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Biomedical subjects

L Puig

Publications and source records attributed to L Puig.

At least 55 records · Page 3Linked to original sources
PubMed1998

Brooke-Spiegler syndrome variant: segregation of tumor types with mixed differentiation in two generations.

Brooke-Spiegler syndrome (BSS) is an autosomal dominantly inherited disease characterized by the development of multiple trichoepitheliomas and cylindromas. Other lesions have been reported to occur in patients with BSS, including parotid basal cell adenomas, milia, organoid nevi, basal cell carcinomas, and spiradenomas. Spiradenomas and cylindromas have so many features in common that they have been regarded as polar extremes belonging to a spectrum of cutaneous adnexal neoplasms. We report on a 61-year-old woman with multiple spiradenomas on the scalp and periauricular areas and her 28-year-old daughter, with multiple facial trichoepitheliomas. Occasional features of pilar and ductal differentiation were found in tissue specimens of tumors from the mother and daughter, respectively. Co-existence of multiple spiradenomas and trichoepitheliomas and segregation of the predominant type of tumor in different members of a family with BSS have been reported occasionally, but never, to our knowledge, in combination. The presence of mixed differentiation in tumor specimens from both patients provides additional evidence in support of the folliculosebaceous apocrine unit (FSAU) hypothesis. Mutations in genes regulating proliferation and differentiation of putative stem cells of the FSAU would give rise to different combinations of adnexal skin tumors as well as to other neoplasms.

Adult↗
PubMed1998

Benign myoepithelioma of the skin.

A case of cutaneous myoepithelioma is reported. The tumor was composed of spindle-shaped, epithelioid, and plasmacytoid (hyaline) cells. It exhibited a widespread immunoreactivity for low molecular weight keratins and protein S-100, being irregularly positive for smooth muscle actin. Ultrastructural studies of tumor cells showed a variable content of intermediate filaments, with focal densities resembling smooth muscle dense bodies. A well-developed basal lamina, pinocytotic vesicles, and some desmosomes were also observed. In spite of being accepted as an individual entity, myoepitheliomas probably belong to a family of lesions that include mixed tumors. Therefore, this case can be considered as a salivary-gland-type tumor, probably originating from myoepithelial cells of sweat glands. The existence of this unique neoplasm provides further support to the debated role of myoepithelial cells in the development of mixed tumors.

Actins↗
PubMed1998

[Report on the activity of the blood bank accreditation program (1987-1995)].

PURPOSE: To show the incidence of the deficiencies detected in the Blood Banks for the accreditation by the Transfusion Accreditation Committee (CAT), previously named PABAS. MATERIALS AND METHODS: Analysis of the reports of the accreditation of 85 Blood Banks made by the PABAS during the period 1987-1995. RESULTS: Eighty-five (20.8%) of the 407 Community Blood Centers, Hospital-Based Blood Banks and Transfusional Services of Spain had been surveyed. There were 244 deficiencies, of which 31 (12.7%) were of the equipment, 114 (46.7%) of the procedures used, and 99 (40.6%) of the documentation. The activities with more incidence of faults were: Control of the temperatures of the storage of units 53 (21.7%), label of the components 38 (15.5%), quality system of the institution surveyed 32 (13.1%), transfusional procedures 30 (12.3%), and on the procedure of the selection of donors 29 (11.9%). By contrary, the areas of work with fewer incidences of faults were those related with the collection of the blood and components 10 (4.1%) and the laboratory 14 (5.7%). CONCLUSIONS: Low percentage of the Community Blood Centers, Hospital-Based Blood Banks and Transfusional Services, which ask to be accredited by the Transfusion Accreditation Committee. The 83.7% of the errors detected are of the procedures and documentation, which could be easily corrected by the training and continuous improving of the quality, and without need of new inversions in equipment.

Accreditation↗
PubMed1997

Carpal tunnel syndrome presenting as sclerodactylia, nail dystrophy and acro-osteolysis in a 60-year-old woman.

