76 research outputs found

    Non melanoma skin cancer pathogenesis overview

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    (1)Background: Non-melanoma skin cancer is the most frequently diagnosed cancer in humans. The process of skin carcinogenesis is still not fully understood. However, several studies have been conducted to better explain the mechanisms that lead to malignancy; (2) Methods: We reviewed the more recent literature about the pathogenesis of non-melanoma skin cancer focusing on basal cell carcinomas, squamous cell carcinoma and actinic keratosis; (3) Results: Several papers reported genetic and molecular alterations leading to non-melanoma skin cancer. Plenty of risk factors are involved in non-melanoma skin cancer pathogenesis, including genetic and molecular alterations, immunosuppression, and ultraviolet radiation; (4) Conclusion: Although skin carcinogenesis is still not fully understood, several papers demonstrated that genetic and molecular alterations are involved in this process. In addition, plenty of non-melanoma skin cancer risk factors are now known, allowing for an effective prevention of non-melanoma skin cancer development. Compared to other papers on the same topic, our review focused on molecular and genetic factors and analyzed in detail several factors involved in non-melanoma skin cancer

    Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature

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    Macrophage activation syndrome (MAS) is a potentially fatal condition. It is a rare complication of several autoimmune disorders, including systemic lupus erythematosus (SLE) and systemic juvenile idiopathic arthritis (sJIA). The incidence of MAS associated with SLE is about 0.9–4.6% [1]. MAS is a multifarious disease, presenting with several signs and symptoms, including high fever, hepatomegaly, splenomegaly, hemorrhagic manifestations (e.g., purpura), and dysfunction of the central nervous system, like lethargy. Furthermore, MAS is characterized by several alterations in laboratory tests, including pancytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia. MAS is classified among the group of hemophagocytic lymphohistiocytosis (HLH), which includes familial HLH and secondary HLH. Secondary HLH is triggered by several causes, including infection, drugs, malignancy, and rheumatic disorder [2]. We report a rare case of MAS that occurred as first manifestation of SLE treated with high dose intravenous methylprednisolone and oral cyclosporine

    Resolution of a Case of Pediatric Pemphigus Vulgaris Treated with Rituximab

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    Pemphigus vulgaris (PV) is an infrequent autoimmune bullous disease involving the skin and mucous membranes, which is rare in pediatrics. Although the main therapy for childhood PV are steroids, immunosuppressive drugs are often needed to control the disease. We report the case of an 11-year-old Caucasian boy who presented with a 10 months history of PV unresponsive to steroids and to intravenous immunoglobulin. The therapeutic use of rituximab allowed a long-lasting and complete remission. According to a good safe profile and to our case report, as well as the literature, rituximab may be considered an safe and efficacious treatment for PV.</p

    Nasopharyngeal Melanoma

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    Mucosal nasopharyngeal melanoma is a rare head and neck melanoma. Prognosis is poor (5-year overall survival rate of 10–30%) with high rates of metastases and local recurrence. Head and neck mucosal melanoma represents 0.8–3.7% of all melanomas and 0.03% of all neoplasms; the most commonly involved sites are the nose, paranasal sinuses, oral cavity, pharynx, and larynx. A slight female predominance has been described and the median age of presentation is 64.3. Irritants and carcinogenic substances, such as tobacco smoke and formaldehyde, seem to be related to its development. A lack of specific clinical features often leads to a late diagnosis. At an early stage, clinical features can include epistaxis, obstruction, difficulty breathing, serous otitis media, and nasal discharge; subsequently, pain, facial distortion, proptosis, and diplopia predominate the clinical pictures. Masses are mostly polyploid, friable, and bloody. They can be amelanotic or surrounded by black- or brown-pigmented dots. Nasopharyngeal melanoma resembles other common polypoid lesions; therefore, histology plays a pivotal role in confirming the diagnosis. Computed tomography, facial and total body scan, as well as magnetic resonance imaging are mandatory for a correct staging. Surgical treatment remains the gold standard. External or intranasal incision depends on tumor site and size. Sentinel lymph node biopsy is not usually performed. Neck dissection is indicated in cases of clinical and/or radiological positivity. Radiotherapy is mostly palliative, as radiotherapy lacks efficacy for mucosal melanomas. The effectiveness of target therapy and/or immunotherapy is undergoing evaluation

    Anorectal Melanoma

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    Anorectal melanoma (AM) is a rare malignancy, characterized by aggressive behavior and a poor prognosis. AM is more frequent in female patients aged over 50 years. AM accounts for 0.4–1.6% of all melanomas, 23.8% of all mucosal melanomas, and 1% of all anorectal malignant tumors. There are many theories regarding AM pathogenesis. Some consider that AM may be related to oxidative stress in the region and/or to immunosuppression. Others propose that AM may derive from Schwannian neuroblastic cells or cells of the amine-precursor uptake and decarboxylation system of the gut. Assessment of pigmented lesions located on hidden areas is difficult. Together with late and nonspecific signs and symptoms which usually occur only in conjunction with large masses, diagnosis of these mucosal melanomas is often delayed. Most frequently, the signs and symptoms are obstruction, rectal bleeding, pain, or rectal tenesmus. There are various histological variants of AM: epithelioid, spindle cell, lymphoma-like, and pleomorphic. Surgery (abdominoperineal resection or local excision) is the most effective treatment for AM; however, this is not associated with improved overall survival. Recurrence is more frequent in cases of anorectal and rectal involvement when compared with anal-only involvement. There is currently no consensus about the most appropriate systemic treatment. The efficacy of some protocols previously used in patients with cutaneous melanomas is currently being studied in mucosal melanoma

    Histology of non-melanoma skin cancers. An Update

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    Non-melanoma skin cancer (NMSC) is the most frequently diagnosed cancer in humans. Several different non-melanoma skin cancers have been reported in the literature, with several histologic variants that frequently cause important differential diagnoses with other cutaneous tumors basal cell carcinoma (BCC) is the most common malignant skin tumor, with different histologic variants that are associated with a greater or less aggressive behavior and that usually may be confused with other primitive skin tumors. Actinic keratosis, Bowen's disease, keratoacanthoma, and invasive squamous cell carcinoma (SCC) correspond to the other line of NMSC, that may have only local tumoral behavior, easy to treat and with local management (as in the case of actinic keratosis (AK), Bowen's disease, and keratoacanthoma) or a more aggressive behavior with a potential metastatic spread, as in case of invasive SCC. Therefore, histopathology serves as the gold standard during daily clinical practice, in order to improve the therapeutical approaches to patients with NMSC and to understand the distinct histopathological features of NMSC. Here, we reported the main pathological features of different non-melanoma skin cancers

    Paraneoplastic pemphigus. A trait d’union between dermatology and oncology

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    Paraneoplastic pemphigus is a rare autoimmune disease of the skin associated with neoplasm. Nowadays, the pathogenesis of paraneoplastic pemphigus is not fully understood. Due to its rarity, various criteria have been proposed for the diagnosis. For this reason, several diagnostic methods have been considered useful for the diagnosis of paraneoplastic pemphigus including indirect immunofluorescence, direct immune of fluorescence, immunoprecipitation,immunoblotting, and enzyme-linked immunosorbent assay (ELISA). However, the polymorphic clinical features and the various results of laboratory tests and pathological evaluation present a challenge for the clinician
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