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Biomedical subjects

J Peyrí

Publications and source records attributed to J Peyrí.

At least 19 recordsLinked to original sources

Calciphylaxis associated with alcoholic cirrhosis.

Calciphylaxis is an uncommon disease characterized by calcification of dermal vessels that determines skin necrosis. Calciphylaxis has been almost exclusively reported in association with renal failure and altered phosphor-calcium metabolism. Only a few cases have been described in hyperparathyroidism, malignancies, and, recently, cirrhosis. We report a patient that developed calciphylaxis related to end-stage alcoholic cirrhosis, without any alteration in the phosphocalcic and parathyroid hormone metabolisms. Possible contributing factors were repeated albumin infusions and low levels of protein C and S.

Biopsy↗

Recommendations for the use of etanercept in psoriasis: a European dermatology expert group consensus.

BACKGROUND: Psoriasis is a chronic, inflammatory skin disorder that has a significant impact on quality of life and, particularly in moderate to severe cases, adversely affects the patient's overall health and well-being. Biological treatments, such as etanercept, are being widely adopted across Europe for treatment of moderate to severe psoriasis due to favourable safety and efficacy profiles. The increase in usage, combined with a growing body of clinical evidence, has identified a need to clarify the best use of etanercept within its current treatment label. OBJECTIVE: To prepare a series of recommendations agreed by an expert group of dermatologists, relating to the most effective use of etanercept for psoriasis in Europe, within the product license. METHODS: An expert panel of dermatologists from across Europe completed a Delphi survey to address the current use of etanercept in psoriasis in Europe. In June 2005 the results were presented to the expert panel at their nominal group meeting, and a consensus was agreed. RESULTS: It was recommended that, where possible, patients are initiated on the 50 mg twice-weekly (BIW) dose. Etanercept should be given until remission is achieved (maximum 24 weeks) and retreatment should be initiated according to the physician's judgement. Before commencing treatment, contraindications, such as infection or previous malignancy (within 5 years), should be ruled out. CONCLUSIONS: The consensus presented herein provides valuable clarification of use of etanercept according to the label, which may have wider implications relating to the use of all biological therapies in psoriasis.

Adolescent↗

Coumarin necrosis induced by renal insufficiency.

Cutaneous necrosis is an infrequent complication of coumarin therapy. Skin necrosis has usually been reported in patients with congenital protein C deficiency or, less commonly, protein S deficiency. However, this complication may also occur with acquired and transient protein C and/or S deficiency. In coumarin therapy there is a relatively hypercoagulable state at the start of treatment, and most lesions appear between the third and sixth days. We describe a 75-year-old man receiving coumarin therapy (acenocumarol) for 7 years who was given a nonsteroidal anti-inflammatory agent (diclofenac) for a pain in his knee. Two days later, his renal function deteriorated and skin necrosis became evident. Biopsy showed histological changes consistent with coumarin-induced necrosis. Protein C and S levels were normal. We concluded that in our patient acute renal insufficiency aggravated by diclofenac treatment probably associated with an inadvertent withdrawal could have been the precipitating factor for transient protein C deficiency.

Acenocoumarol↗

Primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases.