Skin changes associated with carpal tunnel syndrome have rarely been reported. A 60-year-old patient was referred to our Department of Dermatology for evaluation of an aseptic gangrene of the distal phalanx of the third finger of her left hand, progressive nail dystrophy, sclerodactylia and spontaneous ulcerations. These features were seen on the digits with sensory supply from the median nerve. The patient had hand paresthesias for at least 10 years. No abnormal results were found in biochemical, hematological or immunological determinations. An echo-Doppler study of the upper limbs was normal. An electromyographic study was consistent with a bilateral carpal tunnel syndrome (CTS). Ulcerative and mutilating CTS can occasionally produce sclerodactylia with ulcerative lesions on distal fingers, nail dystrophy and acro-osteolysis as a consequence of mechanical compression of the autonomic fibers of the median nerve. The clinical picture includes Raynaud's phenomenon in some patients. Correct diagnosis is important, since ulcerative and mutilating CTS is a marker of a severe neural lesion, which is amenable to treatment by surgical decompression.

Carpal Tunnel Syndrome↗
PubMed1997

Primary neuroendocrine carcinoma of the skin expresses tyrosinase mRNA: detection by a specific nested PCR technique.

BACKGROUND: Tyrosinase transcripts have been identified in a wide range of normal organs and neoplasms such as cutaneous nevi, breast carcinomas, liposarcomas and schwannomas. The cells responsible for these positive results have not been accurately identified yet, but they might correspond to either fully differentiated melanocytes, melanocytic precursors or cells bearing potential for melanocytic differentiation. OBJECTIVE: To assess the expression of tyrosinase mRNA in surgical resection specimens and peripheral blood samples from 2 patients with primary neuroendocrine carcinomas of the skin (PNCS). METHODS: The expression of tyrosinase mRNA transcripts was tested by means of a reverse-transcriptase and nested PCR technique, using specific primers, in tissue samples from surgical specimens and heparinized blood samples from 2 patients with PNCS. RESULTS: The results of the test were positive in the 2 specimens of PCNS but negative in blood samples. CONCLUSION: Our findings provide further support for the hypothesis that PNCS originates from a pluripotent stem cell capable of neuroendocrine cell differentiation, keratin production, and, as reported herein, tyrosinase mRNA expression. It remains to be seen whether this technique can also be applied to patients with PNCS, as an adjunct to staging and treatment planning.

Aged↗
PubMed1997

Masson's intravascular papillary endothelial hyperplasia mimicking Stewart-Treves syndrome: report of a case.

Masson's intravascular papillary endothelial hyperplasia, also called Masson's pseudoangiosarcoma, represents a benign vascular proliferation, presently considered as a peculiar histopathologic reaction pattern of the endothelium to diverse stimuli. Differentiation from angiosarcoma represents the main diagnostic concern. We report a case of Masson's intravascular papillary endothelial hyperplasia presenting in a 55-year-old woman with ipsilateral lymphedema secondary to surgery and radiation therapy for breast carcinoma. The diagnosis was intravascular papillary endothelial hyperplasia. This is the first description of Masson's pseudoangiosarcoma in this clinical context, to our knowledge. We believe it represents a peculiar morphologic pattern of endothelium proliferation secondary to venous stasis and thrombosis due to lymphedema.

Arm↗
PubMed1996

CD44 distribution in sweat gland tumors suggests it has different functional roles in the various cell types.

CD44 is a polymorphic group of membrane glycoproteins with multiple functions that include cell adhesion. Since on normal sweat glands CD44 is expressed only in eccrine coil secretory cells, it has been considered as a possible marker of this type of differentiation. We have immunohistochemically investigated the distribution of CD44 in paraffin-embedded samples of 41 benign and malignant sweat gland tumors by using a monoclonal antibody directed against the standard isoform of CD44. CD44 was strongly expressed in epithelial cells at the peripheral row of syringomas and in cuticular areas of eccrine poromas. Apocrine tumors such as apocrine hidrocystoma, syringocystadenoma papilliferum, or hidradenoma papilliferum showed intense CD44 positivity in the portion of cells in contact with the neighboring stroma and focally on the luminal side of cells with apocrine secretion. Cylindromas and spiradenomas presented focal CD44 positivity, virtually limited to clear cells. Malignant neoplasms exhibited irregular CD44 staining, which was more intense in the less differentiated zones and tumors. Our results indicate that CD44 is not a useful marker for a specific form of sweat gland differentiation. Nevertheless, its characteristic patterns of distribution might reflect the variety of functional roles assumed by the different CD44 isoforms in each epithelial cell.

Adenoma, Sweat Gland↗
PubMed1996

Reiter's syndrome-like pattern in AIDS-associated psoriasiform dermatitis.

