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BACKGROUND: Proteus Syndrome (PS) is a rare hamartoneoplastic disorder consisting of a disproportionate and asymmetric overgrowth of body parts, leading to severe body and face disfigurement. Individuals with PS frequently need to undergo a number of surgical procedures including Otolaryngology interventions. Their peculiar anatomy turns even the most straightforward intervention to a challenging one. CASE DESCRIPTION: A 31-year-old adult male with PS presented with recurrent epistaxis and nasal obstruction. A nasal mass was found during the physical examination, which was surgically removed under general anesthesia. Histology revealed juvenile angiofibroma. CONCLUSION: This is the first reported case of a patient with PS presenting with juvenile nasopharyngeal angiofibroma. Although vascular malformations may appear in these patients, juvenile nasopharyngeal angiofibroma should be taken under consideration when PS patients present with recurrent epistaxis. HIPPOKRATIA 2017, 21(3): 147-149.

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OBJECTIVE: Human leukocyte antigen-DR (HLA-DR) has been implicated in eutopic and ectopic glandular epithelial cells in endometriosis. We investigated the expression of HLA-DR in endometriotic and adenomyotic tissues within the stromal and glandular cells. Moreover, we correlate the HLA-DR expression according the transvaginal ultrasonography findings. METHODS: We studied operative and pathologic reports of 113 women who underwent laparoscopic or laparotomy treatment of endometrioma or adenomyosis. Tissues from 51 women with endometrioma and 62 women with adenomyosis were retrospectively evaluated. The distribution and intensity of the HLA-DR immunostaining was assessed using electron microscopy. Pathologic finding of the uterine junction zone and the size of endometrioma were evaluated with the laparoscopic results and the ultrasound findings. RESULTS: In adenomyosis tissues, the percentage of HLA-DR cells expression was significantly higher in stromal cells (83.9%) compared to glandular cells (25.8%), (p<0.001). The number of HLA-DR-positive endometriotic glandular cells was significantly higher than the total glandular adenomyotic cells (p<0.005). HLA-DR-positive cells was significantly different between stromal (p<0.016) and glandular cells (p<0.044) in each side of endometrioma. Finally, HLA-DR-positive percentage cells were significantly more frequent in the secretory phase than the proliferative in stromal and glandular cells in both groups. CONCLUSION: HLA-DR antigen expression in endometrium and adenomyotic tissues. However, HLA-DR expression is distributed preferentially in glandular epithelial cells in endometrioma and in the adenomyotic stroma. In both groups the HLA-DR expression was significantly higher in the secretory phase than the proliferative or glandular and stroma cells. Larger perspective studies are needed to establish the expression of HLA antigens in immune reactions which occur in adenomyosis and endometriosis.

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BACKGROUND: Poikiloderma of the face and neck (Civatte) has not been studied in depth for decades, especially as far as the histopathology is concerned. MATERIAL AND METHODS: We studied 50 consecutive patients with poikiloderma of Civatte (PC). Their evaluation included: history, physical examination, lesional skin biopsy and histological examination of sections stained with hematoxylin-eosin, PAS, Fontana-Masson, acid orcein Giemsa for elastic fibers and toluidine blue for mast cells. In 10 randomly selected subjects, a second skin biopsy was performed and specimens were examined under the electron microscope. RESULTS: There were 34 females (68%) and 16 males. The mean age at diagnosis was 47.8 years for females and 61.7 years for males. Histological examination revealed an atrophic (62%), flattened (84%) epidermis with hyperkeratosis (92%) and occasional follicular plugging (34%). In some cases, mild hydropic degeneration of the basal cell layer was evident (46%). Melanin was irregularly distributed in the lower epidermis (94%), and melanophages were often present in the dermis (92%). The most prominent and constant feature (100%) was solar elastosis of the papillary dermis. The blood vessels were almost invariably dilated (96%) with a mild perivascular lymphohistiocytic infiltrate (78%), sometimes with plasmacytes (56%). At the ultrastructural level, the epidermis showed only minor changes. The dermoepidermal junction was intact. The most constant findings were swelling and disruption of the collagen fibers as well as focal degeneration of the collagen bundles. Occasionally, several vacuolar spaces were found just under the basal lamina. Melanin-laden macrophages scattered in the dermis were also detected. CONCLUSIONS: PC shows distinct histological and ultrastructural features, supporting the theory that it represents a separate entity. The histology of PC is characteristic but not pathognomonic. On this basis, the differential diagnosis from Riehl's melanosis, poikiloderma atrophicans et vasculare and other acquired poikilodermas can be made. Ultrastructural findings were consistent with the histological findings. Changes of the dermal connective tissue (solar elastosis) predominated, providing morphological evidence for the role of ultraviolet radiation in the pathogenesis of PC.

