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Ramon M Pujol

Publications and source records attributed to Ramon M Pujol.

At least 19 recordsLinked to original sources
PubMed2006

Aberrant nuclear BCL10 expression and lack of t(11;18)(q21;q21) in primary cutaneous marginal zone B-cell lymphoma.

Inhibition of apoptosis seems to play an important role in the pathogenesis of marginal zone lymphoma. Apoptosis regulator B-cell lymphoma 10 (BCL10) may show aberrant nuclear localization in some aggressive extracutaneous MALT lymphomas, often in association with a MALT1 gene t(11;18)(q21;q21) translocation. The possible occurrence of this association in primary cutaneous marginal zone lymphoma (PCMZL) remains insufficiently explored. The aim of this study was to evaluate BCL10 protein expression pattern and its possible relationship to the presence of t(11;18)(q21;q21) and other MALT1 gene abnormalities in PCMZL and to assess their clinical significance. The study included 42 consecutive PCMZL patients diagnosed on the basis of the World Health Organization/European Organization for the Research and Treatment of Cancer classification criteria. BCL10 expression was immunohistochemically evaluated in all cases, whereas t(11;18)(q21;q21) reverse transcriptase polymerase chain reaction amplification was performed on 21 samples. In addition, the presence of other MALT1 gene translocations was explored in 26 samples by interphase fluorescence in situ hybridization using a MALT1 locus-specific probe. We observed the presence of aberrant nuclear BCL10 expression in a significant number of PCMZL cases (36%, 15/42). This aberrant expression was significantly related to the development of extracutaneous disease. In contrast, neither the t(11;18)(q21;q21) translocation nor other MALT1 gene translocations could be demonstrated. t(11;18)(q21;q21), strongly linked to extracutaneous MALT lymphomas, does not seem to play a role in PCMZL. The participation of other MALT1 gene translocations in PCMZL pathogenesis seems also unlikely.

Adaptor Proteins, Signal Transducing↗
PubMed2006

Annually recurring erythema annulare centrifugum: a distinct entity?

Four patients presenting a peculiar clinical variant of erythema annulare centrifugum are reported. The lesions were clinically and histopathologically indistinguishable from classic superficial erythema annulare centrifugum but constant annual and seasonal recurrences for many years or decades were observed. No clear precipitating factor could be identified. No associated symptoms were present and the eruption regressed spontaneously after a variable period of days to months. Annually recurring erythema annulare centrifugum seems to represent a rare distinct clinical entity that has received little attention in literature. Clinicopathologic features of this peculiar clinical disorder and the differential diagnosis with other recurrent seasonal eruptions are reviewed.

Aged↗
PubMed2006

Granulomatous dermatitis with enlarged histiocytes: a characteristic pattern of granulocyte colony-stimulating factor. Report of two cases and review of the literature.

Two patients developed a pruritic maculopapular rash following treatment with granulocyte colony-stimulating factor (G-CSF). Histopathological examination disclosed a superficial dermal inflammatory infiltrate composed of interstitially arranged large histiocytic CD68+ cells and perivascularly disposed lymphocytes. Large histiocytes were interspersed among the collagen bundles with associated slight deposits of mucin. Elastophagocytosis was occasionally observed, and a slight reduction of dermal elastic fibers was also noted. There was no evidence of frank granuloma formation, fibrosis or significant changes of collagen bundles. This histopathological variant of granulomatous dermatitis with "enlarged histiocytes" seems to be characteristic of an unusual cutaneous reaction secondary to CSF treatment.

Acute Disease↗
PubMed2005

Clinical approach to cutaneous vasculitis.

PURPOSE OF REVIEW: This review provides the readers with an update on the clinical approach of a patient seeking treatment with cutaneous vasculitis. It outlines the work-up for assessing patients with cutaneous vasculitis and discusses the essential features of the main conditions included within this category. RECENT FINDINGS: Recent works on genetic and infectious factors implicated in the pathogenesis of Henoch-Schonlein purpura are discussed. Special attention is given to the prognosis and response to treatment. Also, recent reports on cutaneous vasculitis secondary to connective tissue diseases are reviewed. SUMMARY: With this review, the reader will be able to establish the steps to be followed in the clinical approach to a patient seeking treatment with cutaneous vasculitis.

Connective Tissue Diseases↗
PubMed2005

Peripheral T-cell lymphoma with secondary epithelioid granulomatous cutaneous involvement: a clinicopathologic study of four cases.

