Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 19 de 19
Filter
2.
Rev. med. (Säo Paulo) ; 98(3)maio-jun. 2019. ilus
Article in Portuguese | LILACS | ID: biblio-1009758

ABSTRACT

A síndrome de Morbihan é uma doença rara, de fisiopatologia pouco conhecida, tipicamente caracterizada por edema e eritema facial, de aparecimento lento, simétrico, afetando terço superior da face. Este relato de caso se trata de um paciente com caso atípico de Síndrome de Morbihan, com acometimento assimétrico de face. Foram ressaltados aspectos clínicos e histopatológicos para diagnóstico dessa rara patologia, possíveis diagnósticos diferenciais e opções de tratamento. Também foi buscado difundir formas menos típicas desta doença.


Morbihan syndrome is a rare condition and its pathogenesis is not fully known. This entityh is characterized by facial edema and facial erythema, with a slow, symmetrical appearance, affecting the upper portion of the face. This case report is about a patient with an atypical case of Morbihan syndrome, with asymmetric facial involvement. Clinical and histopathological aspects were highlighted aiming the diagnosis of this rare pathology, as well as possible differential diagnoses and treatment options. It has also been aimed to call attention to less typical forms of this disease.


Subject(s)
Humans , Male , Middle Aged , Rosacea/diagnosis , Edema/physiopathology , Erythema/physiopathology , Face/physiopathology , Dermatology
3.
Int J Dermatol ; 57(6): 675-680, 2018 Jun.
Article in English | MEDLINE | ID: mdl-29603194

ABSTRACT

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a cutaneous T-cell lymphoma mainly affecting the hair follicle, which seems to represent a place of immune privilege phenomenon. OBJECTIVES: To explore a possible role of immune privilege (IP) in FMF analyzing the major histocompatibility complex (MHC) expression. METHODS: Immunohistochemistry for HLA-G and MHC-II was performed to formalin-fixed paraffin-embedded cutaneous skin biopsies of FMF patients (n = 43), conventional mycosis fungoides (CMF; n = 13), alopecia areata (AA; n = 13), and normal scalp skin (NS; n = 12). RESULTS: HLA-G expression was lower in FMF (34%: 14/41) and CMF (18%: 2/11) groups compared to alopecia areata (92%:11/12) and normal scalp skin group (100%: 12/12). MHC-II expression in hair follicle was greater in the FMF group (18/42: 43%) compared to AA (0%) and NS (0%). HLA-G and MHC-II expression in cellular infiltrate had no difference among FMF and CMF groups and was different compared to the AA group. CONCLUSIONS: Our data support the hypothesis of disruption of immune privilege based on the lower expression of HLA-G and higher expression of MHC-II in the follicular epithelium in mycosis fungoides compared to alopecia areata and normal scalp skin. The lack of difference between FMF and CMF groups did not support the role of these molecules as a driver of folliculotropism. The expression of MHC molecules seems to be different between neoplastic and inflammatory infiltrates. The definitive significance of expression of the MHC molecules remains unclear, and more studies are necessary to fully understand the role of these molecules in cutaneous lymphomas.


Subject(s)
Lymphoma, T-Cell, Cutaneous/immunology , Lymphoma, T-Cell, Cutaneous/pathology , Mycosis Fungoides/immunology , Mycosis Fungoides/pathology , Skin Neoplasms/immunology , Skin Neoplasms/pathology , Age Factors , Aged , Biopsy, Needle , Brazil , Chi-Square Distribution , Cohort Studies , Female , HLA-G Antigens/immunology , Hair Follicle/pathology , Histocompatibility , Humans , Immunohistochemistry , Incidence , Lymphoma, T-Cell, Cutaneous/epidemiology , Male , Middle Aged , Mycosis Fungoides/epidemiology , Neoplasm Invasiveness/pathology , Neoplasm Staging , Prospective Studies , Sex Factors , Skin Neoplasms/epidemiology , Statistics, Nonparametric
4.
Int J Dermatol ; 55(5): e256-61, 2016 May.
Article in English | MEDLINE | ID: mdl-26711732

