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1.
Int J Gynecol Cancer ; 33(10): 1548-1556, 2023 10 02.
Article in English | MEDLINE | ID: mdl-37699707

ABSTRACT

OBJECTIVES: To evaluate the prevalence of post-operative complications and quality of life (QoL) related to sentinel lymph node (SLN) biopsy vs systematic lymphadenectomy in endometrial cancer. METHODS: A prospective cohort included women with early-stage endometrial carcinoma who underwent lymph node staging, grouped as follows: SLN group (sentinel lymph node only) and SLN+LND group (sentinel lymph node biopsy with addition of systematic lymphadenectomy). The patients had at least 12 months of follow-up, and QoL was assessed by European Organization for Research and Treatment of Cervical Cancer Quality of Life Questionnaire 30 (EORTC-QLQ-C30) and EORTC-QLQ-Cx24. Lymphedema was also assessed by clinical evaluation and perimetry. RESULTS: 152 patients were included: 113 (74.3%) in the SLN group and 39 (25.7%) in the SLN+LND group. Intra-operative surgical complications occurred in 2 (1.3%) cases, and all belonged to SLN+LND group. Patients undergoing SLN+LND had higher overall complication rates than those undergoing SLN alone (33.3% vs 14.2%; p=0.011), even after adjusting for confound factors (OR=3.45, 95% CI 1.40 to 8.47; p=0.007). The SLN+LND group had longer surgical time (p=0.001) and need for admission to the intensive care unit (p=0.001). Moreover, the incidence of lymphocele was found in eight cases in the SLN+LND group (0 vs 20.5%; p<0.001). There were no differences in lymphedema rate after clinical evaluation and perimetry. However, the lymphedema score was highest when lymphedema was reported by clinical examination at 6 months (30.1 vs 7.8; p<0.001) and at 12 months (36.3 vs 6.0; p<0.001). Regarding the overall assessment of QoL, there was no difference between groups at 12 months of follow-up. CONCLUSIONS: There was a higher overall rate of complications for the group undergoing systematic lymphadenectomy, as well as higher rates of lymphocele and lymphedema according to the symptom score. No difference was found in overall QoL between SLN and SLN+LND groups.


Subject(s)
Endometrial Neoplasms , Lymphedema , Lymphocele , Humans , Female , Quality of Life , Prospective Studies , Sentinel Lymph Node Biopsy/adverse effects , Lymph Nodes/surgery , Lymph Nodes/pathology , Lymph Node Excision/adverse effects , Endometrial Neoplasms/pathology , Prevalence , Lymphedema/epidemiology , Lymphedema/etiology , Lymphedema/pathology , Neoplasm Staging , Retrospective Studies
2.
Ger Med Sci ; 21: Doc04, 2023.
Article in English | MEDLINE | ID: mdl-37405192

ABSTRACT

Background: Lymphedema is a chronic, progressive clinical condition that evolves with intense fibrosis, the most advanced stage of which is stage III (lymphostatic fibrosclerosis). Aim: The aim of the present study was to show the possibility to reconstruct the dermal layers with the intensive treatment of fibrosis using the Godoy method. Case description: A 55-year-old patient with an eight-year history of edema of the lower limb of the leg had constant episodes of erysipelas, despite regular treatments. The edema progressed continually, associated with a change in the color of the skin and the formation of a crust. Intensive treatment (eight hours per day for three weeks) was proposed with the Godoy method. The ultrasound was performed and results revealed substantial improvement in the skin, with the onset of the reconstruction of the dermal layers. Conclusion: It is possible to reconstruct the layers of the skin in fibrotic conditions caused by lymphedema.