BACKGROUND: The prevalence of psoriasiform dermatitis in HIV-infected patients is similar to that in the general population, but its clinical severity and the immunosuppression in these patients pose special problems of therapeutic management. Furthermore, a distinctive clinical pattern has been reported in some cases. In order to assess these features in our clinical setting, we have done a retrospective study on the clinical records of all human immunodeficiency virus (HIV)-positive patients with psoriasiform dermatitis requiring systemic treatment. METHODS: The clinical records were reviewed of seven HIV-positive patients who were referred between 1988 and 1994 to a University Hospital Dermatology Department from an HIV-clinic because of psoriasiform dermatitis, resistant to topical treatment. RESULTS: The clinical appearance was rather uniform, with the following common features: facial seborrhea, flexural and acral involvement, with pustulosis of the palms and soles, and frequent arthritis. Lesions appeared in nonterminal stages of acquired immunodeficiency syndrome (AIDS). Three patients developed cutaneous lesions after the diagnosis of HIV infection was made and showed the most severe clinical involvement and arthritis. Etretinate, followed by RePUVA, proved to be the most effective systemic therapy prescribed, with only rare adverse effects. Methotrexate was shown to be effective, but it's use was accompanied by hematologic toxicity. Cyclosporine A treatment was moderately effective and was not associated with progression of AIDS. CONCLUSIONS: A characteristic Reiter-like clinical picture was observed in AIDS-related psoriasiform dermatitis. Etretinate and RePUVA were effective and safe in controlling the lesions. Physiopathologic mechanisms involved in the development of AIDS-related psoriasis might provide an explanation for the outstanding similarity of the clinical pattern in those patients.

Acquired Immunodeficiency Syndrome↗
PubMed1996

CD44 and melanocytic tumors: a possible role for standard CD44 in the epidermotropic spread of melanoma.

CD44 is a polymorphic family of cell membrane glycoproteins that mediate cell-matrix and cell-cell interactions involved in the mechanisms of tumor invasion and metastasis, and are subject to differential regulation during normal and malignant cell growth. We have investigated immunohistochemically the expression of CD44S and the variant isoforms CD44v3 and CD44v6 in paraffin-embedded tissue from 5 Spitz nevi, 3 compound melanocytic nevi, 2 blue nevi, 6 primary melanomas, 15 cutaneous metastases (three epidermotropic, nine dermal and three ulcerated) and 10 lymph node metastases of melanoma. Melanocytes were extensively positive for CD44S in primary melanomas and benign melanocytic proliferations. Among 15 cases of cutaneous metastases of melanoma, the three epidermotropic metastases, as well as one of the three ulcerated ones were positive for CD44S. CD44S expression was diminished or totally absent in six of the nine dermal metastases, in two of the ulcerated metastases and in seven of the ten lymph node metastases. CD44v3 and CD44v6 melanocytic expression was absent in all the lesions studied. According to our results, selective retention of CD44S expression by melanocytes in epidermotropic metastases of melanoma seems to indicate that preservation of CD44S may contribute to the intraepidermal spread of melanoma.

Antibodies, Monoclonal↗
PubMed1996

Mycosis fungoides associated with unusual epidermal hyperplasia.

A 58-year-old white woman presented with widespread pruritic brownish plaques and hyperpigmented flexural lesions with a velvety appearance. On histopathological examination, the macules were diagnostic of mycosis fungoides, plaque stage, and the flexural lesions showed epidermal hyperplasia with a seborrhoeic keratosis-like appearance. There was intense mucin deposition and marked reduction of elastic fibres in the papillary dermis, as well as a moderately dense dermal lymphoid infiltrate composed of CD4+ T cells with occasional atypia and focal epidermotropism. The clinical and pathological manifestations of cutaneous T-cell lymphomas, including mycosis fungoides (MF), may show considerable variation. Apart from the three classic stages, a number of unusual clinical presentations and a broad spectrum of histopathological findings have been reported in the literature. In this report, a case of MF, plaque stage, with flexural lesions clinically reminiscent of (pseudo)acanthosis nigricans is presented. On histopathological examination, there was epidermal hyperplasia with a seborrhoeic keratosis-like appearance, with intense deposition of mucin and marked reduction of elastic fibres in the papillary dermis, together with a lymphoid infiltrate composed mainly of CD4+ cells with occasional atypia and epidermotropism. Cytokines produced by the lymphocytes in the infiltrate might account for the epithelial and dermal changes that characterize this peculiar variant of MF.