ABSTRACT

BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a rare variant of mycosis fungoides with clinical peculiarities, refractoriness to conventional treatments, and worse prognosis when compared to classic mycosis fungoides. OBJECTIVE: To evaluate the clinical and epidemiological characteristics of FMF patients in a single center in Brazil. METHODS: Data were retrospectively collected from patients with FMF who attended the Cutaneous Lymphoma Clinic, University of São Paulo Medical School, between 1987 and 2013. RESULTS: Thirty-three patients were included (median age 46 years old at diagnosis; 20 male; 27 white). The median disease duration before diagnosis was 3 years. Regarding stage at diagnosis, 61% had advanced stage disease (≥IIb). Follicular papules were reported in 66% and alopecia in 59% of the cases. The most involved regions were limbs, followed by trunk and head. Pruritus was present in 81% of the patients. The median time of patients' follow-up was 38 months. At the last follow-up visit, 67% of the patients were alive with active disease, 27% deceased, and 6% were in complete remission. Four patients had large cell transformation. At the time of diagnosis, 25% of the patients showed eosinophilia. LIMITATIONS: Retrospective study with partial unavailable data. CONCLUSIONS: The characteristics of our patients with FMF correlated with aspects previously described in the literature, which were at a more advanced stage at diagnosis and had a less favorable outcome. Pruritus is a very common complaint. Large cell transformation should be monitored as it is implicated in poor prognosis.


Subject(s)
Hair Follicle , Mycosis Fungoides/epidemiology , Mycosis Fungoides/pathology , Skin Neoplasms/epidemiology , Skin Neoplasms/pathology , Adolescent , Adult , Aged , Aged, 80 and over , Alopecia/etiology , Brazil/epidemiology , Eosinophilia/etiology , Female , Follow-Up Studies , Humans , Male , Middle Aged , Mycosis Fungoides/therapy , Neoplasm Staging , Pruritus/etiology , Retrospective Studies , Skin Neoplasms/therapy , Survival Rate , Young Adult
5.
J Am Acad Dermatol ; 72(3): 508-15, 2015 Mar.
Article in English | MEDLINE | ID: mdl-25582534

ABSTRACT

BACKGROUND: The pathogenetic mechanism of CD30(+) cutaneous lymphoproliferative disorders (CLPD) associated with pseudocarcinomatous hyperplasia (PCH) and granulocytic inflammation surrounding atypical CD30(+) lymphocytes remains unclear. OBJECTIVE: We sought to characterize clinical and pathological findings of a cohort of patients with PCH associated with CD30(+) CLPD and to analyze the cytokine profile of the atypical lymphocytes. METHODS: We retrospectively reviewed medical records and pathological material of CD30(+) CLPD with PCH. Immunohistochemistry for T-helper (Th)17 cytokine profile was performed. RESULTS: In all, 25 patients with a median age of 52 years were included. The median follow-up was 3.7 years. Histologically, an infiltrating pattern of PCH was observed in 14 cases with a neutrophilic-rich infiltrate (P = .21), and epidermal pattern in 11 cases with eosinophil-rich infiltrate (P = .03). Th17 or Th22 cytokines were detected in tumor cells in 81% cases tested. Tumor cells expressed Th17 transcription factor retinoic acid receptor (ROR)-related orphan receptor gamma-2 in 2 of 7 samples tested and 1 was positive for aryl hydrocarbon receptor. LIMITATIONS: This is a retrospective study of a small sample. CONCLUSIONS: PCH in CD30(+) CLPD is associated with Th17/Th22 cytokine expression in the atypical lymphocytes. Although these lesions commonly regress spontaneously and are associated with an indolent course, some cases develop a generalized process and tumor progression.


Subject(s)
Granulocytes/immunology , Interleukin-17/immunology , Ki-1 Antigen/immunology , Lymphoproliferative Disorders/immunology , Skin Diseases/immunology , Skin/pathology , T-Lymphocytes, Helper-Inducer/immunology , Adolescent , Adult , Aged , Aged, 80 and over , Carcinoma/pathology , Child , Female , Humans , Hyperplasia/immunology , Male , Middle Aged , Retrospective Studies , Young Adult
6.
São Paulo; s.n; 2015. 87 p. ilus, tab, graf.
Thesis in Portuguese | LILACS | ID: biblio-972068