Subject(s)
Dermis , Fibrosis , Lymphedema , Skin Diseases , Humans , Middle Aged , Chronic Disease , Fibrosis/diagnostic imaging , Fibrosis/etiology , Fibrosis/pathology , Fibrosis/therapy , Lymphedema/complications , Lymphedema/diagnostic imaging , Lymphedema/pathology , Lymphedema/therapy , Skin/diagnostic imaging , Skin/pathology , Skin Diseases/complications , Skin Diseases/diagnostic imaging , Skin Diseases/pathology , Skin Diseases/therapy , Dermis/diagnostic imaging , Dermis/pathology , Ultrasonography/methods
3.
An Bras Dermatol ; 98(3): 287-295, 2023.
Article in English | MEDLINE | ID: mdl-36746732

ABSTRACT

BACKGROUND: stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. OBJECTIVES: This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. METHODS: Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. RESULTS: The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. STUDY LIMITATIONS: Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. CONCLUSIONS: STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.


Subject(s)
Hemangiosarcoma , Lymphangiosarcoma , Lymphedema , Humans , Young Adult , Adult , Retrospective Studies , Lymphangiosarcoma/complications , Lymphangiosarcoma/diagnosis , Hemangiosarcoma/complications , Hemangiosarcoma/diagnosis , Hemangiosarcoma/pathology , Lymphedema/complications , Lymphedema/pathology , Chronic Disease
4.
Rev. cir. (Impr.) ; 73(2): 197-202, abr. 2021. ilus
Article in Spanish | LILACS | ID: biblio-1388814

ABSTRACT

Resumen Introducción: El linfocele es una patología que puede ocurrir por la disrupción linfática durante una cirugía, siendo frecuente luego de una linfadenectomía inguinal. Existen diversos enfrentamientos para prevenirlo o minimizarlo, sin embargo, los resultados son inconsistentes. Caso clínico: Reportamos el caso de una mujer, con linfocele recurrente en zona inguinal derecha y linfedema distal de la extremidad secundario a una biopsia ganglionar, tratado exitosamente mediante supermicrocirugía para restaurar el drenaje linfático. La extremidad inferior tenía un exceso de volumen de 7,03%. Se realizaron estudios preoperatorios con linfografía por resonancia magnética y linfografía con verde de indocianina para identificar los vasos linfáticos y realizar anastomosis linfático-venosas (ALV). Se identificaron tres vasos linfáticos aferentes y se realizó una capsulectomía total. Se realizaron tres ALV término-terminales supermicroquirúrgicas en zona inguinal y una ALV distal en pierna. Durante seguimiento no hubo recidiva del linfocele, evidenciándose una reducción del exceso de volumen de la extremidad afectada de un 105,26%. El linfocele inguinal y linfedema pueden ser tratados exitosamente mediante supermicrocirugía, restaurando el flujo linfático de manera fisiológica, evitando la recurrencia de linfocele y mejorando los síntomas del linfedema.


Introduction: Lymphocele may occur after the disruption of lymphatic channels during a surgical procedure. After inguinal lymphadenectomy are very common, and many different approaches have been tried to prevent or minimize the formation of lymphoceles with inconsistent results. Clinical Case: We report a case of a female patient who presented with right recurrent inguinal lymphocele and lower limb lymphedema after lymph-node biopsy that was successfully treated with lymphatic supermicrosurgery restoring the lymph flow. Lower extremity had an excess volume of 7,03% compared to the healthy contralateral limb. Preoperative study with magnetic resonance lymphangiography and indocyanine green lymphography were done to identify intraoperatively lymphocele afferent and distal lymphatic vessels to perform lymphovenous anastomosis (LVA). Three different afferent lymphatics were identified and total capsulectomy was performed. Three end-to- end supermicrosurgical LVA in the groin and one distal LVA on the leg were performed. The surgery was uneventful, and there were no postoperative complications. In the follow-up, no lymphocele was noticed and lymphedema had visibly reduced with a reduction of excess volume of 105.26%. Inguinal lymphocele and lymphedema can be successfully treated with supermicrosurgery since it is a physiological approach to restore the lymphatic flow, in order to avoid lymphocele recurrence and to improve lymphedema symptoms.


