Macroglosia en Amiloidosis Primaria como Clave Diagnostica: reporte de un Caso / Primary Amyloidosis with Macroglossia as Diagnostic Key: case Reporte

Amiloidosis se refiere a un grupo de enfermedades caracterizadas por el deposito extracelular de proteínas insolubles llamadas amiloide. La presentación intraoral de amiloidosis con macroglosia como signo primario es una entidad extraña, la cual puede afectar la funcionalidad de los pacientes aparte de la patología subyacente. Reportamos el caso de una paciente femenina de 85 años sin antecedentes mórbidos que al examen físico presenta macroglosia. La biopsia del tejido afectado fue estudiada bajo inmunohistoquimíca con tinción rojo congo, asociada al análisis de laboratorio que consignó un aumento en el componente monoclonal de cadenas livianas lambda. Se diagnosticó como amiloidosis primaria (AL). Se considera fundamental el entrenamiento al odontólogo general para el diagnóstico precoz de estos signos clínicos, por la baja sobrevida de la amiloidosis y la probabilidad de patologías ocultas como mieloma múltiple, el cual está asociado en un 20 % con AL.

Amyloidosis groups a large set of diseases characterized by the deposit of an extracellular insoluble protein known as amyloid. Intraoral presentation of macroglossia induced by amyloidosis is a rare entity that can impair patient function and hide other pathological conditions. We report a case of an 85-year-old woman with no morbid background were the physical examination revealed macroglossia. Primary systemic amyloidosis (AL) definitive diagnosis was made after the tongue biopsy resulted positive under congo red histochemical staining, and serum immunoelectrophoresis had elevated levels for lambda light chains. Primary dental dentistry training on semiotics is imperative to diagnose early stages of hidden pathologies with low life expectancy such as AL, which in 20 % of cases are associated with life threatening diseases like multiple myeloma.

Papilas Fungiformes Pigmentadas de la Lengua. Características Clínicas, Histológicas y Dermatoscópicas de una Serie de Casos Ecuatorianos / Pigmented Fungiform Papillae of the Tongue. Clinical, Histological and Dermatoscopic Characteristics A Serie of Ecuadorian Cases

RESUMEN: Las papilas fungiformes pigmentadas de la lengua, cuyas siglas son PFPT, del inglés Pigmented fungiform papillae of the tongue, es una condición asintomática, no progresiva que se presenta en personas de piel oscura, en las cuales las papilas fungiformes cambian de su color rosado natural, a una gama de café a negro. El objetivo de nuestro estudio es reportar y describir las caractetísticas clínicas, dermatoscópicas e histológicas de la PFPT por primera vez en una serie de pacientes ecuatorianos. Estudio prospectivo simple en el Centro de Especialidades Dermatológicas Garzón, período de dos años. El criterio de inclusión fue cambio de coloración a nivel lingual, se recolectaron datos demográficos, clínicos; fotografías, dermatoscopía, y biopsia, para tinción con hematoxilina-eosina y Fontana-Mason. Examinamos 8.640 pacientres, 15 (12 mujeres, 3 varones) fueron diagnosticados de PFPT. La edad promedio fue 31 años, todos fueron mestizos, con fototipo de piel predominante III y IV. El tiempo de evolución promedio en años fue 5,8. Ninguno tuvo antescedentes familiares o personales relacionados a la patología. La evaluación clínica demostró que el patrón de distribución de acuerdo a la clasificación de Holzwanger en la gran mayoría fue tipo II (13/15). En todos los casos la dermatoscopía y la histología fueron específicas demostrando hallazgos típicos y comprobatorios de PFPT.

ABSTRACT: The pigmented fungiform papillae of the tongue, whose acronyms are PFPT, of the English Pigmented fungiform papillae of the tongue, is an asymptomatic, nonprogressive condition that occurs in dark-skinned people, in which the fungiform papillae change their color natural pink, to a range of brown to black. The aim of our study is to report and describe the clinical, dermatoscopic and histological characteristics of the PFPT for the first time in a series of Ecuadorian patients. A simple prospective study at the Garzón Dermatological Specialty Center, a two-year period. The inclusion criteria was lingual change of color, demographic, clinical data were collected; photographs, dermatoscopy, and biopsy, for staining with hematoxylin-eosin and FontanaMason. We examined 8,640 patients, 15 (12 women, 3 men) were diagnosed with PFTP. The range of age was 31 years, all were mestizos, with skin phototype predominant III and IV. The range of evolution time in years was 5.8. None had family or personal precedents related to the pathology. The clinical evaluation showed that the pattern of distribution according to the Holzwanger classification in the great majority was type II (13/15). In all cases, the dermatoscopy and histology were specific, demonstrating typical and evidential findings of PFPT.

