Oral Manifestations of Neurofibromatosis: A Rare Case Report.

Neurofibroma (NF) is a rare benign non-odontogenic tumor of the oral cavity. NF may present either as solitary lesions or as part of the generalized syndrome of NF or von Recklinghausen’s disease of the skin. The heterogeneous nature of NF was established by Riccardi et al. and he recognized at least seven types of NF. Among these proposed classifications of the disease, two distinct forms are generally accepted namely, a peripheral form known as NF Type I (NF-I), and a central form known as NF-II. NF-I represents the classic form of this disease, described by Recklinghawsen in 1882. Clinically, oral NF usually appears as slow growing, painless, pedunculated or sessile nodules. For illustration, a case of a NF with oral findings is been presented.

Mouth - A Diagnostic Mirror of Various Diseases.

The mouth is a unique site, due to the presence of hard and soft tissues in close approximation. It serves in various purposes of speech, mastication and digestion. It is an important entry point for many pathogens in the body. Many systemic diseases manifest in the oral cavity and mouth can show early signs or the only signs of a disease process at a site elsewhere. As the mouth is an easily accessible site, the indicators it shows of various diseases should not be overlooked. A dentist thus can frequently be exposed to such conditions and play a key role in the diagnostic procedure of various systemic diseases. Appropriate knowledge of these oral manifestations is essential for early diagnosis, treatment and referral of cases.

Oral manifestion of Langerhans cell histiocytosis mimicking inflammation.

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.

Manifestaciones gastrointestinales de la epidermolisis bulosa distrófica recesiva / Gastrointestinal manifestations of recessive dystrophic epidermolysis bullosa

Se presentan ocho niños con manifestaciones gastrointestinales secundarias a epidermolisis bulosa distrófica recesiva, como enfermedad de base. Las lesiones en orden de frecuencia fueron estenosis esofágica de grado y extensión variable en seis, historia de lesiones ampollosas de repetición en ocho, constipación en cinco y fusión de la lengua al piso de la boca (anquiloglosia) en dos. Se conluye que la lesión principal y más temida es la estenosis esofágica. Epidermolisis bulosa distrófica recesiva