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Biomedical subjects

P Iranzo

Publications and source records attributed to P Iranzo.

At least 19 recordsLinked to original sources

Dermoscopy in Gorlin-Goltz syndrome.

BACKGROUND: Gorlin-Goltz syndrome (GGS) is an autosomal dominant disorder mainly characterized by the presence of multiple basal cell carcinomas (BCC), odontogenic keratocysts of the jaw, and volar pits. This syndrome is associated with a wide spectrum of developmental anomalies and neoplasms. OBJECTIVE: To describe dermoscopic characteristics of cutaneous GGS lesions performing histopathologic correlation. MATERIALS AND METHODS: Cutaneous lesions and tumors from five patients affected by GGS were included. Clinical and dermoscopy images were obtained and excision with ulterior histopathology performed in suspicious tumors. RESULTS: With dermoscopy, BCCs can be detected in early stages by the presence of blue-gray globules in lesions less than 3 mm in diameter. In larger lesions, arborizing telangiectasia may also be present. Acral pits that are often overlooked during physical examination have a characteristic dermoscopy with red globules that are mainly distributed in parallel lines inside flesh-colored, irregular-shaped, and slightly depressed lesions. Dermoscopy improves the visualization of these pits. CONCLUSION: Dermoscopy can help in the diagnosis of the GGS as well as in the management of affected patients.

Adult↗

Efficacy of conversion to sirolimus in posttransplantation Kaposi's sarcoma.

UNLABELLED: The increased incidence of Kaposi's sarcoma (KS) in organ transplantation has been related to the KS herpes virus and the permissive effect of immunosuppressive therapy. We postulated that conversion to SRL in renal recipients with KS favored regression of KS lesions without increasing the risk of graft rejection. METHODS: In this study we performed a retrospective chart review of 7 caucasian renal transplant recipients affected by KS to determine demographic data, etiology of ESRD, immunologic risk factors, immunosuppressive treatment, KS disease follow-up, and renal function before and after SRL conversion. RESULTS: All seven patients were under calcineurin inhibitor treatment at the onset of KS which was limited to the skin, without regression despite attempts to minimize immunosuppression. After conversion to SRL, six patients showed progressive regression of KS lesions, with only hyperpigmented atrophic cutaneous lesions remaining after a mean time of 8.1 months (2-18 months). The seventh patient has completed 9 months follow-up with a near complete regression of KS lesions. One patient returned to hemodialysis after 13 months following irreversible acute renal failure not directly related to SRL conversion; in the other six, renal function was stable. The mean serum creatinine was 1.87 +/- 0.64 versus 1.74 +/- 0.68 mg/dL, pre-conversion versus the end of follow up, respectively. Mean SRL blood level was 9.2 +/- 2.0 ng/mL. CONCLUSION: After SRL conversion, patients with KS showed progressive regression without an increased risk of acute rejection. SRL offers a promising approach to the management of posttransplantation KS and probably other malignancies in organ transplant recipients.

Animals↗

[Bronchiolitis obliterans associated with paraneoplastic pemphigus: a paraneoplastic autoimmune multiorgan syndrome].

INTRODUCTION: Paraneoplastic pemphigus is a mucocutaneous disease characterized by well defined clinical and immunopathological features associated with neoplasia. Recent evidence of bronchial epithelium involvement has led to the suggestion that this process is a paraneoplastic autoimmune multiorgan syndrome. CLINICAL OBSERVATION: We report the case of a patient with lichenoid eruptions on the skin and mucous membranes who later developed progressive dyspnea. With a suspected diagnosis of paraneoplastic autoimmune multiorgan syndrome, the following diagnostic tests were performed: histology and immunofluorescence of the skin, oral mucosa, and bronchial epithelium; indirect immunofluorescence of serum; pulmonary function tests; and evaluation for an occult neoplasm. Findings of pathology and immunofluorescence confirmed the suspected diagnosis. The computed thoracoabdominal tomography revealed signs of bronchiolitis and the presence of a retroperitoneal tumor. CONCLUSIONS: Awareness of the mucocutaneous manifestations of paraneoplastic autoimmune multiorgan syndrome, and confirmation of this diagnosis by simple laboratory techniques can facilitate the early detection of occult neoplasia and forestall respiratory involvement.