Lingual amyloidosis associated to long-term hemodialysis: Two case reports.

The ß2-microglobulin amyloidosis is a newly recognized type of systemic amyloidosis, which occurs in patients with chronic renal failure undergoing long-term hemodialysis. The dialysis-related amyloidosis (DRA) of the tongue is an uncommon and late complication, which occurs in patients undergoing chronic hemodialysis for more than 20 years. The aim of this paper is to report two cases of lingual amyloidosis associated with long-term hemodialysis. Two patients were referred to the Oral Medicine Clinic due to the presence of multiple nodules on the tongue and macroglossia. The patients had history of chronic renal failure and had been dialysed for more 20 years with bio-incompatible cellulose membrane. Biopsies of the lesions were performed and the histopathological features and Congo red stain revealed oral amyloidosis. Both patients remain in clinical follow-up after 12 and 6 months, respectively. Recognition of the amyloidosis tongue lesions can minimize the complications caused by this condition such as obstruction of the upper airways, making it imperative to carry out early treatment. Furthermore, in patients with oral amyloidosis, lesions in areas of trauma may ulcerate and cause pain and dysphagia becoming necessary periodic surgical removal and regular follow-up of these patients.

Resolución de úlcera sublingual en paciente con parálisis cerebral: informe de un caso / Resolution of sublingual ulcer in cerebral palsy patient: case report

Male patient, 19 years old, diagnosed with severe mixed tetraparesis, predominantly spastic, secondary to severe perinatal asphyxia, West syndrome and intellectual disability, GMFCS V. october 2012 presents a 1 cm white-colored and painless ulcer on the ventral surface of the tongue, showing indurated borders, and no signs of palpable lymph nodes, compatible with Riga-Fede disease. the patient is referred to the commune hospital. August 2013 attends a dental control presenting the ulcer with similar conditions, awaiting treatment from the hospital. April 2014 he returns for a health control in the same conditions. this time a non invasive intervention is performed, covering the incisal edges of teeth 3.1 and 4.1 with composite resin. A two weeks later control shows a smaller ulceration. By June 2015 the ulcer has healed completely, and stayed healed until january 2016. the mother reports decreased tongue protrusion in frequency and intensity since January 2015. Conclusion: Sublingual traumatic ulcer can be healed by eliminating the cause of the trauma, using a non invasive technique.

Paciente de sexo masculino, 19 años de edad con diagnóstico de tetraparesia mixta severa de predominio espástico, secundaria a asfixia perinatal, síndrome de West y discapacidad intelectual, GMFCS V. En octubre de 2012 presenta úlcera en cara ventral de la lengua, de 1 cm de diámetro aproximadamente, de bordes indurados, fondo de color blanquecino e indolora, sin ganglios palpables, compatible con diagnóstico de enfermedad de Riga Fede. Se deriva en esa oportunidad al hospital de su comuna. En agosto de 2013 asiste nuevamente a control presentando la úlcera con similares condiciones, en espera de tratamiento en el hospital. En abril de 2014 vuelve a control en iguales condiciones. En esta oportunidad se realiza intervención no invasiva, recubriendo superficies incisales de dientes 3,1 y 4,1 con resina compuesta. En control a las dos semanas se observa la úlcera más pequeña. En junio de 2014 se observa ausencia de úlcera, situación que persiste hasta control de enero de 2016. La madre manifiesta que protrusión lingual ha disminuido en frecuencia e intensidad desde enero de 2015. Conclusión: La úlcera sublingual de origen traumático puede ser resuelta eliminando la causa del trauma, de forma no invasiva.

Delayed tongue necrosis simultaneous with bilateral osteoradionecrosis of the jaw secondary to head and neck irradiation.

We present a rare case of massive tongue necrosis occurring simultaneously with bilateral osteoradionecrosis (ORN) of the jaw in a patient with a history of treatment, including surgery and postoperative radiotherapy, for a retromolar trigone carcinoma 8 years earlier. There is a distinct possibility that the extractions and administration of local anesthesia with a vasoconstrictor contributed to the onset of ORN; together, these events may have influenced the blood supply to the tongue. A glossectomy was performed after hyperbaric oxygen therapy. One month after the surgical procedure, the patient responded satisfactorily to the treatment, showing significant improvement in speech and oral food intake, as well as significant decrease in lingual pain. Although the simultaneous occurrence of these oral complications is rare, the practitioner must be aware of the factors that instigate ORN and compromise vasculature as well as the clinical signs of tongue necrosis. Additionally, the possibility of tongue necrosis secondary to irradiation of the head and neck should be taken into consideration when an irradiated patient undergoes tooth extractions under local anesthesia with agents containing epinephrine.