BACKGROUND: Primary cutaneous marginal zone B-cell lymphoma (MZCL) has recently been described. Differentiation from follicular centre cell lymphomas and lymphocytomas is often difficult due to insufficient experience and a lack of large series of patients. OBJECTIVES: To characterize primary cutaneous MZCL better, we report clinical, histopathological, immunophenotypic and molecular genetics features in a series of 22 patients. METHODS: All patients were treated and followed up at the same institution. Diagnosis of MZCL was based on the World Health Organization classification criteria. All samples were routinely tested with a wide panel of monoclonal antibodies. DNA was extracted from every sample following standard methods. IgH rearrangement and t(14;18)(q32;q21) studies were performed in all samples. RESULTS: Twenty-two patients (20 men, two women; mean age 50 years, range 24-77) were included. The mean follow-up was 43 months. Seventy per cent of patients presented with characteristic skin lesions on the trunk or extremities, consisting of deep red to violaceous infiltrated plaques, nodules or tumours frequently surrounded by diffuse or annular erythema. Four patients presented with lesions on the head and neck area. Two patients had disseminated skin lesions. The main histopathological features were non-epidermotropic, dense lymphocytic infiltrates mainly distributed in a nodular pattern. Adnexal involvement was usually present, with eventual formation of lymphoepithelial complexes. Cytologically, the infiltrate was polymorphous with marginal zone B cells and B-monocytoid cells. Blastoid CD30+ cells were often observed. Colonized reactive germinal centres and lymphoplasmocytoid differentiation were frequently present. Neoplastic cells were CD20+, CD79a+, CD5- and CD10-. Monotypic expression of light chains was observed in 18 cases (13 kappa; five lambda). Clonal IgH rearrangements were detected in 14 cases. The bcl-2 mutation t(14;18)(q32;q21) was demonstrated in two cases. Most patients were treated with local radiotherapy. Complete response rate with this approach was 100%. Six patients (27%) had skin recurrences from 6 months to 8 years after first treatment. Five patients (23%) had extracutaneous involvement. Two of them had a large cell transformation and one died of lymphoma. Three of four patients with head and neck presentation developed extracutaneous disease. CONCLUSIONS: MZCL appears to be a well recognizable entity, clinically, histologically and immunophenotypically. Although prognosis is generally good, the disease has potential for skin as well as extracutaneous recurrences. Large cell transformation and head and neck presentation may be associated with a worse prognosis.

Adult↗

Foreign bodies in granulomatous cutaneous lesions of patients with systemic sarcoidosis.

OBJECTIVE: To assess the presence of foreign material in the granulomatous cutaneous lesions of patients with systemic sarcoidosis. DESIGN AND SETTING: Observational study reevaluating histological specimens at a university referral hospital. PATIENTS: Sixty-five patients diagnosed as having sarcoidosis who developed granulomatous cutaneous involvement. MAIN OUTCOME MEASURES: To detect the presence of polarizable foreign particles in cutaneous biopsy specimens and to evaluate the association with clinical features of the patients. RESULTS: Granulomatous cutaneous involvement was demonstrated in 65 (15.3%) of 425 patients with systemic sarcoidosis. In 14 (22%) of the 65 patients, the cutaneous biopsy specimen showed foreign particles in polarized light. The skin lesions corresponded to 3 different clinical patterns: an admixture of papules and infiltration of previously undetected minute scars (n = 6); scar sarcoidosis (n = 4); and subcutaneous nodules (n = 4). The lesions were located most frequently in the extremities, involving the knees in 10 patients. CONCLUSIONS: The presence of polarizable foreign body material in granulomatous cutaneous lesions is not infrequent in patients with systemic sarcoidosis. Inoculation of foreign matter from a previous inapparent minor trauma may induce granuloma formation in individuals with sarcoidosis.

Adult↗

[Skin reactions to interferon alpha in a series of 92 patients with multiple sclerosis].

BACKGROUND: A significant proportion of multiple sclerosis patients develop cutaneous reactions by interferon beta 1b (IFN-beta). The am of this study was to describe clinically and histologically the cutaneous reactions by IFN-beta. PATIENTS AND METHODS: A series of 92 patients with multiple sclerosis in treatment with IFN-beta was followed. Cutaneous biopsy was performed in cases with persistent lesions. RESULTS: 9 out of 92 patients presented eritematous plaques, 4 cutaneous ulcers and 1 sclerodermiform plaques. Vascular thrombosis was demonstrated in 2 cases. CONCLUSIONS: In patients with multiple sclerosis treated with IFN-beta the persistent skin lesions are more frequent in female patients. Generally is not necessary to stop the treatment.

Adult↗

Cardiac involvement and molecular staging in a fatal case of mycosis fungoides.

