Psoriasis in childhood and adolescence

Psoriasis is a chronic, immunologically mediated, recurrent and universal inflammatory disorder. Approximately one third of adults refer onset before 16 years of age. The sooner the onset, the worse is the prognosis. In children, lesions may be physically disfiguring, leading to psychological impairment and evident loss of quality of life. Systemic therapy used in psoriasis, as well as phototherapy, has limited use in children due to accumulative effects of drugs, low acceptance, and risk of teratogenicity. In this section, we discuss the main clinical aspects of psoriasis in childhood and adolescence. differential diagnosis, therapeutic options, and prognosis.

Idiopathic Toe Walking A Kinematic and Kinetic Profile

Purpose: The differential diagnosis in children who walk on their toes includes mild spastic diplegia and idiopathic toe walking (ITW). A diagnosis of ITW is often one of exclusion. To better characterize the diagnosis of ITW, quantitative gait analysis was utilized in a series of patients with an established diagnosis of ITW. Study Design: Patients with an established diagnosis of ITW were analyzed by quantitative gait analysis. Data were recorded as each subject walked in a self-selected toe-walking pattern. The subject was then asked to ambulate making every effort to walk in a normal heel-toe reciprocating fashion. Data were collected to determine if this group of idiopathic toe walkers was able to normalize their gait. Data sets were compared with each other and with historical normal controls. Results: Fifty-one neurologically normal children ( 102 extremities) with ITW were studied in the Motion Analysis Laboratory at a mean age of 9.3 years. In the self-selected trials, significant deviations in both kinematics and kinetics at the level of the ankle were identified. Disruption of all 3 ankle rockers and a plantar flexion bias of the ankle throughout the gait cycle were most commonly seen. When asked to attempt a normal heel-toe gait, 17% of the children were able to normalize both stance and swing variables. In addition, 70% were able to normalize some but not all of the stance and swing variables. Conclusion: Quantitative gait analysis is an effective tool for differentiating mild cerebral palsy from ITW. Kinematic and kinetic distinctions between the diagnoses are evident at the knee and ankle. The ability to normalize on demand at least some of the kinematic and kinetic variables associated with toe walking is seen in most children with ITW.

Psychiatric diagnoses of patients with psychogenic non-epileptic seizures

Objective: Our purpose was to present and discuss the psychiatric diagnoses of patients who presented psychogenic non-epileptic seizures (PNES) during video-electroencephalographic monitoring (VEEG). Methods: Out of 98 patients, a total of 28 patients presented PNES during the diagnostic procedure. In those cases in which the PNES that occurred during VEEG were validated by clinical history (clinical validation), and by showing the recorded event on video to an observer close to the patient (observer validation), was defined psychogenic non-epileptic seizure disorder (PNESD). Psychiatric diagnoses were made according to DSM-IV. Results: In 27, psychogenic non-epileptic seizures disorder was diagnosed. Fourteen patients presented only with psychogenic non-epileptic seizure disorder, 13 with both psychogenic non-epileptic seizures disorder and epilepsy, and one patient with epilepsy only. Psychiatric diagnoses were: 17 (63%) patients with conversion disorder, five (19%) with somatization disorder, two (7%) with dissociative disorder NOS, two (7%) with post-traumatic stress disorder and one (4%) with undifferentiated somato-form disorder. Conclusions: Dissociative-conversion non-epileptic seizures are the most frequent finding, representing the pseudoneurological manifestation of mental disorders that have these symptoms as a common feature. Provisionally, they may be defined as dissociative-conversion non-epileptic seizure disorders. (C) 2007 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Rare sellar lesions

Sellar masses are associated most commonly with pituitary adenomas. Many other neoplastic, inflammatory, infectious, and vascular lesions, however, may affect the sellar region and mimic pituitary tumors. These lesions must be considered in a differential diagnosis. This article describes the characteristics of rare sellar masses that provide clues to their differential diagnosis.

Placental transmogrification of the lung presenting as giant bullae with soft-fatty components

A 44-year-old man presented with progressive dyspnea and a previous pneumothorax. Chest CT scan showed a mediastinal shift due to giant bullae containing soft tissue and fatty components in the left lower lung Lobe, and a right upper lung lobe partially collapsed. The pulmonary function tests revealed forced vital capacity (FVC) 53% (of the predicted) and forced vital capacity in 1 s (FEV1) 52%. Then, resection of the lower lobe was performed with intention to prevent other pneumothoraxes and to revert the upper lobe collapse. The pathological examination showed a placental. transmogrification of the lung (PTL). One month after the surgery, the patient was asymptomatic, the pulmonary function tests normalized and the upper lobe was well expanded. In conclusion, we described the first CT finding of soft tissue and fatty components within the PTL-related bullae, and the PTL should be considered in the differential diagnosis of pulmonary lesions with soft-fatty and air components. (c) 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Hemangioma of the Rib