A 47-year-old man with tongue swelling.

Intermittent tongue angioedema can be the initial presentation of several disorders including angiotensin-converting-enzyme inhibitor induced angioedema and hereditary angioedema. Persistent angioedema on the other hand, can be associated with amyloidosis, tumors, thyroid disorders and acromegaly. We present a case of intermittent episodes of tongue swelling progressing to macroglossia.

Topical application of Aloe vera and vitamin E on induced ulcers on the tongue of rats subjected to radiation: clinical and histological evaluation.

OBJECTIVE: The aim of this study is to assess the effect of two types of antioxidants, vitamin E (VE) and Aloe vera (AV), on healing of induced oral lesions after radiation in a murine model by clinical and histological analysis. METHODS: The animals were randomly divided into three groups of 12 animals each (400 mg VE, 70 % AV and control) and two time periods (5 and 7 days). They were irradiated with a single dose of 30 Gy, and after 24 h, a lesion was produced on the ventral tongue of each animal. The products were applied daily in their respective group until euthanasia. RESULTS: On clinical analysis, there was a higher frequency of lesions in the animals of the control group at both periods. The area of the lesions was also greater in the control group compared with the groups AV and VE (5 days p = 0.006; 7 days p = 0.002). On microscopic analysis, the degree of inflammation differed between the study groups and experimental periods. At 5 days, the statistical difference was not significant among the groups evaluated, but at 7 days, animals in the control group showed intense inflammation, while those in groups VE and AV exhibited mild to moderate inflammation (p = 0.002). CONCLUSION: The results suggest that VE and AV contributed to the decrease in inflammatory response and healing of the lesions induced on the tongue of rats subjected to radiation.

Influence of bruxism and splint therapy on tongue pressure against teeth.

OBJECTIVES: This study estimated the maximum tongue pressure (MTP) against teeth in bruxers, and verified the effect of a stabilization splint. METHOD: Forty subjects (mean age: 26.57 years) were assigned to two groups (n520): bruxers and nonbruxers. Sleep bruxism was diagnosed by self-report, presence of tooth wear and tongue indentation, and by using a BiteStrip2 device. Maximum tongue pressure was measured by intraoral appliances containing pressure sensors. After baseline evaluation, bruxers received a stabilization splint and non-bruxers received a palatal splint, both for nocturnal use. Maximum tongue pressure was reassessed after 30 and 60 days. Data were submitted to repeated-measures ANOVA and Tukey test (Pv0.05). RESULTS: Bruxers showed an increased MTP against teeth (Pv0.05). Splint therapy decreased MTP for bruxers and non-bruxers. DISCUSSION: Bruxers present tongue indentations or ulcerations; however, there is no evidence on their MTP. Further research should focus on therapies to avoid tongue ulcerations in bruxers.

[Intraoral paratrichosis after autograft]. / Paratricosis en cavidad oral tras autoinjerto.

BACKGROUND: Radial graft is one of the optimal treatments for reconstruction after tongue cancer, but it is not free of side effects. Hypertrichosis over the graft, causing an intraoral paratrichosis, might downgrade the quality of life and even require further interventions. CLINICAL CASE: The case is presented of a 58 year-old man, who developed hypertrichosis after surgery for tongue carcinoma. DISCUSSION: The therapeutic options, from choosing a graft from less hairy areas to laser depilation or de-epithelisation of the graft are discussed. CONCLUSIONS: Intraoral paratrichosis is a serious complication that can produce dysphagia, nauseas, and vomiting, and can evolve into mal nutrition and produce an important decrease in life quality of our patients.