Polymerase chain reaction (PCR) amplification of T-cell receptor-gamma gene rearrangement was used for molecular staging in a case of primary cutaneous T-cell lymphoma (CTCL) with fatal evolution. Although initial evaluation was negative for systemic involvement, the patient died due to heart failure. Autopsy findings revealed lymphomatous myocardial infiltration, but other tissues and organs examined, including lymph nodes, liver, spleen, lung and bone marrow, appeared to be free of disease. Molecular analysis from frozen samples obtained during the initial evaluation, as well as paraffin-embedded material obtained during autopsy, revealed the presence of clonal rearranged bands in all tissues examined except the bone marrow. Subsequent hybridization of PCR products with a tumour-specific oligoprobe confirmed the PCR results, suggesting widespread dissemination of the lymphomatous process. The use of molecular analysis can add significant information about the extent of disease in patients with CTCL and may be helpful in the establishment of therapeutic options.

Fatal Outcome↗

Diffuse plane xanthoma: clinicopathologic study of 8 cases.

BACKGROUND: Diffuse plane xanthoma (DPX) usually has been described in association with a reticuloendothelial disease. However, the incidence of an underlying disease and its relation to clinical and histopathologic features have not been previously described. OBJECTIVE: We describe the clinicopathologic features of a series of patients with DPX. METHODS: Eight patients with DPX were diagnosed and studied between 1987 and 1996. RESULTS: Three of the 8 patients had a reticuloendothelial disease (benign monoclonal gammopathy in 2 and chronic myelomonocytic leukemia in 1). These 3 patients had larger cutaneous lesions and involvement of trunk and extremities. Histologic examination showed only foamy macrophages in 2 of the 8 patients. The remainder exhibited macrophages, foamy histiocytes, lymphocytes, and Touton cells. CONCLUSION: The incidence of underlying disease associated with DPX seems to be lower than expected. However, patients with DPX should be observed for the potential occurrence of an associated condition.

Aged↗

Multiple follicular hamartomas with sweat gland and sebaceous differentiation, vermiculate atrophoderma, milia, hypotrichosis, and late development of multiple basal cell carcinomas.

We describe a 46-year-old woman with multiple symmetrical papulocystic lesions on the face, neck, chest, back, and upper arms since childhood. Vermiculate atrophoderma, entropion, and progressive loss of eyelashes was also noted. Since the age of 30, 12 basal cell carcinomas have developed on the face. Histopathologic examination revealed multiple anastomosing nests and strands that arose from the lower part of a hair follicle in early lesions. In more advanced lesions, multiple keratinizing microcysts within a moderately sclerotic stroma were noted in the upper and mid dermis. In some biopsy specimens, focal areas showing sweat gland (ductal) and sebaceous differentiation were also observed. Basal cell carcinomas developed from the basaloid component of the hamartomatous proliferation.

Atrophy↗

Prostatic adenocarcinoma with cutaneous metastases overlying oestrogen-induced gynaecomastia.

Carcinoma of the prostate gland is the second most frequent malignancy in males, accounting for 17% of cancer in men; between a third and one-half of these patients will have distant metastases at onset, but rarely cutaneous. We now report a case of prostatic adenocarcinoma with such metastases involving the right nipple and periareolar skin, overlying an area of hormone-induced gynaecomastia.

Adenocarcinoma↗

Cutaneous involvement in sarcoidosis. Relationship to systemic disease.

Sarcoidosis is an antigen-mediated disease defined by granuloma formation in different organs. It involves mainly the mediastinal and peripheral lymph nodes, lungs, eyes, skin, liver, and spleen. Cutaneous lesions of sarcoidosis may be specific, showing histologically noncaseating granulomas, or nonspecific, most typically erythema nodosum. Frequently, both types of skin lesions are the means of presentation of the disease and may contribute to the diagnosis. A workup for systemic sarcoidosis should be undertaken in every patient with sarcoid cutaneous granulomas. Some types of cutaneous lesions have prognostic significance. Lupus pernio and plaques are associated with more severe systemic involvement and more chronic course, while erythema nodosum is the hallmark of acute and benign disease.

Acute Disease↗