely open fontanelles and sagittal suture and apparent overgrowth of the gingiva. The skull abnormalities may repr...

BACKGROUND Pachyonychia congenita (PC) is a group of autosomal dominant keratinizing disorders caused by a mutation in one of 4 keratin genes. Previous classification schemes have relied on data from case series and case reports. Most patients in these reports were not genetically tested for PC. OBJECTIVE We sought to clarify the prevalence of clinical features associated with PC. METHODS W...

OBJECTIVE The aim of this study was to investigate the prevalence of molar incisor hypomineralization (MIH) and its relationship with systemic conditions in a group of Iranian children. METHODS The study population comprised of 433 7-9 year olds, from four schools in Zahedan, Iran. Subjects were evaluated clinically by one examiner, and at a separate session, their mothers completed a coded m...