Subject(s)
Humans , Female , Aged , Lymphocele/etiology , Lymphedema/diagnosis , Microsurgery/methods , Lymphocele/complications , Treatment Outcome , Lymphedema/pathology
5.
Rev. argent. cir. plást ; 26(2): 88-91, apr-jun 2020. fig, tab
Article in Spanish | LILACS | ID: biblio-1147144

ABSTRACT

El lindefema masivo localizado es una condición rara pero su incidencia se encuentra en aumento. El linfedema puede ser primario o secundario a infecciones, radioterapia, cirugías, cáncer, enfermedades del sistema inmune o a compresión. Algunos autores han sugerido un incremento en su incidencia debido a la epidemia de obesidad. El linfedema escrotal produce una morbilidad importante debido a mala higiene, infecciones, incontinencia urinaria, disfunción sexual, pérdida de la libido e inclusive limitación en la deambulación. Las opciones de tratamiento no quirúrgico de la linfedema incluyen el drenaje linfático manual, prendas de compresión, terapia física y ejercicio. Usualmente, el tratamiento conservador falla debido a la dificultad anatómica que presenta la región genital para la aplicación de dichas terapias. El tratamiento quirúrgico puede ser efectivo en casos graves y moderados. Existen diferentes variantes a la técnica quirúrgica, pero la resección del tejido afectado y la reconstrucción con tejido sano es primordial. El pronóstico de esta patología tiende a ser bueno; sin embargo, se reporta una alta tasa de recidiva.


Massive localized lymphedema is a rare but rising condition that could be primary in nature or secondary to infections, radiation, surgery, malignancies, autoimmune or compression. Some authors have suggested and increased incidence due to the raising epidemic of obesity. Scrotal lymphedema causes significant morbidity such as poor hygiene, infections, urinary incontinence, sexual dysfunction, loss of libido and a limited ambulation. Although non operative treatment options for lymphedema include manual lymph drainage, compression garments, exercises, sequential gradient pump. Usually, non-operative techniques fail due to the difficult in the anatomic area. Surgical treatment can be effective in moderate to severe cases of giant scrotal lymphedema. There are many variations of the surgical technique, however, resection of the affected tissue and reconstruction with healthy skin is primordial. The prognosis tends to be good, although there is reported a significant amount of relapse, mostly dependent on the weight lost and diet of the patients


Subject(s)
Humans , Male , Adult , Scrotum/surgery , Lymphedema/pathology , Elephantiasis , Neoplasms , Obesity
6.
Cancer Rep (Hoboken) ; 2(2): e1143, 2019 04.
Article in English | MEDLINE | ID: mdl-32721135

ABSTRACT

BACKGROUND: Stewart Treves-Syndrome (STS) was first characterized as angiosarcoma in the homolateral limb of a patient with breast cancer and lymphedema. Now, other conditions represent STS. It's a rare condition. The diagnosis is easier in the presence of single or multiple purple nodules. Even though other dermatological aspects have been reported, no study has grouped its characteristics. AIM: Evaluate the dermatological characteristics of classical STS (c-STS). METHODS AND RESULTS: We report a patient with chronic lymphedema with a history of recurrent erysipelas that rapidly developed multiple papules in the superior limb. It was initially diagnosed as bullous erysipelas, but unsatisfactory evolution led to biopsy, which demonstrated an unsuspected epithelioid angiosarcoma. We have also performed a review of dermatologic aspects of c-STS using PubMed and Lilacs databases. PICTOS methodology and PRISMA flow chart were considered. The main dermatological aspects associated with c-CTS were summarized. Using a systematic evaluation from 109 articles, 29 were selected and 44 patients were described to whom we added one case. The mean time with lymphedema was 10 years. Of the patients analyzed, 97.2% were female; 95.6% were submitted to radical mastectomy; 81.2% presented with multiple lesions, 67.4% of the lesions were reported as nodules or tumors, 53.4% were purple, 33.4% were associated with an ecchymotic halo, and 33.4% were ulcerated lesions. CONCLUSION: When evaluating patients with chronic lymphedema with new dermatological abnormalities, clinical suspicion, or unfavorable evolution, the knowledge of clinical signs is important for diagnosis, and a biopsy must be considered. Papules associated with erythematous-wine color and bluish hematoma aspect must raise clinical suspicion.