Autoimmune Diseases↗

Morphoea in three siblings.

Atrophoderma of Pasini and Pierini (APP) is an uncommon form of localized morphoea that occurs as superficial, hyperpigmented plaques distributed mainly on the trunk and proximal part of the limbs. There is little information about the influence of genetic and environmental factors on disease susceptibility and expression for localized scleroderma, although APP familial cases have been reported. We report three siblings without a family history of autoimmune disease presenting cutaneous lesions suggesting morphoea (APP variant).

Adult↗

Subacute cutaneous lupus erythematosus associated with cinnarizine and thiethylperazine therapy.

We report the case of a woman who developed subacute cutaneous lupus erythematosus (SCLE) after exposure to the sun while taking cinnarizine and thiethylperazine. The patient recalled that 10 years previously, a similar eruption had appeared after sunbathing, while she was taking only cinnarizine. SCLE development in this patient was probably drug related and there is some evidence that cinnarizine played an important pathogenic role.

Adult↗

[Kaposi's sarcoma associated with the acquired immunodeficiency syndrome. An analysis of 67 cases with a study of the prognostic factors].

The features and prognostic factors of 67 cases of Kaposi's sarcoma (KS) associated to the acquired immunodeficiency syndrome (AIDS) diagnosed at the Hospital Clinic, Barcelona, are analyzed. All the patients were male; mean age was 39.7 years, ranging from 22 and 62 years. 64 were homosexuals (95.5%), two were homosexual-drug addicts (3%) and one was drug addict (1.5%). Prevalence of cytomegalovirus and herpes virus infections were 91.1% and 89.5% respectively. In 42 cases (62.7%) KS was the initial AIDS presentation. The most common localization was the skin (89.5%), followed by the digestive tract (52.2%) and the lymph nodes (22.4%). Staging distribution was: 20 patients (29.8%) were in stage I, 11 patients (16.4%) in stage II, 7 patients (10.4%) in stage III, and 29 patients (43.2%) in stage IV. Constitutional symptoms associated to KS were found in 37 patients (55%). Overall 39 patients have already died, and the actuarial survival possibility of these 67 cases was 55% after 12 months. Univariant statistical analysis showed the presence of six variables with prognostic significance (p less than 0.05): staging, symptomatology, total white blood count, total lymphocyte count, T helper lymphocyte count and hemoglobin. Multivariant statistical analysis only chose the staging and symptomatology variables as independent (p less than 0.01 and p less than 0.001, respectively).

Acquired Immunodeficiency Syndrome↗

AIDS and Salmonella-infected abdominal aortic aneurysm.

The case of a bisexual male patient with acquired immunodeficiency syndrome (AIDS) and an infected abdominal aortic aneurysm requiring surgery is presented. Attention is drawn to the fact that an unpredictable number of operations will be needed in AIDS patients in the next future.

Acquired Immunodeficiency Syndrome↗

[Toxic skin reaction caused by hydantoins in a patient with AIDS].

A case of clinically characteristic hypersensitivity reaction to phenytoin in a AIDS patient with cerebral toxoplasmosis is reported. There was response to therapy with systemic steroids. Laboratory data and histopathologic features of the skin are reported.

Acquired Immunodeficiency Syndrome↗

[Pemphigus herpetiformis associated with neoplasm of the lung].

We report a case of a 57 year-old male who developed atypical bullous disease and in whom an underlying carcinoma of the bronchus was found. The cutaneous eruption fulfilled the clinical, histological and immunological features of pemphigus herpetiformis. Reports of pemphigus herpetiformis and internal malignancy are extremely rare.

Carcinoma, Small Cell↗