ABSTRACT

INTRODUÇÃO: A micose fungóide foliculotrópica (MFF) é subtipo de linfoma cutâneo de células T que atinge especialmente o folículo piloso e parece ter prognóstico mais reservado. Informações clínicas sobre a população acometida por linfomas cutâneos no Brasil são escassas. O fenômeno de imunoprivilégio (IP) diz respeito à habilidade de alguns órgãos em permanecer protegidos contra reações inflamatórias. Tem sido sugerido que o folículo piloso normal represente um local de IP. Nesse estudo aventou-se a possibilidade de haver uma quebra no equilíbrio desse fenômeno na MFF, com alteração na expressão de moléculas do complexo maior de histocompatibilidade (MHC) e na expressão de MHC não-clássicos (HLA-G), com algum papel no mecanismo do foliculotropismo. Os objetivos foram: descrever o perfil clínico-epidemiológico de paciente com MFF, descrever a histologia e imunofenótipo dos casos de MFF e investigar os mecanismos envolvidos na predileção dos linfócitos atípicos pelo folículo piloso. METODOLOGIA: Os prontuários de pacientes com diagnóstico de MFF provenientes do ambulatório de Linfomas Cutâneos da Faculdade de Medicina da Universidade de São Paulo (FMUSP) foram revisados (n=33). O material histológico de biópsias de pele dos pacientes com MFF provenientes dos ambulatórios de Linfomas Cutâneos da FMUSP e da Northwestern University foi analisado por meio de escala semi-quantitativa (n=43). Na coloração de hematoxilina-eosina foram avaliados os seguintes parâmetros: infiltrado neoplásico epidérmico, infiltrado neoplásico dérmico, presença de acantose/espongiose, de mucinose folicular, de fibroplasia do tecido conjuntivo, de eosinófilos, de plasmócitos, o tamanho celular e o grau de dano folicular. Analisou-se a positividade do infiltrado neoplásico para os seguintes marcadores celulares: CD1a, CD56, TIA-1 e CD117. As expressões do complexo de histocompatibilidade HLA-G e do MHCII no infiltrado celular e no epitélio folicular foram investigadas no grupo de...


INTRIODUCTION: Folliculotropic mycosis fungoides (FMF) is a subtype of cutaneous T cells lymphoma affecting mainly the hair follicle and seems to have a less favorable prognosis. Clinical information on the population affected by cutaneous lymphomas in Brazil is scarce. The immune privilege (IP) phenomenon involves the ability of some body sites remaining protected from inflammatory reactions. It has been suggested that normal hair follicle represents an IP location. We hypothesized that a collapse of this phenomenon would occur in FMF, with changes in the expression of classical major histocompatibility molecules (MHC) and in the expression of nonclassical MHC molecules (HLA-G) with a role in folliculotropism mechanism. The objectives of this study were to describe the clinical and epidemiological profile of patients with MFF, describe the histology and immunophenotype of cases of MFF and investigate the expression of MHC molecules. METHODS: The medical records of patients from the outpatient Cutaneous Lymphoma Clinic of the University of Sao Paulo Medical School (FMUSP) diagnosed with MFF were reviewed (n = 33). The histological material from skin biopsies of patients with MFF from the Cutaneous Lymphomas Clinic of FMUSP and Northwestern University was stained and evaluated by semi-quantitative scale. In hematoxylin-eosin staining the following parameters were evaluated: epidermal neoplastic infiltrate, dermal neoplastic infiltrate, acanthosis/spongiosis, follicular mucinosis, connective tissue fibroplasia, presence of eosinophils and plasma cells, cell size and degree of follicular damage. We analyzed the positivity of the neoplastic infiltrate for the following cellular markers: CD1a, CD56, TIA-1, and CD117. Finally, the expression of histocompatibility complex HLA-G and MHC II in the neoplastic infiltrate and the follicular epithelium was investigated in MFF group and compared to patients with classical mycosis fungoides (CMF) and to normal skin. MHCII...


Subject(s)
Humans , Adolescent , Young Adult , Adult , Middle Aged , Aged , Aged, 80 and over , Mycosis Fungoides , Lymphoma, T-Cell, Cutaneous , Hair Follicle , Immunophenotyping
7.
Am J Dermatopathol ; 36(11): 875-81, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25238449

ABSTRACT

Chronic actinic dermatitis/actinic reticuloid (CAD/AR) is an eczematous hypersensitivity reaction to ultraviolet rays that can vary from mild eczematous cases to AR, the most severe cases which may resemble cutaneous T-cell lymphoma. Diagnosis is based on clinical, histopathologic, and photobiologic features. In this study, we characterize the histopathologic and immunohistochemical features of 40 biopsies from 37 patients with established CAD. The cohort included 30 men and 7 women, ranging in age from 38 to 84 years (median, 62 years) and with a median duration of symptoms at presentation of 3 years (range, 1 to 40 years). All patients presented with erythematous lichenified plaques on sun-exposed areas. Severe cases (12/37) had extension to non-exposed areas. Positive photo-testing (20/20) and patch-testing (10/10) results, and cases with a high peripheral blood eosinophila (7/24) and HIV positivity (4/37) were noted. Skin biopsies demonstrated eczematous features including parakeratosis, acanthosis, spongiosis, and prominent dermal fibroplasia. Dermal dendrocytes were prominent in all cases with frequent multinucleated giant cells positive for factor XIIIa and S100 protein. Most cases displayed a brisk lymphocytic infiltrate with subtle exocytosis, atypical lymphocytes, and increased numbers of Langerhans cells, eosinophils, and plasma cells. There was a predominance of CD8 T cells within the epidermis (20/25) and a low CD4:CD8 ratio was noted in 20 of 25 cases. T-cell clonality studies were negative in 10 of 10 cases. CAD/AR may be difficult to distinguish from eczematous variants of cutaneous T-cell lymphoma. Important clues to differentiate both conditions include the identification of prominent dermal dendrocytes with multinucleated giant cells, eosinophils, plasma cells, and a low CD4:CD8 ratio.


