1,721,037 research outputs found
Treatment of skin involvement wth quinacrine and hydroxycloroquine in connective tissue diseases.
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Multidisciplinary team improved effective and global outcome in a patient with tuberous sclerosis
No full textWe present a case report of a young woman with disfiguring facial angiofibromas, affected by tuberous sclerosis and depression. Depression and embarrassment, caused by her facial lesions forced her to refuse to go out, avoiding the vital dialysis appointments. Thus an aesthetic problem created a survival problem. Physicians performed a prospective global functioning evaluation of a depressed, disfigured patient, not focusing to a parceled aspect such as anxiety, satisfaction or depression. After preliminary physician examinations the woman was operated to the face, obtaining excellent aesthetic results. Monthly a psychiatric evaluation was performed. With these synergic treatments the patient obtained a big improvement of aesthetic conditions and global functioning, she re-attended to dialysis and improved her relational approach. GAF (Global Assessment Functioning) scale score improved from the 61 to 71. We decided to report only GAF scale in order to underline the aspect of a global improvement. Previous studies about tuberous sclerosis and dermatological treatment often reported a parceled patient’s view. In conclusion, for the first time in literature we performed a global evaluation of the patient’s functioning, demonstrating how a surgical-dermatological treatment can improve the psychological, social and somatic features in a patient with tuberous sclerosis, but only with a concurrent mood evaluation and support
Urticarial vasculitis and urticarial autoinflammatory syndromes
No full textUrticaria is a frequent disorder classified as acute and chronic forms, which presents with wheals that can be associated with angioedema. Several entities may manifest with urticarial skin lesions, encompassing a heterogeneous group of conditions that have to be differentiated from ordinary urticaria. This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and Schnitzler's Syndrome, both rare multisystem forms that may masquerade as common urticaria. UV is a small-vessel vasculitis with predominant skin involvement, characterized by wheals persisting for more than 24 hours, burning rather than itching and resolving with hyperpigmentation as well as by other cutaneous manifestations including purpura, papules, vesicles, bullae and necrotic-ulcerative lesions. Histology shows a classic pattern of leukocytoclastic vasculitis, with possible presence of upper dermal edema. CAPS are classified as three distinct entities: familial cold autoinflammatory syndrome, Muckle-Wells Syndrome and chronic infantile neurological cutaneous and articular syndrome, which represent a spectrum of disorders caused by different mutations in a single gene, NLRP3 (NOD-like receptor 3). This gene encodes for cryopyrin, an inflammasome protein that activates interleukin-1β, leading to an overproduction of this pivotal proinflammatory cytokine. Histologically, urticarial lesions are generally characterized by a perivascular neutrophilic infiltrate. Unlike urticaria, neither UV nor urticarial autoinflammatory syndromes do respond to antihistamines: thus, it is important not to misdiagnose such conditions in order to give the patients specific treatments, potentially preventing serious systemic complications
Health-related quality of life in psoriasis : an analysis of Psocare project patients
No full textAim. Psoriasis is a common, chronic, immune-mediated skin disorder that may be complicated by psoriatic arthritis in up to one-third of patients. Psoriasis treatments are increasingly effective, yet more expensive, thus requiring rational decision-making on interventional priorities. The ability to perform cost-utility analyses is hindered by the lack of algorithms that allow the inference of utility measures, like QALY, from specific dermatological health-related quality-of-life (HR-QoL) measures (e.g. Dermatology Life Quality Index [DLQI]). This study aimed to assess whether psoriasis-related HR-QoL data (DLQI) could be used to obtain utility measures for use in economic analyses. Methods. Psoriasis patients attending 11 Italian Psocare project treatment centers over a 19-day period were enrolled and completed a questionnaire, including several HR-QoL scales and sociodemographic/clinical data, and underwent a clinical examination. Data were subjected to a Multiple Correspondence Analysis and multiple regression analysis to determine the contribution of single items to the HR-QoL. Results. DLQI and Psychological General Well-Being Index (PGWBI) scores were most closely correlated with the EuroQol health status index. Age and gender were considered confounding factors, while pain and arthritis contributed significantly to HR-QoL deterioration. For disease severity, the need for hospitalization and the number of examinations, but not the Psoriasis Area Severity Index (PASI), contributed to HR-QoL deterioration. Conclusion. Recent historical clinical and HR-QoL data from psoriasis patients can reproducibly define a health status index, such as the EuroQol SD-SQ, that could be used reliably to estimate QALYs for use in cost-utility analyses to compare the cost-benefit profiles of competing therapies
Biological drugs : differing mechanism of action
No full textMetastatic malignant melanoma is an incurable malignancy with extremely poor prognosis. Patients bearing this diagnosis face a median survival time of approximately 9 months with a probability of surviving 5 years after initial presentation at less than 5%. This is contrasted by the curative nature of surgical resection of early melanoma detected in the skin. To date, no systemic therapy has consistently and predictably impacted the overall survival of patients with metastatic melanoma. However, in recent years, a resurgence of innovative diagnostic and therapeutic developments have broadened our understanding of the natural history of melanoma and identified rational therapeutic targets/strategies that seem poised to significantly change the clinical outcomes in these patients. Herein we review the state-of-the-art in metastatic melanoma diagnostics and therapeutics with particular emphasis on multidisciplinary clinical management
Physicians' opinions and clinical practice patterns for actinic keratosis management in Italy
No full textWe report dermatologists' opinions and clinical practice patterns about clinical factors driving decision making in the management of actinic keratosis (AK) in Italy
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