An asymptomatic 48-year-old woman presented to our hospital with a tumor of the rib incidentally diagnosed on a chest roentgenogram. The patient was investigated and underwent tumor resection of the chest wall. The pathologic study revealed that it was cavernous hemangioma. This tumor of the bone is a distinctly uncommon benign vascular tumor, generally occurring in the spine or skull. Hemangiomas involving the rib are even more rare, with only 22 cases described in the literature. However, we suggest that this tumor of the rib should be considered in the differential diagnosis, principally in asymptomatic patients. (Ann Thorac Surg 2011;91:595-6) (C) 2011 by The Society of Thoracic Surgeons

Ossifying fibroma: A rare cause of orbital inflammation

Purpose: To describe the clinical and radiologic features of 4 cases of ossifying fibroma affecting the orbit and to review the literature on orbital involvement by the tumor. Methods: Small case series. Results: Four patients (3 children and 1 adult) with ossifying fibromas invading the orbit were examined. Two of the 3 children were examined for ossifying fibromas on the orbital roof. One had the psammomatoid form of the disease and the other the trabecular variant. Despite striking differences in the histologic pattern and in the radiologic appearance of the lesions, both children displayed a significant degree of orbital inflammation mimicking orbital cellulitis. The third child and the adult patient had the orbit involved by trabecular ossifying fibromas invading the orbital floor. The tumor of the adult clearly originated in the maxilla, filled the maxillary sinus, and eroded the orbital floor. The tumor of the third child occupied the maxillary, ethmoid, and sphenoid sinuses. In both cases, the clinical presentation was painless eye dystopia and proptosis. Conclusions: Regardless of the histologic pattern (trabecular or psammomatoid), ossifying fibromas can induce a substantial degree of orbital inflammation in children and must be included in the differential diagnosis of acute orbital inflammation during childhood.

Fungal infection by Paracoccidioides brasiliensis mimicking bone tumor

Paracoccidioides brasiliensis infection causes a systemic mycosis originally described in Latin America but with Current reports of worldwide distribution. The clinical presentation of paracoccidiodomycosis as an isolated long-bone lesion in children is quite unusual. This article describes a 10-year-old male with a lytic femoral bone lesion caused by P. brasiliensis infection that was first suspected of being of neoplasic etiology. The text also emphasizes the importance of including endemic fungal infections in the differential diagnosis of bone lesions.

The immunohistochemical profile of oral inflammatory myofibroblastic tumors

Objective. The aim of this study was to demonstrate the immunohistochemical profile of oral inflammatory myofibroblastic tumors (IMTs) along with morphologic analysis. Study design. Three cases diagnosed as oral IMTs were selected to compile an immunohistochemical panel constituted by calponin, caldesmon, Bcl-2, desmin, fibronectin, CD68, Ki-67, S100, anaplastic lymphoma kinase (ALK), alpha-smooth muscle actin, cytokeratins AE1/AE3, muscle-specific actin, CD34, and vimentin. An oral squamous cell carcinoma with a focal area of desmoplastic stroma was used as control for the stained myofibroblastic cells. Results. All oral IMTs were positive for calponin, revealing a strong and diffuse expression in the spindle-shaped cells. The lesions were also positive for vimentin (3/3), fibronectin (3/3), alpha-smooth muscle actin (3/3), and muscle-specific actin (1/3) and negative for h-caldesmon, Bcl-2, desmin, CD68, Ki-67, S100, ALK, cytokeratins AE1/AE3, and CD34. Conclusions. Within the results encountered, the present panel should be of great assistance in the diagnosis of oral IMTs. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011; 111: 749-756)

The neural histogenetic origin of the oral granular cell tumor: An immunohistochemical evidence

Aims: Granular cell tumor (GCT) is a rare neoplasm that can appear in any site of the body, but most are located intraorally. Its histogenetic origin remains unclear. This report analyzes the immunoprofile of 15 cases of granular cell tumors, occurring in 13 women and 2 men and the lesions were located on the tongue or upper lip. Patient age ranged from 7 to 52. Methods: The patients demographic data and the cytological and architectural features of the lesions were analyzed in oral GCTs (n = 15). The lesions were also submitted to a panel of immunohistochemical stains with antibodies against S-100, p75, NSE, CD-68, Ki-67, Synaptofisin, HHF-35, SMA, EMA, Chromogranin, Progesterone, Androgen and Estrogen. Results: Among the fifteen cases analyzed, the most common location was the tongue (84.6%). Histologically, the tumors exhibited cellular proliferation composed mainly by polygonal cells presenting an abundant granular eosinophilic cytoplasm. The nuclei were central, and the cell membranes were moderately clear. No mitotic figures were observed. The immunohistochemical analysis showed positivity in all cases for S-100, p75, NSE and CD-68, and no immunoreactivity for Ki-67, Synaptofisin, HHF-35, SMA, EMA, Chromogranin, Progesterone, Androgen and Estrogen. Conclusion: The immunoprofile of granular cell tumors showed nerve sheath differentiation - lending support to their neural origin - and helping to establish a differential diagnosis between this lesion and other oral granular cell tumors, whether benign or malignant.