Subject(s)
Immunohistochemistry , Photosensitivity Disorders/metabolism , Photosensitivity Disorders/pathology , Skin/chemistry , Skin/pathology , Adult , Aged , Aged, 80 and over , Biomarkers/analysis , Biopsy , Diagnosis, Differential , Female , Humans , Lymphoma, T-Cell, Cutaneous/chemistry , Lymphoma, T-Cell, Cutaneous/immunology , Lymphoma, T-Cell, Cutaneous/pathology , Male , Middle Aged , Photosensitivity Disorders/immunology , Predictive Value of Tests , Skin/immunology
8.
ISRN Dermatol ; 2014: 950754, 2014.
Article in English | MEDLINE | ID: mdl-25006489

ABSTRACT

Background. Cryopeeling is a technique that uses cryotherapy not only on actinic keratoses lesions, but also all over the photodamaged skin. Objectives. To investigate the histological changes induced by two cryopeeling methods (liquid nitrogen (LN) and portable system (PS)). Methods. Sixteen patients (n = 16) with multiple actinic keratoses on the forearms were treated with cryopeeling technique using LN for one forearm and PS for the other, randomly. Skin biopsies were taken before and after the procedures. Results. There was no statistical difference between the epidermal and Grenz zone thicknesses or density of elastic fibers after treatments. The amount of melanin pigment was lower after PS treatment (P < 0.05). In a blind analysis of paired pre- and postprocedure slides, it was not possible to identify cases which underwent treatment, both in global analysis of quality of the skin and in specific analysis (considering only the aspect of stratum corneum). Discussion. The results indicate the inconsistency of histological improvement after treatments, and, likely, since the method causes superficial exfoliation, a reliable marker was not found in the analysis. Conclusions. Despite cosmetic benefits on photodamaged skin and efficient treatment of actinic keratoses lesions, cryopeeling was not able to induce measurable histological changes in solar elastosis, epidermal organization, or epidermal and Grenz zone thicknesses. One should keep in mind the possibility of hypopigmentation risk of the method.

9.
J Am Acad Dermatol ; 71(3): 555-60, 2014 Sep.
Article in English | MEDLINE | ID: mdl-24813299

ABSTRACT

BACKGROUND: Granulomatous cutaneous T-cell lymphoma (G-CTCL) is a rarely encountered entity. Most G-CTCL is CD4(+), with granulomatous mycosis fungoides representing the vast majority of cases. Because of the rarity of CD8(+) G-CTCL, there is a paucity of data regarding the clinicopathologic features and expected course. OBJECTIVE: To describe the clinical and histopathologic features of G-CTCL. METHODS: This is a retrospective review of collected cases. RESULTS: We present 4 cases of CD8(+) G-CTCL. Patients presented with papules and nodules on the trunk and extremities without antecedent patch or plaque disease. In all cases, biopsy specimens were obtained, and these revealed a dense granulomatous infiltrate accompanied by an atypical lymphoid infiltrate of CD8(+) T cells. T-cell clonality studies were positive in 3 of 4 cases. Staging was negative for nodal involvement, but lung granulomas were seen in all cases. In all 4 cases, the patient's medical history was significant for immunodeficiency, either primary or iatrogenic. All 4 patients had slowly progressive disease. LIMITATIONS: This is a small retrospective case series. CONCLUSIONS: CD8(+) G-CTCL appears to be associated with immunodeficiency. The finding of a CD8(+) G-CTCL should prompt an evaluation for underlying immunodeficiency. Additional studies are required to validate these conclusions.


Subject(s)
CD8-Positive T-Lymphocytes/pathology , Immunocompromised Host , Lymphoma, T-Cell, Cutaneous/pathology , Skin Neoplasms/pathology , Adult , Agammaglobulinemia/immunology , Agammaglobulinemia/metabolism , Agammaglobulinemia/pathology , Aged , CD8-Positive T-Lymphocytes/immunology , CD8-Positive T-Lymphocytes/metabolism , Common Variable Immunodeficiency/immunology , Common Variable Immunodeficiency/metabolism , Common Variable Immunodeficiency/pathology , Disease Progression , Genetic Diseases, X-Linked/immunology , Genetic Diseases, X-Linked/metabolism , Genetic Diseases, X-Linked/pathology , Granuloma/immunology , Granuloma/pathology , Humans , Lymphoma, T-Cell, Cutaneous/immunology , Lymphoma, T-Cell, Cutaneous/metabolism , Male , Middle Aged , Mycosis Fungoides/immunology , Mycosis Fungoides/metabolism , Mycosis Fungoides/pathology , Retrospective Studies , Skin Neoplasms/immunology , Skin Neoplasms/metabolism
10.
Case Rep Dermatol Med ; 2014: 639537, 2014.
Article in English | MEDLINE | ID: mdl-24826352