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OBJECTIVES: Assessment of the histopathologic effect of transtympanic and intramuscular administration of dexamethasone in an in vivo experimental animal model of middle-ear mucosal inflammation. METHODS: Fifty healthy rabbits weighting 1500-1800 g were randomly divided in three groups. In 10 animals (control group), 0.5 ml of a 20 mg/ml histamine solution was injected transtympanically. In 20 rabbits (group A), histamine challenge followed a three day intramuscular pretreatment with dexamethasone at 1 mg/kg per day. In 20 rabbits (group B), histamine challenge followed pretreatment with dexamethasone via a transtympanic route (0.3 ml, 1.2 mg dexamethasone). Middle-ear mucosa was obtained for histopathology 30 minutes after histamine administration. The following parameters were assessed: inflammation, acute inflammatory component, presence of eosinophils, inflammatory activity and fibrosis. RESULTS: Oedema, vascular dilatation and congestion, inflammation, the presence of an acute (polymorphonuclear) inflammatory component, the presence of eosinophils, and inflammatory activity were found to be of a lesser grade in the mucosae of group B. All differences were found to be statistically highly significant (p<0.01) using the Mann-Whitney test. CONCLUSION: Our findings validate the transtympanic route of dexamethasone administration in counteracting histamine effects.

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We report a case of Netherton syndrome manifested as congenital ichthyosiform erythroderma, trichorrhexis invaginata and atopy, who in early adulthood developed multiple, aggressive epithelial neoplasms in sun-exposed areas of the skin, in areas with papillomatous skin hyperplasia and at the left parotid region. The occurrence of cutaneous neoplasia has been reported in syndromes with congenital ichthyosis and suggests that the underlying genetic defects may cause the development of cancer in prone patients.

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Malignant tumors of the temporal bone are rare neoplasms. Adenoid cystic carcinoma (ACC) is the most common malignant tumor of minor salivary glands, while a quite rare tumor of the major. It is considered a slow-growing tumor with a course that is characterized by local recurrences and late distant metastases to lungs (80-90%), bone and liver. When metastases occur in bone especially the spine, the course of disease is usually fulminant. Intracranial involvement can occur by direct extension, hematogenous or perineural spread and represents an advanced stage of the disease. In this paper, we present a rare case of temporal bone ACC reporting for the first time simultaneous bony and pulmonary distant metastases. The origin, the pathology, the imaging techniques, the differential diagnosis, the treatment options and the prognosis of these tumors are discussed.

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Angiosarcomas are rapidly growing malignant neoplasms arising from the vascular endothelial cells. Most common sites are the extremities and the retroperitoneal space, with only four per cent of angiosarcomas arising in the head and neck area, whilst the paranasal sinuses are one of the rarest locations. We report the case of a maxillary sinus angiosarcoma in a 72-year-old male patient. The first biopsy was inconclusive, whereas the second revealed an angiosarcoma. Medial maxillectomy was performed with subsequent external irradiation.