Epithelioid granuloma formation has rarely been observed in specific cutaneous lesions from T-cell lymphomas other than those of mycosis fungoides/Sézary syndrome (MF/SS). Three patients diagnosed with nodal and/or extranodal (tonsillar) non-Hodgkin's peripheral T-cell lymphoma (PTCL) and one patient with angioimmunoblastic T-cell lymphoma (AILD), developed specific cutaneous involvement showing prominent epithelioid cell and/or granulomatous inflammation. The original diagnostic lesions had no granulomatous features. In addition to a specific lymphomatous infiltrate, prominent dermal and/or subcutaneous granulomatous infiltrates were observed. Sarcoid-like granulomas were observed in two patients (one of them presented a granuloma annulare-like pattern in early lesions), granulomatous panniculitis was noted in one patient and in one patient with AILD, masses of epithelioid cells were noted. The clinicopathological features of cutaneous involvement by PTCL showing a florid epithelioid and/or granulomatous cell reaction are reviewed. Various histopathological patterns can be observed. The diagnostic difficulties of these cases are stressed.

Aged↗
PubMed2005

Comparative genomic hybridization analysis of cutaneous large B-cell lymphomas.

The aim of the present study was to identify genetic aberrations in a series of patients with cutaneous large B-cell lymphoma (LBCL) using comparative genomic hybridization (CGH). Eighteen consecutive patients with primary (13 patients) (PCLBCL) and secondary (five patients) (SCLBCL) cutaneous large B-cell lymphoma were included in the study. Nine cases corresponded to PCLBCL leg type and four cases primary cutaneous follicle centre-cell lymphoma (PCFCL). Chromosomal imbalances (CIs) were detected in 14 of 18 samples (77.8%). All of nine cases with PCLBCL leg type and two of four cases with PCFCL showed CIs (100% and 50%, respectively). Regarding SCLBCL, in three of five cases (60%), CIs were detected. The most frequently detected gains involved 2q, 5q, 3 and 7q and amplifications affected 18, 12 and 13. Frequent losses were found in 17p. In PCLBCL leg type, the most frequent gains involved 2q and 7q, amplifications were localized in chromosomes 12, 13 and 18 and losses affected chromosomes 17p and 19. In PCFCL, gains located in 3q, 4 and 7q were found. Our study seems to confirm clear-cut differences between primary cutaneous LBCL and nodal diffuse LBCL, and it suggests the presence of genotypic differences between cases of PCLBCL leg type and cases of PCFCL.

Adult↗
PubMed2005

Oral involvement in lymphomatoid papulosis. Report of two cases and review of the literature.

Oral involvement in cutaneous CD30+ T-cell lymphoid proliferations is rare and has received little attention in the dermatologic literature. The authors report 2 patients with self-healing, recurrent papulonodular eruptions with the classic clinical, histopathological and immunophenotypic features of lymphomatoid papulosis, which developed two ulcerated papules and an ulcerative nodule on the dorsum of the tongue, respectively. The lesions appeared coincident with a new cutaneous relapse of the disease. Histopathological and immunophenotypic features were similar to those of the cutaneous lesions. All lesions regressed spontaneously after several weeks. Since then, and after follow-up periods of 3 and 7 years, respectively, no evidence of extracutaneous involvement has been detected. Oral involvement in lymphomatoid papulosis is an uncommon event, probably without prognostic significance. Previously reported cases are reviewed. The differential diagnosis of atypical T-cell lymphoid infiltrates observed in the oral mucosa is discussed.

Diagnosis, Differential↗
PubMed2005

Dystrophic xanthomatosis in primary CD30-positive cutaneous T-cell lymphoma. Report of two cases and review of the literature.

The development of xanthomatous changes in lesions of primary cutaneous T-cell lymphomas (CTCL) is a rare event. It is usually observed in regressing skin tumours or plaques spontaneously or after a specific treatment. The pathogenetic mechanisms implicated in these changes are poorly understood. We are reporting 2 cases of extensive dystrophic xanthomatous changes, developing in regressing lesions of primary cutaneous CD30-positive T-cell lymphoproliferative disorders. Previously reported cases of primary CTCL developing secondary (dystrophic) xanthomata are reviewed.

Adult↗
PubMed2004

Sclerodermatomyositis associated with severe arthritis.