ABSTRACT

Dyschromatosis symmetrica hereditaria (DSH), also known as reticulated acropigmentation of Dohi, is an autosomal dominant disease with high penetrance, characterized by hypo- and hyperpigmented macules of varying sizes on the dorsal of the extremities with reticulated pattern. This paper presents a female patient with typical dermatological lesions, but only diagnosed in adulthood. It is necessary to perform differential diagnosis with other pigmentary disorders. This entity is not very common in South America, and the vast majority of cases were described in Japanese population. Since it is a benign disease, it is important to be aware of this diagnosis in order to establish the correct conduct for these patients.

11.
JAMA Dermatol ; 150(4): 412-8, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24500411

ABSTRACT

IMPORTANCE: To our knowledge, this is the first comprehensive study addressing comorbidities associated with primary cutaneous marginal zone B-cell lymphomas (PCMZLs). OBJECTIVE: To determine if patients with PCMZL were at risk for other medical conditions. DESIGN, SETTING, AND PARTICIPANTS: Case-control study at a cutaneous lymphoma clinic and a dermatopathologic consultation service at a single academic institution using an extensive questionnaire of illnesses, symptoms, and environmental exposures for 80 sequential patients with PCMZL and 80 matched controls. MAIN OUTCOMES AND MEASURES: The frequency of several morbidities was obtained in both groups from data gathered from the questionnaire and corroborated by reviewing medical records when available. RESULTS: We found a high incidence of past or present gastrointestinal tract problems in 49 patients (61.2%) with PCMZL compared with 30 participants (37.5%) in the control group (CG) (P = .003). Gastroesophageal reflux was reported in 50.0% (40 vs 27 in the CG, P = .04) and gastric ulcers in 10.0% (8 vs 3 in the CG, P = .13); 20.0% of the cohort had positive Helicobacter pylori serology (16 vs 2 in the CG, P = .003). Colon disorders, including irritable bowel syndrome and inflammatory bowel disease, were more common in the PCMZL cohort (20 vs 7 in the CG, P = .01). Autoimmunity was reported in 20.0% of participants (16 vs 6 in the CG, P = .03). Eight patients had a history of Hashimoto thyroiditis. Three patients had a positive antinuclear antibody. Two had a diagnosis of lupus erythematosus and 1 had Sjögren syndrome. Sicca syndrome was noted in 12.5% (10 vs 3 in the CG, P = .052). A history of noncutaneous malignant neoplasms was observed in 21.3% of the patients (17 vs 8 in the CG, P = .050). Other notable morbidities did not reach statistical significance. CONCLUSIONS AND RELEVANCE: Our results indicate a high incidence of systemic conditions in patients with PCMZL, especially involving the gastrointestinal tract, but also autoimmunity and cancer.


Subject(s)
Autoimmunity , Gastrointestinal Diseases/epidemiology , Helicobacter Infections/epidemiology , Helicobacter pylori , Lymphoma, B-Cell, Marginal Zone/epidemiology , Skin Neoplasms/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Antibodies, Antinuclear/blood , Case-Control Studies , Comorbidity , Female , Hashimoto Disease/epidemiology , Humans , Incidence , Lupus Erythematosus, Systemic/epidemiology , Lymphoma, B-Cell, Marginal Zone/blood , Lymphoma, B-Cell, Marginal Zone/pathology , Male , Middle Aged , Neoplasms/epidemiology , Sjogren's Syndrome/epidemiology , Skin Neoplasms/blood , Skin Neoplasms/pathology , Surveys and Questionnaires , Young Adult
12.
Am J Dermatopathol ; 36(5): 402-8, 2014 May.
Article in English | MEDLINE | ID: mdl-24394306

ABSTRACT

We report 7 cases of a CD8 lymphoid proliferation of the ear and face with a cytotoxic T-cell phenotype, but an indolent clinical course. All patients presented with stable or slowly growing asymptomatic lesions on the ear, nose, or lower eyelid. Histopathology showed a dense diffuse dermal infiltrate of small- to medium-sized atypical lymphocytes without destructive features. The lymphocytes were positive for CD3, CD8, ß-F1, and TIA-1 and negative for CD4, CD30, CD56, granzyme B, and PD-1. Of note, the proliferation index was low in available cases. All patients remained in complete remission at median follow-up of 14 months regardless of treatment modality. Staging was negative for extracutaneous disease in all patients. The clinically indolent behavior and histopathologic phenotype together with a low proliferation index (10%-15%) emphasize the importance of accurate diagnosis and appropriate clinical management to avoid overtreatment and complications of therapy.