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A 78-year-old farmer presented with symptomless skin lesions for evaluation. Two years prior, he had developed idiopathic pulmonary fibrosis (IPF) and had been treated thereafter with oral prednisolone 20 mg/day and occasionally with colchicine 1 mg/day. On examination, erythematoviolaceous, slightly infiltrated plaques, measuring approximately 5 x 9 cm, rubbery in consistency, intermingled with pustules, sometimes eroded, with distinctive borders, were noted on the dorsum of both hands and on the extensor surface of both forearms. The lesions had developed over a 20-day period. The skin of these areas was atrophic or eroded with multiple ecchymoses (Fig. 1). The abnormal laboratory findings included an elevated white blood cell count of 17,100/mm3, with 79% neutrophils, 16% lymphocytes, and 5% monocytes, C-reactive protein of 33.15 mg/dL (normal, <0.8 mg/dL), and immunoglobulin G of 598 mg/dL (normal, 701-1545 mg/dL). Other blood and urine tests performed were within normal limits. The diagnosis of IPF was reconfirmed through radiology, high-resolution computed tomography, and spirometry, as well as bronchoscopy and bronchoalveolar lavage fluid analysis. Coexistence of presumptive pulmonary alternariosis was excluded. Hematoxylin and eosin stained sections of the excised cutaneous specimen showed focal ulceration of the epidermis adjacent to a mainly intradermal abscess cavity. Within the latter, remnants of a partly destroyed hair follicle were seen amongst degenerating polymorphonuclear leukocytes, as well as many histiocytes and a few Langhans-type multinucleated giant cells. Minute collections of polymorphonuclear leukocytes were seen in the adjacent epidermis. Periodic acid-Schiff (PAS) and Gomori's silver methenamine stains showed a multitude of broad branching fungal hyphae and large spores within the aforementioned cavity, both free and within the cytoplasm of giant cells (Fig. 2). Immunohistochemistry was performed by means of the alkaline phosphatase anti-alkaline phosphatase (APAAP) method. Sections showed that the infiltrate consisted of an almost equal number of B and T lymphocytes, whereas histiocytes and the few giant cells were labeled with anti-CD68 antibodies. Skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid Skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid structures. Biopsy material was plated on Sabourand's dextrose agar with cloramphenicol (0.05 mg/mL). After 7 days at 27 degrees C, dark, gray-white colonies with a dark brown underside appeared. Microscopic examination of the colonies revealed hyphae with typical conidia having transverse and longitudinal septa. Based on macroscopic and microscopic examination, the isolates were identified as Alternaria alternata (Fig. 3). Treatment with prednisolone was reduced to 10 mg/day and the patient received oral itraconazole (200 mg/day). This resulted in progressive improvement of alternariosis, and the lesions healed completely within 3 months, when treatment was interrupted. Two years later, there is no evidence of recurrence.

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Heparin analogues in low doses have antiproliferative and immunomodulatory properties. The aim of this study was to evaluate the effect of low-dose enoxaparin administered subcutaneously in lichen planus (LP). Eighteen patients with various types of LP were treated in an open study for 6-13 weeks. Efficacy and safety data were recorded. Complete remission was observed in 11 of 18 patients (61%) and marked improvement in two (11%). Widespread cutaneous involvement and reticulated oral LP had the best response, while in LP of the scalp the response was poor. Enoxaparin is a promising alternative therapy for various types of LP.

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A case of supraglottic laryngeal squamous cell carcinoma with sebaceous differentiation in a 68-year-old Caucasian male is described. A right cervical lymph node metastasis, excised during the laryngectomy operation, consisted exclusively of squamous cells. Despite intensive post-operative chemotherapy, the patient developed metastatic foci in the left cervical area and left pulmonary hilus and died seven months later. This is the second case report of a laryngeal squamous cell carcinoma with sebaceous differentiation in the literature.

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A case of a cerebral gliosarcoma in a 63-year old female located in the left parietooccipital area of the brain is herewith described. Histology showed the tumour to consist of two components: a gliomatous resembling glioblastoma, and a sarcomatous with a prominent myxoid intercellular substance, a feature reported only thrice before in the medical literature. We report the histochemical and immunohistochemical characteristics of our case, and compare them with those of previous case reports.

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