Solitary Neurofibroma of the Temporal Bone

Neurofibroma is a benign peripheral nerve sheath tumor that can be occasionally found in the head and neck region as multiple lesions associated with neurofibromatosis type 1 (NF-1) or as a solitary tumor. The real frequency of isolated neurofibromas not associated with NF is uncertain, and lesions in the temporal region are extremely rare. The aim of the current article was to report an unusual case of solitary neurofibroma localized in the temporal and infratemporal regions with 10 years of evolution in a female patient without any other manifestation or familiar history of NF-1. The patient underwent surgical treatment for complete excision of the lesion, and the 2-year follow-up revealed no signs of recurrence.

Follicular lymphoma in the palate with clinical appearance similar to salivary gland tumors

Intraoral presentation of follicular lymphoma is rare, and only three cases in the palate have been reported to date. The present case report describes an uncommon case of follicular lymphoma affecting the palate. The clinical aspect was similar to salivary gland neoplasm, and an incisional biopsy was important to establish the correct diagnosis and consequently to plan the treatment. Also discussed is the differential diagnosis among follicular lymphoma, mucosa-associated lymphoid tissue lymphoma, and follicular lymphoid hyperplasia with regard to the histopathologic and immunohistochemical features. (Quintessence Int 2010; 41: 661-663)

Intravascular papillary endothelial hyperplasia: Report of 4 cases with immunohistochemical findings

Intravascular papillary endothelial hyperplasia (IPEH) is a benign endothelial proliferation, usually intravascular, that may mimic angiosarcoma. In this report, four new cases of IPEH involving the oral region are described. The affected sites were the lower lip, labial comissure and the submandibular region. After clinical evaluation, the complete removal of the lesions showed a circumscribed and soft mass. Histologically, the major feature was a reactive proliferation of endothelial cells composed of small papillary structures with hypocellular and hyalinized cores arising in an organized thrombus. Immunohistochemical staining for CD34 was strongly positive in endothelial cells. Vimentin and laminin immunolabelling were also consistent with a vascular origin. In order to verify the proliferative potential of the lesions, the Ki-67 antibody was used, revealing low percentage of labeled cells (<20%). No immunoreactivity for GLUT-1 was observed. Since the complete removal is curative, no additional treatment was necessary, and no signs of recurrence had been observed until now. Due to the particular features of IPEH, it is important for pathologists and clinicians to become familiar with this lesion. Additionally, the specific histological arrangement, including the absence of cellular pleomorphism, mitotic activity and necrosis, represents a guide to help in the differential diagnosis. Moreover, the vascular origin and the proliferative index should be assessed by immunohistochemistry in order to provide an accurate diagnosis.

Nasopharyngeal angiofibroma. surgical approach using a transoral Le Fort I osteotomy following embolization: a case report

Nasopharyngeal angiofibroma (NA) is a rare vascular benign nonencapsulated neoplasm, characterized by local aggressiveness and destructive potential, typically diagnosed in adolescent males. We report a case of NA affecting a 15-year-old male that presented with a persistent nasal obstruction and epistaxis with 1 year of evolution. Clinical and radiological patterns pointed out a differential diagnosis of NA. Arteriography demonstrates the vascular support of the tumor and evinces the embolization of the internal maxillary artery. The surgical approach was procedure by Le Fort I osteotomy exposing the tumor and promoting easy access for posterior removal. The surgery was carried out without hemorrhagic problems. The maxilla was fixed in the original position with 4 L-shape plaques. Histopathological findings supported the diagnosis of NA. The patient presented after 8 months of postoperative follow-up, without clinical signs of recurrence or residual tumor and without palatal or maxillary teeth paresthesia.

Peroxiredoxin I is differentially expressed in multiple myelomas and in plasmablastic lymphomas

BACKGROUND: Plasmablastic lymphoma (PBL) and multiple myeloma (MM) are B cell-derived malignancies that share many morphologic and immunophenotypic traits, making the differential diagnosis particularly complicated. We have recently demonstrated that peroxiredoxin I (PrdxI) is expressed in plasma cells but not in B lymphocytes, suggesting that its expression is development-associated. AIM: To analyze PrdxI expression in PBL and in MM in order to study its utilization as an additional diagnostic molecular tool. METHODS AND RESULTS: Eight cases of PBL and nine of MM were studied by immunohistochemistry. We have demonstrated that PrdxI expression is closely connected with the immunoglobulin production capacity of the cells, which means high in MM, but absent in PBL cases, except one, wherein few cells were stained. CONCLUSIONS: We hypothesize PrdxI as a component of the unfolded protein response (UPR), an adaptive pathway essential for plasma cell differentiation. As we have not detected immunoglobulin in our PBL cases, we suggest that UPR was not activated in the cells, accounting for the impediment of the developmental process, and for the inhibition of PrdxI expression observed. PrdxI could be considered an additional plasma cell functional marker and could also be speculated as a therapeutic target in the treatment of MM. Oral Diseases (2008) 14, 741-746