Subject(s)
CD8-Positive T-Lymphocytes/pathology , Lymphoma, T-Cell, Cutaneous/pathology , Skin Diseases/pathology , Adult , Aged , Aged, 80 and over , Biomarkers, Tumor/analysis , Ear/pathology , Face/pathology , Female , Humans , Immunohistochemistry , Male , Middle Aged
14.
Semin Cutan Med Surg ; 31(4): 234-40, 2012 Dec.
Article in English | MEDLINE | ID: mdl-23174493

ABSTRACT

The purpose of this review is to summarize the most important molecular techniques for the diagnosis of cutaneous lymphomas. When making a diagnosis, we are looking for the solid clinicopathological correlation. Molecular analysis includes immunophenotyping and clonality analysis, and is important for 2 principal reasons: (1) to confirm the diagnosis in cases where the clinical and/or pathological presentations are nondiagnostic, and (2) to further characterize the nature of the lymphoma. More specifically, we are trying to discern whether the lymphoma is primarily cutaneous or systemic with secondary skin involvement, and we are also attempting to subclassify the tumor. Recently, many techniques have provided a more accurate diagnosis of cutaneous lymphomas and some prognostic implications, including polymerase chain reaction, fluorescence in situ hybridization, and flow cytometry. Fluorescence in situ hybridization is not routinely used in the diagnosis of cutaneous lymphoma, but many studies have shown potential future applications in various areas. Other techniques, such as comparative genomic hybridization, are still confined to the research arena, but have added some insight into the molecular pathogenesis of cutaneous T-cell lymphoma.


Subject(s)
Cytogenetic Analysis/methods , Lymphoma, B-Cell/genetics , Lymphoma, T-Cell, Cutaneous/genetics , Comparative Genomic Hybridization , Flow Cytometry/methods , Gene Rearrangement, T-Lymphocyte/genetics , Humans , Immunophenotyping , In Situ Hybridization, Fluorescence/methods , Lymphoma, B-Cell/pathology , Lymphoma, Non-Hodgkin , Lymphoma, T-Cell, Cutaneous/pathology , Oligonucleotide Array Sequence Analysis , Skin Neoplasms/genetics , Skin Neoplasms/pathology
15.
Am J Surg Pathol ; 36(11): 1656-65, 2012 Nov.
Article in English | MEDLINE | ID: mdl-23073324

ABSTRACT

We reviewed our multicenter experience with gamma-delta (γδ) T-cell lymphomas first presenting in the skin. Fifty-three subjects with a median age of 61 years (range, 25 to 91 y) were diagnosed with this disorder. The median duration of the skin lesions at presentation was 1.25 years (range, 1 mo to 20 y). The most common presentation was deep plaques (38 cases) often resembling a panniculitis, followed by patches resembling psoriasis or mycosis fungoides (10 cases). These lesions tended to ulcerate overtime (27 cases). Single lesions or localized areas of involvement resembling cellulitis or pyoderma were reported in 8 cases. The most common anatomic site of involvement was the legs (40 cases), followed by the torso (30 cases) and arms (28 cases). Constitutional symptoms were reported in 54% (25/46) of the patients, including some with limited skin involvement. Significant comorbidities included autoimmunity (12 cases), other lymphoproliferative disorders (5 cases), internal carcinomas (4 cases), and viral hepatitis (2 cases). Lymphadenopathy (3/42 cases) and bone marrow involvement (5/28 cases) were uncommon, but serum lactose dehydrogenase (LDH) was elevated in 55% (22/39) of the patients. Abnormal positron emission tomography and/or computed tomography scans in 20/37 subjects mostly highlighted soft tissue or lymph nodes. Disease progression was associated with extensive ulcerated lesions resulting in 27 deaths including complications of hemophagocytic syndrome (4) and cerebral nervous system involvement (3). Median survival time from diagnosis was 31 months. Skin biopsies varied from a pagetoid pattern to purely dermal or panniculitic infiltrates composed of intermediate-sized lymphocytes with tissue evidence of cytotoxicity. The most common immunophenotype was CD3+/CD4⁻/CD5⁻/CD8⁻/BF1⁻/γ-M1+/TIA-1+/granzyme-B+/CD45RA-/CD7-, and 4 cases were Epstein-Barr virus positive. This is the largest study to date of cutaneous γδ T-cell lymphomas and demonstrates a variety of clinical and pathologic presentations with a predictable poor outcome.