Female↗
PubMed1996

[Post-transfusion purpura. Description of 2 cases diagnosed at the same hospital in a 6-month period].

Two new cases of postransfusional purpura diagnosed at the same hospital within the space of six months are described. This finding supports the idea that despite being an infrequent disorder, a substantial rise in PTP cases inside and outside our country, has been recorded. This increase has coincided with a greater interest in platelet immunology and, particularly, in complications associated with blood transfusion. Both cases constitute an example of the clinical epidemiological profile which characterizes the patients suffering from this disorder. The patients are two women aged 74 and 60 years who after 8 and 9 days, respectively, of being transfused with red cells developed a severe thrombocytopenia accompanied by generalized haemorrhagic diathesis. The serological studies performed revealed the presence, in both patients, of an HPA-1a platelet specific antibody. The platelet genotyping enabled us to confirm this specificity after detecting an HPA-1 (a-b+) platelet genotype. The treatment with immunoglobulins at high doses proved to be effective in both cases. The adsorption-elution experiments of the antibody versus HPA-1 (a + b) platelets were positive in the patient with the highest antibody titre (1024). This finding support the most recent hypothesis concerning the pathogenic mechanism of PTP. According to this theory, the antibody, which is detected in the acute phase of the PTP, would not yet have acquired the restricted specificity corresponding to it. This could enable it to react with a structure shared by the HPA-1a positive and HPA-1a negative platelets.

Aged↗
PubMed1995

Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange.

The aim of this study was to investigate pretreatment prognostic factors that could be useful in predicting the response to plasma exchange in thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). Thirty-two patients with TTP/HUS, treated with plasma exchange at our institution from 1980 to 1994, were studied. The main clinical and laboratory data at the beginning of plasma exchanges were analyzed by the Cox stepwise logistic regression, applied to either treatment failure or death. Seventeen (53%) patients attained a complete remission and 22 (69%) survived (five in advanced renal failure and long-term hemodialysis). Longer delay in initiating plasma exchanges, presence of stupor or coma, and higher creatinine levels at the beginning of plasma exchanges were independent predictors of treatment failure. Stupor or coma at the beginning of plasma exchanges was the only predictor of mortality from unremitted TTP/HUS. Hemoglobin levels, platelet count, and LDH activity, traditionally envisaged as markers of disease activity, neither correlated with previous duration of TTP/HUS nor had any prognostic value. Early diagnosis of TTP/HUS and prompt initiation of intensive plasma exchange emerged from this study as the most effective interventions for improving the prognosis of TTP/HUS patients.

Adolescent↗
PubMed1995

Epidermal expression of 65 and 72 kd heat shock proteins in psoriasis and AIDS-associated psoriasiform dermatitis.

BACKGROUND: Psoriasiform dermatitis is common in patients with AIDS. The expression of heat shock proteins by keratinocytes has been postulated to be a significant factor in the physiopathology of psoriasis and might be subject to modulation in HIV-infected patients. OBJECTIVE: We sought to evaluate the epidermal expression of 65 and 72 kd heat shock proteins (HSPs) in lesions of AIDS-associated psoriasiform dermatitis (AIDS-PD) and compare it with that in psoriasis vulgaris and seborrheic dermatitis in patients not infected with HIV. METHODS: Sections from paraffin-embedded blocks of biopsy specimens of AIDS-PD (eight cases), psoriasis vulgaris (eight cases), seborrheic dermatitis (four cases), and normal skin (four cases) in non-HIV-infected patients were immunohistochemically stained by the avidin-biotin-peroxidase method and two monoclonal antibodies directed against the major 65 kd HSP antigen (HSP65) and against 70/72 kd HSP. The intensity, distribution, and cellular pattern of the epidermal stain were graded and assessed blindly. RESULTS: The epidermal expression of HSP65 in biopsy specimens from AIDS-PD lesions was irregular, with less intensity and less tendency to perinuclear arrangement than in psoriasis or seborrheic dermatitis not associated with AIDS. The expression of HSP72 was also less intense and more uniform in AIDS-PD. CONCLUSION: The altered interplay of T cells and keratinocytes in a situation of immune derangement such as AIDS might account for the differences observed in the expression of HSP65 and HSP72 by keratinocytes in psoriasis and AIDS-PD.

Acquired Immunodeficiency Syndrome↗