Piercings en cabeza y cuello / Head and neck piercings

Los piercings corporales son una práctica habitual en la sociedad actual, y consisten en la perforación de un tejido para insertar un aro u otro elemento ornamental. Este procedimiento implica la creación de una puerta de entrada cutánea o mucosa que puede permitir el ingreso de microorganismos, además de la posible formación de cicatrices anómalas. Asimismo, el aro puede predisponer a otras complicaciones como reacciones de hipersensibilidad, desplazamientos o aspiración. Debido a esta amplia gama de posibles complicaciones, los piercings debieran realizarse en establecimientos autorizados, por personal entrenado y bajo técnica aséptica. El personal médico debe conocer el diagnóstico y manejo de estas complicaciones, y dado que la mayoría de las perforaciones se realizan en el área de cabeza y cuello, el otorrinolaringólogo debiera estar especialmente familiarizado con los aspectos médicos de ésta práctica.

Body piercing is considered a normal practice in today's society. It consists in the perforation of corporal tissue to insert a ring or other ornamental object with an esthetic purpose. This procedure implicates the creation of a cutaneous or mucosal entry point that could allow pathogenic microorganisms into the body, and could also promote the formation of anomalous scar tissue. Other complications, such as hypersensitivity reactions, displacement and aspiration, have been reported. Due to this wide range of possible complications, body piercings should be performed only in authorized establishments, by trained staff and under supervised aseptic technique. Health care providers should be aware about the diagnosis and treatment of these complications. Given that most piercings are placed in the head and neck area, otolaryngologists should be especially familiarized with the medical aspects of this practice.

Life-threatening expansive sublingual hematoma: a stab wound with lingual artery injury.

Vascular injuries are a constant risk in facial trauma, although bone and soft tissues of the face have provided some protection to the larger blood vessels. However, penetrating injuries usually do not have this type of protection and can damage significant vascular arteries. This article presents a case of a stab wound, which led to airway obstruction arising to a large sublingual hematoma due to lingual artery injury. A healthy 44-year-old man was stabbed in the submandibular region and admitted with an airway obstruction. He was subjected to an emergency tracheotomy and evolved with progressive sublingual edema. Computed tomography (CT) angiography showed a left lingual artery injury with the formation of an expansive hematoma. The CT angiography findings helped to identify the cause of the hematoma and guided the surgery to drain the hematoma after ligation of the lingual artery. The treatment was safely performed as planned and evolved uneventfully. The patient recovered fast and well and presented normal functions 6 months after the treatment. This surgical technique is an effective method for treating such injuries because it can be safely performed when guided by CT angiography. The authors argue that the demand for vascular lesions should be routine in patients who have facial trauma.

Pyogenic granuloma on the tongue: a pediatric case report.

Pyogenic granuloma (PG) is a rare, benign, vascular, hyperplasic, soft tissue lesion caused by diverse factors, including traumatic injuries. This article presents a case involving the surgical removal of PG on the tongue of a 4-year-old boy who had difficulty with speech and eating because of the tongue lesion. The parents reported that the child had the habit of nibbling on and sucking his tongue. The lesion was excised and histopathological analysis confirmed the diagnosis of PG; however, because the child continued to nibble and suck on his tongue, the lesion recurred. A second surgery was performed with the same histopathological diagnosis. At a one-year follow-up, the child had ceased his tongue habits, and no recurrence was seen.

Sialolithiasis in the duct of submandibular gland: a case report in patient with epidermolysis bullosa.

AIM: To describe the options of treatment to remove a sialolith associated with the submandibular gland duct in a patient with epidermolysis bullosa (EB). BACKGROUND: Treatment of patients with EB is very complex and involves a multidisciplinary team. This condition is characterized by a spectrum of blistering and mechanical fragility of the skin. One main feature of this disease is the esophageal constriction and possible constriction to the submandibular duct. This alteration may induce the formation of calculi in this duct, which is called sialolith. Once the sialolith obliterates the trajectory of the duct this will lead to a sialolithiasis. The calculi have to be removed. CASE REPORT: Seventeen years old female patient with dystrophic EB developed a sialolith at the submandibular duct. She has a limited mouth opening and her tongue was collapsed with mouth floor. The first choice of treatment was the lithotripsy, once this procedure is less invasive and a surgical remove could worse the collapsed tongue. She was with acute pain and with a great augmentation in the submandibular area. Once the patient was debilitated and has difficult to swallow she invariably needed to be hospitalized in order to receive intravenous medication. During the hospitalization the sialolith could be seen through the opening of the duct and the calculi was removed with local anesthesia. CONCLUSION: The treatment of sialolithiasis usually does not present major challenges, nevertheless if the sialolithiasis is associated with EB, the treatment became an extremely challenge. In this particular case the option of treatment was the less invasive. CLINICAL RELEVANCE: This case report has an enormous clinical relevance once there is no protocol to treat patients with EB and buccal diseases.