Subject(s)
Lymphoma, T-Cell, Cutaneous/diagnosis , Receptors, Antigen, T-Cell, gamma-delta/metabolism , Skin Neoplasms/diagnosis , T-Lymphocytes, Cytotoxic/pathology , Adult , Aged , Aged, 80 and over , Brain Neoplasms/epidemiology , Brain Neoplasms/pathology , Comorbidity , Diagnosis, Differential , Female , Humans , L-Lactate Dehydrogenase/blood , Lymph Nodes/pathology , Lymphohistiocytosis, Hemophagocytic/epidemiology , Lymphohistiocytosis, Hemophagocytic/pathology , Lymphoma, T-Cell, Cutaneous/metabolism , Lymphoma, T-Cell, Cutaneous/mortality , Male , Middle Aged , Positron-Emission Tomography , Skin Neoplasms/metabolism , Skin Neoplasms/mortality , T-Lymphocytes, Cytotoxic/metabolism , Tomography, X-Ray Computed , United States/epidemiology
16.
Dermatol Clin ; 30(4): 749-61, vii-viii, 2012 Oct.
Article in English | MEDLINE | ID: mdl-23021057

ABSTRACT

Recent epidemiology studies have identified a steady increase in the incidence of cutaneous lymphomas over the past few decades. Although possible explanations for this increased incidence include heightened awareness of these conditions as well as a more refined diagnostic acuity by dermatologists and pathologists, an increase secondary to environmental factors cannot be discounted. Our understanding of cutaneous lymphomas keeps evolving. Consequently, our knowledge and understanding of cutaneous lymphomas requires reconsideration of past dogma and critical revision of the new proposals. In this article, some hot topics and important new findings in the field are reviewed.


Subject(s)
Lymphoma, B-Cell, Marginal Zone/pathology , Lymphoma, T-Cell, Cutaneous/pathology , Lymphoma, T-Cell/pathology , Multiple Myeloma/pathology , Panniculitis/pathology , Skin Neoplasms/pathology , Antigens, CD/metabolism , Humans , Immunohistochemistry , Lymphoma, B-Cell, Marginal Zone/classification , Lymphoma, T-Cell, Cutaneous/classification , Lymphoma, T-Cell, Cutaneous/metabolism , T-Lymphocytes, Helper-Inducer/metabolism
17.
An Bras Dermatol ; 86(3): 440-4, 2011.
Article in English, Portuguese | MEDLINE | ID: mdl-21738958

ABSTRACT

BACKGROUND: Cryopelling uses diffuse cryotherapy not only on lesions of actinic keratosis but all over the photodamaged skin. OBJECTIVES: The aim of this study was to compare two cryopeeling methods (liquid nitrogen- LN and portable system - PS) and demonstrate their efficiency in the treatment of actinic keratoses, patient tolerance, researcher and patient preference and aesthetic results. METHODS: Sixteen patients (N = 16) with multiple actinic keratoses on the forearms were subjected to cryopeeling with LN on one of the forearms and PS on the other, randomly. RESULTS: In the treatment of actinic keratoses, LN obtained 74% efficiency and PS, 62% (p = 0.019). The mean visual analogue scale (0-10) was 5.7 ± 1.61 with LN and 4.3 ± 1.44 with PS (p = 0.003). There was no significant statistical difference between the two methods in terms of researcher and patient preference. An analysis of the photos showed improvement of the skin appearance with both treatments (p <0.001). Treatment with LN obtained some degree of improvement in 62.5% of the cases, while treatment with PS obtained some degree of improvement in 52% of the cases (p> 0.05). DISCUSSION: Treatment with the PS showed better tolerance, but was less efficient than LN. Although LN has been the preferred method, there was no statistical difference between the methods. CONCLUSIONS: The cryopeeling technique may be an option in the treatment of photodamage. The PS can be an interesting alternative in clinical practice with good tolerance and acceptable results in the treatment of actinic keratoses.