Overlap syndromes are a spectrum of diseases with clinical features of two or more classic connective tissue diseases. Polymyositis or dermatomyositis that is associated with features of systemic sclerosis, also called sclerodermatomyositis, is an uncommon overlap syndrome, with a rare antibody directed against the nucleolar antigen PM-Scl. This disease generally has good prognosis. We report a case of sclerodermatomyositis with an aggressive pulmonary and articular course.

Adult↗
PubMed2004

Comparative analysis of TCR-gamma gene rearrangements by Genescan and polyacrylamide gel-electrophoresis in cutaneous T-cell lymphoma.

Demonstrating T-cell clonality has become an important approach supporting a diagnosis of malignant T-cell neoplasms. A comparative study between Genescan analysis, polyacrylamide gel and agarose gel electrophoresis in visualizing T-cell receptor gamma gene rearrangement was performed on 25 biopsy specimens from 18 patients with different forms of cutaneous T-cell lymphomas. Clonality was detected in 17 biopsy specimens when PCR products were evaluated by Genescan analysis. Seventeen showed discrete bands when visualized in polyacrylamide gel and 14 cases were clonal when visualized with agarose gel. In five cases, a clonal population was seen in the gels, but not with Genescan. On sequencing the PCR products we demonstrated non-clonality of these five samples. Our results confirm that PCR-Genescan is a useful, reliable and specific screening method for detecting dominant clones in patients with T-cell lymphoma.

Electrophoresis, Polyacrylamide Gel↗
PubMed2004

Lymphomatoid papulosis associated with mycosis fungoides: clinicopathological and molecular studies of 12 cases.

The association of mycosis fungoides and a primary cutaneous CD30+ lymphoproliferative disorder has been reported and probably represents different clinical aspects of a unique T-cell monoclonal expansion. In this study, 12 patients (6 men and 6 women) presented with lymphomatoid papulosis and mycosis fungoides. A TCRgamma gene rearrangement study was performed by an automated high-resolution PCR fragment analysis method on skin biopsy specimens taken from the different clinical lesions in each patient. An indolent clinical course was observed in the majority of patients. T-cell clonality was identified in 7 of 12 lymphomatoid papulosis lesions (58%) and in 6 skin biopsies of plaque stage mycosis fungoides (50%). In each individual case, where T-cell clonality was detected, both mycosis fungoides and lymphomatoid papulosis specimens exhibited an identical peak pattern by automated high-resolution PCR fragment analysis, confirming a common clonal origin. Only one case showed a clonal TCRgamma rearrangement from the lymphomatoid papulosis lesion, which could not be demonstrated in the mycosis fungoides specimen. The demonstration of an identical clone seems to confirm that both disorders are different clinical manifestations of a unique T-cell monoclonal proliferation. Our results also seem to confirm that the association of mycosis fungoides with a primary cutaneous CD30+ lymphoproliferative disorder usually carries a favourable prognosis.

Adult↗
PubMed2004

Necrolytic migratory erythema: clinicopathologic study of 13 cases.

BACKGROUND: The clinical mucocutaneous manifestations of glucagonoma syndrome are recognized easily when they occur in the classic pattern of acral or periorificial lesions evolving in recurrent crops, with an annular and migratory distribution, in a patient with diabetes mellitus who has had recent weight loss and anemia. Not infrequently, noncharacteristic clinical and histopathologic features are observed and, in these cases, the diagnosis of pancreatic neoplasm may be delayed. AIM: To review the clinical and histopathologic features of cutaneous manifestations of glucagonoma syndrome. METHODS: The clinicopathologic features of 13 patients (eight women) with widespread or localized cutaneous eruption as a manifestation of islet cell pancreatic carcinoma with marked glucagon secretion (glucagonoma) were reviewed. RESULTS: The definitive diagnosis of the cutaneous eruption was established at the time of diagnosis of the pancreatic neoplasm (three patients) or afterwards (10 patients). In nine patients, the mucocutaneous manifestations preceded the diagnosis of the pancreatic neoplasm by 1 month to 3 years (mean, 12 months). In only eight biopsy specimens were the histopathologic features considered to be suggestive or characteristic of necrolytic migratory erythema. Diffuse parakeratosis, that occasionally arose abruptly from normal epidermis, was observed in 12 biopsy specimens. By the time necrolytic migratory erythema was diagnosed, the pancreatic carcinoma had metastasized to the liver, regional lymph nodes, or bone in 12 patients. CONCLUSION: Increased awareness of the polymorphic mucocutaneous and nonspecific histopathologic features of glucagonoma syndrome is needed to avoid unnecessary delay in the diagnosis of this syndrome.

Adult↗