Sublingual traumatic ulcerative lesions caused by the eruption of first primary mandibular molars: a case report / Lesión ulcerativa traumática sublingual causada por la erupción del primer molar temporal: reporte de un caso

Traumatic ulceration of the ventral surface of the tongue is an uncommon condition in infants and toddlers, which is often associated with natal and neonatal teeth in newborns. The aim of this report is to present a case of bilateral ulcerative ulcers on the ventral surface of the tongue caused by the eruption of first primary mandibular molars in a 14-month-old male child. The child was able to point to the lesions and also refused to consume certain beverages and foods, which favored an early diagnosis. Clinical treatment consisted of manual smoothing of the sharp edges of both mandibular first molars and a home regimen of lidocaine hydrochloride solution (Xylocaine®, Astra) and a steroid solution of triamcinolone acetonide (Omcilon ­ A, Brystol-Myers) for symptoms relief and lesion healing, respectively. Complete healing of both lesions and normal feeding were both observed at a one-week follow-up exam. Early detection of the lesions and parental compliance with recommended home regimens were key factors for a successful treatment outcome, since untreated cases of tongue ulcerative lesions may evoke feeding difficulties and failure to thrive. Although there are several reports of ulcerative lesions on the tongue caused by dental eruption, they are usually associated with the eruption of mandibular primary incisors (Riga-Fede disease). However, there are no reports of ulcerative lesions caused by other primary teeth.

La ulceración traumática de la superficie ventral de la lengua es una condición poco común en bebés y niños pequeños, la cual se asocia a menudo con los dientes natales y neonatales en los recién nacidos. El objetivo de este reporte es presentar un caso de úlceras bilaterales en la superficie ventral de la lengua causada por la erupción de los primeros molares mandibulares temporales en un infante de sexo masculino de 14 meses de edad. En niño fue capaz de señalar las lesiones y se negó a consumir ciertas bebidas y alimentos, lo que favoreció un diagnóstico precoz. El tratamiento clínico consistió en el suavizado manual de los bordes afilados de los primeros molares inferiores y un régimen casero de solución de clorhidrato de lidocaína (Xilocaína®, Astra) junto a la solución esteroidal de acetónido de triamcinolona (Omcilon - A, Brystol-Myers) para el alivio de los síntomas y curación de la lesión respectivamente. La curación completa de ambas lesiones y la alimentación normal se observó en una semana del control de seguimiento. La detección temprana de las lesiones y el cumplimiento de los padres con los regímenes caseros recomendados, son factores claves para un resultado exitoso del tratamiento, ya que los casos no tratados de estas lesiones ulcerosas pueden provocar dificultades en la alimentación y una evolución inadecuada. Aunque existen varios informes de lesiones ulcerosas en la lengua causada por la erupción dental, que se asocian generalmente con la erupción de los incisivos mandibulares temporales (enfermedad de Riga-Fede). Sin embargo, no existen informes de lesiones ulcerosas causadas por otros dientes temporales.

Síndrome de Richner-Hanhart e suas manifestações otorrinolaringológicas: relato de caso / Richner-Hanhart Syndrome and its otorhinolaryngologic manifestations: case report

INTRODUÇÃO: A Síndrome de Richner-Hanhart se caracteriza pela ausência em grau variável de porções distais de uma ou mais extremidades, associada à micrognasia e microglossia severa. A etiologia desta rara síndrome permanece desconhecida. RELATO DE CASO: Neste artigo relatamos o caso de um paciente com Síndrome de Richner-Hanhart que apresentava ausência de terço anterior da língua, micrognatia, malformações dos pododáctilos e quirodáctilos, de pés e mãos, além de desvio de septo nasal para esquerda. COMENTÁRIOS FINAIS: O tratamento consiste em dieta com restrição de tirosina e acompanhamento contínuo com otorrinolaringologista e fisioterapeuta.

INTRODUCTION: Richner-Hanhart Syndrome is characterized by the absence in a variable degree of distal portions of one or more extremities, in association with micrognathia and severe microglossia. The etiology of this infrequent syndrome remains unknown. CASE REPORT: In this article, we report the case of a patient with Richner-Hanhart Syndrome, who showed an absence of the back third of the tongue, micrognathia, malformation of fingers and toes, as well as nasal septum deviation to the left. FINAL COMMENTS: The treatment comprises a tyrosine-restricted diet together with a continuous follow-up with an otorhinolaryngologist and a physiotherapist.