Subject(s)
Cryosurgery/methods , Keratosis, Actinic/surgery , Nitrogen/therapeutic use , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Treatment Outcome
18.
An. bras. dermatol ; 86(3): 440-444, maio-jun. 2011. ilus, graf
Article in Portuguese | LILACS | ID: lil-592138

ABSTRACT

FUNDAMENTOS: O criopeeling utiliza a crioterapia difusa não somente nas lesões de ceratose actínica, mas em toda a pele fotodanificada. OBJETIVOS: Comparar dois métodos de criopeeling (nitrogênio líquido e sistema portátil de éter dimetílico, propano e isobutano) quanto à eficiência no tratamento de ceratoses actínicas, tolerabilidade do paciente, preferência do paciente e do pesquisador e resultado estético. MÉTODOS: Dezesseis pacientes (n=16) com múltiplas ceratoses actínicas nos antebraços foram submetidos ao criopeeling com nitrogênio líquido em um dos antebraços e com o sistema portátil no outro, randomicamente. RESULTADOS: No tratamento das ceratoses actínicas, o nitrogênio líquido obteve 74 por cento de eficiência e o sistema portátil, 62 por cento (p=0,019). A média da escala visual analógica (0-10) foi 5,7±1,61 com o nitrogênio líquido e 4,3±1,44 com o sistema portátil (p=0,003). Não houve diferença estatística entre os métodos quanto à preferência do paciente e do pesquisador. Na análise das fotos, observou-se melhora do aspecto da pele nos dois tratamentos (p<0,001). Com o nitrogênio líquido, em 62,5 por cento das vezes houve algum grau de melhora; com o sistema portátil, em 52 por cento (p>0,05). CONCLUSÕES: A técnica de criopeeling pode ser uma opção no tratamento de fotodano. O sistema portátil pode ser uma alternativa interessante na prática clínica, com boa tolerância e resultados aceitáveis no tratamento de ceratoses actínicas.


BACKGROUND: Cryopelling uses diffuse cryotherapy not only on lesions of actinic keratosis but all over the photodamaged skin. OBJECTIVES: The aim of this study was to compare two cryopeeling methods (liquid nitrogen- LN and portable system - PS) and demonstrate their efficiency in the treatment of actinic keratoses, patient tolerance, researcher and patient preference and aesthetic results. METHODS: Sixteen patients (N = 16) with multiple actinic keratoses on the forearms were subjected to cryopeeling with LN on one of the forearms and PS on the other, randomly. RESULTS: In the treatment of actinic keratoses, LN obtained 74 percent efficiency and PS, 62 percent (p = 0.019). The mean visual analogue scale (0-10) was 5.7 ± 1.61 with LN and 4.3 ± 1.44 with PS (p = 0.003). There was no significant statistical difference between the two methods in terms of researcher and patient preference. An analysis of the photos showed improvement of the skin appearance with both treatments (p <0.001). Treatment with LN obtained some degree of improvement in 62.5 percent of the cases, while treatment with PS obtained some degree of improvement in 52 percent of the cases (p> 0.05). Discussion: Treatment with the PS showed better tolerance, but was less efficient than LN. Although LN has been the preferred method, there was no statistical difference between the methods. CONCLUSIONS: The cryopeeling technique may be an option in the treatment of photodamage. The PS can be an interesting alternative in clinical practice with good tolerance and acceptable results in the treatment of actinic keratoses.


Subject(s)
Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Cryosurgery/methods , Keratosis, Actinic/surgery , Nitrogen/therapeutic use , Treatment Outcome
19.
Brain Res Brain Res Protoc ; 16(1-3): 58-64, 2005 Dec.
Article in English | MEDLINE | ID: mdl-16310404

ABSTRACT

Parkinson's disease is a progressive dyskinetic disorder caused by degeneration of mesencephalic dopaminergic neurons in the substantia nigra pars compacta (SNpc) and, to a lesser extent, in the ventral tegmental area (VTA). Tyrosine hydroxylase (TH) is a rate-limiting enzyme for dopamine synthesis, therefore immunohistochemistry for TH can be used as an important marker of dopaminergic cell loss in these regions. Traditionally, immunohistochemical experiments are analyzed qualitatively by optical microscopic observation or more rarely semi-quantitatively evaluated by densitometry. A common problem with such papers is the lack of a clear explanation of the algorithms and macros employed in the semi-quantitative approaches. In this paper, we describe, in detail, an easy, fast and precise protocol for the analysis of TH immunoreactivity in SNpc and VTA using one of the most popular image analysis software packages (Image Pro-Plus). We believe that this protocol will facilitate the evaluation of mesencephalic TH immunoreactivity in various available animal models of Parkinson's disease.


Subject(s)
Substantia Nigra/enzymology , Tyrosine 3-Monooxygenase/metabolism , Ventral Tegmental Area/enzymology , Animals , Calibration , Cell Count , Densitometry , Image Processing, Computer-Assisted , Immunohistochemistry , Indicators and Reagents , Oxidopamine , Rats , Rats, Wistar , Substantia Nigra/drug effects , Sympathectomy, Chemical , Sympatholytics , Tissue Fixation , Ventral Tegmental Area/drug effects
SELECTION OF CITATIONS
SEARCH DETAIL
...