Ulectomy in a patient with nephrotic syndrome under investigation for Galloway-Mowat syndrome: a case report.

The aim of this study is to report a case in which a patient with nephrotic syndrome underwent surgery to remove fibrous gum tissue (ulectomy). An 8-year-old patient, diagnosed with early onset nephrotic syndrome due to a mutation in the NUP107 gene, had received a kidney transplant and was therefore taking various medications, including immunosuppressants. On oral examination, the patient was found to have a fibrous gingiva that was preventing the eruption of the upper permanent central incisors. A ulectomy was performed and the gingival tissue was sent for histopathological analysis, which showed normal aspects. The upper right central incisor was seen in the oral cavity 15 days after surgery. A second procedure was carried out to facilitate the eruption of the upper left incisor, which was visualized in the oral cavity 30 days later. In addition, oral manifestations such as maxillary atresia, ogival palate and mouth breathing were observed. Therefore, the role of the dental surgeon in the lives of transplanted children is considered important, as they often take various medications that can affect their oral health. Thus, early diagnosis and effective treatment will be essential to prevent future malocclusions and thus improve the quality of life of these patients.

Lingual Actinomycosis Clinically Simulating Nodular Median Rhomboid Glossitis: Literature Review and Report of Additional Case.

Actinomycosis is an uncommon, subacute to chronic, suppurative bacterial infection caused by Actinomyces Israelii. About 3% of all actinomycosis cases occur in the tongue, often affecting adult patients (mean age, 50 years). The clinical characteristics of actinomycosis can resemble malignant or benign tumors, and other infectious diseases. A 56-year-old woman was referred presenting an ulcerated lesion on the tongue 1 year ago. Intraoral examination revealed an edematous nodular lesion with an ulcerated surface, slightly symptomatic, on the midline dorsum of posterior tongue, suggesting nodular median rhomboid glossitis. Cytology smear was negative for fungus. After excisional biopsy, histopathological examination showed a chronic inflammatory infiltrate supported by a fibrovascular connective tissue stroma, and at the deepest part, broad basophilic areas surrounded by neutrophils, containing numerous filamentous bacilli, which were highlighted by Gram and Groccott-Gomori staining. The final diagnosis was lingual actinomycosis. Oral amoxicillin treatment (8/8 h for 2 weeks) was started, and after 1-month complete resolution was observed. Lingual actinomycosis is a rare lesion that must be recognized by dentists, because its early diagnosis and correct treatment reduce the possibility of a clinical complication that compromises the patient's quality of life. Noteworthy, when located on the midline dorsum of posterior tongue, actinomycosis can simulate nodular median rhomboid glossitis, expanding its spectrum of clinical differential diagnosis.

Lip Granular Cell Tumor (GCT) in a Pediatric Patient: Critical Literature Review on Pediatric Intraoral GCT and Acquired and Congenital Intraoral Non-Neural GCT.

Granular cell tumor (GCT) is an uncommon benign neoplasm derived from Schwann cells, frequently affecting the oral cavity, skin, and gastrointestinal tract. On microscopy, pseudocarcinomatous squamous hyperplasia (PSH) and perineural involvement are potential diagnostic pitfalls. GCT should be differentiated from non-neural GCT (NN-GCT). A 13-year-old male patient was referred presenting a nodular lesion on the upper lip several months ago. After excisional biopsy, microscopy revealed GCT without PSH but presenting multifocal perineural involvement. By immunohistochemistry, ALK was negative, whereas Rb and INI1 expression was intact. Moreover, with few intraoral NN-GCTs being assessed, recent studies suggest that acquired dermal NN-GCT subgroup seems to correspond to ALK-rearranged variants of epithelioid fibrous histiocytoma. Accordingly, further research on this topic is strongly encouraged.

Resolution of blood eosinophilia and limited mouth opening after short-term follow-up in a pediatric Langerhans cell histiocytosis case.

Introduction: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia that often affects children, presenting a broad clinical spectrum. Methods: Here, we report a 13-year-old male Salvadorian patient who was referred presenting a nodular swelling at the mandibular angle region, mildly symptomatic, few weeks ago, which relevantly was associated with limited mouth opening. Intraoral examination was unremarkable. Imaginological exams revealed an osteolytic lesion affecting the vestibular cortex at the right mandibular angle. The blood test results were normal, except for eosinophilia (21%; absolute eosinophil count 4 × 109/L). After an incisional biopsy, microscopical and immunohistochemical analyses were consistent with LCH diagnosis, which corresponded to a single system-single site category. After a few weeks, the mandibular movements were re-established, and complete resolution of blood eosinophilia was observed. Conclusion: LCH with blood eosinophilia is rarely reported. To our knowledge, 3 cases have been previously published.

Pseudocarcinomatous Squamous Hyperplasia After Surgical Management Of Pediatric Ameloblastoma: An Immunohistochemical Study.

Pseudocarcinomatous squamous hyperplasia (PSH) affecting the jaws is uncommon. The mandible is the most common location, usually as a complication associated with osteomyelitis, medication-related osteonecrosis or osteoradionecrosis. Herein, we report an 8-year-old male patient presenting a solid ameloblastoma, plexiform type, who was treated by segmental mandibulectomy with bone grafting. Three months later, on the underlying graft, a sinus tract was observed, and a small bone fragment was resected which was diagnosed as bone sequestration associated with PSH. After 7-year follow-up, the patient is well, without alterations. To our knowledge, for the first time, we report in detail the immunohistochemical features of PSH which, in addition to epithelial component profile and chronic inflammatory cells, revealed a diffuse infiltration by dendritic cells, suggesting that epithelial cell-immune cell interactions in PSH pathogenesis should also be considered.