ABSTRACT
Incontinentia pigmenti (IP) or Bloch-Sulzberger syndrome is a rare X-linked dominant genodermatosis related to the NF kappa B essential modulator (NEMO) gene with approximately 800 cases reported worldwide. It usually occurs in females characterized by cutaneous, skeletal, neurological, ocular and dental abnormalities as well as an increased risk of childhood malignancies. Herein, we report a case of IP in a 14-year-old girl emphasizing early diagnosis and adding to the current literature on the subject.
ABSTRACT
Si bien es una lesión benigna, el granuloma central de células gigantes puede provocar erosión ósea y asimetría facial. Se describe el tratamiento quirúrgico exitoso de una lesión de estas características ubicada en el maxilar superior.
Subject(s)
Humans , Female , Adult , Granuloma, Giant Cell/surgery , Granuloma, Giant Cell/diagnosis , Granuloma, Giant Cell/therapy , Maxilla/abnormalities , Maxilla/surgeryABSTRACT
Acute traumatic ulcerations and granulomas of the oral mucosa may result from physical damage via sharp foodstuffs, accidental biting, or talking. Most ulcerations heal within days. Others become chronic, reactive, and exophytic. A histopathologically unique type of chronic traumatic ulceration is the traumatic ulcerative granuloma with stromal eosinophilia (TUGSE). TUGSE exhibits a deep "pseudoinvasive" inflammatory reaction. This lesion may occur under the tongue in infants as a result of chronic mucosal trauma caused by mandibular anterior primary teeth during nursing and is termed Riga-Fede disease (RFD). The clinical presentation many resemble squamous cell carcinoma causing concern. RFD, although not uncommon, is not frequently reported. Thus, dental practitioners are unfamiliar with such lesions. We present a large Riga-Fede lesion in an infant along with the clinical management.
Subject(s)
Chronic Disease , Diagnosis, Differential , Granuloma/pathology , Humans , Infant , Male , Oral Ulcer/diagnosis , Oral Ulcer/pathology , Tongue/injuries , Tongue Diseases/diagnosis , Tongue Diseases/pathology , Tooth EruptionSubject(s)
Biopsy , Granuloma/pathology , Humans , Infant , Male , Mouth/pathology , Oral Ulcer/etiology , Tongue , Tooth/growth & developmentABSTRACT
Congenital fusion of the jaws is quite rare. It may be unilateral or bilateral and involves only the soft tissues or both the hard and soft tissues. This anomaly may be seen separately or in association with syndromes. Maxillomandibular fusion restricts mouth opening causing problems in feeding, swallowing and respiration. This condition can be easily treated. However, in long-standing cases, growth anomalies from TMJ ankylosis may occur.