Subject(s)
Erysipelas/diagnosis , Hemangiosarcoma/diagnosis , Lymphangiosarcoma/diagnosis , Mastectomy/adverse effects , Aged , Breast Neoplasms/diagnosis , Breast Neoplasms/pathology , Diagnosis, Differential , Erysipelas/pathology , Fatal Outcome , Female , Hemangiosarcoma/pathology , Humans , Lymphangiosarcoma/pathology , Lymphedema/diagnosis , Lymphedema/pathology
8.
Breast Cancer Res Treat ; 169(2): 251-256, 2018 Jun.
Article in English | MEDLINE | ID: mdl-29380209

ABSTRACT

PURPOSE: We studied the lymphatic drainage of the upper limb and mammary region directing to the axilla to investigate whether independent pathways can be observed or whether anastomoses and shared drainage occur between them. This analysis aimed to assess the safety of axillary reverse mapping (ARM) in breast cancer treatment and to understand the development of lymphedema after sentinel lymph node biopsy (SLNB) alone. METHODS: Seven unfixed stillborn fetuses were injected with a modified Gerota mass in the peri-areolar area, palm and dorsum of the hands, formalin fixed, and then submerged in 10% hydrogen peroxide solution. Microsurgical dissection was then performed on the subcutaneous cellular tissue of the upper limb, axillary region, and anterior thorax to expose the lymphatic vessels and lymph nodes. RESULTS: The dye injected into the upper limb reached either the lateral axillary group, known to be exclusively responsible for upper limb drainage, or the anterior group, which is typically related to breast drainage. There was great proximity among the pathways and lymph nodes. Communicating lymphatic vessels among these groups of lymph nodes were also found in all studied cases. DISCUSSION: Lymphedema remains a challenging morbidity in breast cancer treatment. ARM and SLNB aim to avoid unnecessary damage to the lymphatic drainage of the upper limb. However, our anatomical study suggests that ARM may have potential oncological risks because preserved lymph nodes may harbor malignant cells due their proximity, overlapping drainage pathways, and connecting lymph vessels among lymph nodes.


Subject(s)
Axilla/pathology , Breast Neoplasms/pathology , Lymphedema/pathology , Mammary Glands, Human/pathology , Upper Extremity/surgery , Axilla/surgery , Female , Fetus , Humans , Lymph Nodes/pathology , Lymph Nodes/surgery , Lymphedema/surgery , Mammary Glands, Human/surgery , Manual Lymphatic Drainage , Sentinel Lymph Node Biopsy , Stillbirth
9.
J Reconstr Microsurg ; 34(1): 29-34, 2018 Jan.
Article in English | MEDLINE | ID: mdl-28841735

ABSTRACT

BACKGROUND: Limb circumference measurements (CM) and perometry are the preferred methods for objectively measuring arm volume in lymphedema surgery research. Understanding the measurement bias involved in these measuring systems is important to properly interpret and compare studies and their results. METHODS: Arm volumes from 91 patients were measured using sequential girths and the truncated cone formula (CM) and with the use of an automated perometer (perometry). The absolute volume of the largest arm (V), the volume difference between the arms (VD), and the relative difference between them (percentage of excess volume [PEV]) were calculated with both methods. The agreement between methods was assessed by the Pearson's correlation test and the Bland-Altman's method. RESULTS: Correlations were strong for V (r = 0.99), VD (r = 88), and PEV (r = 0.86). Volumes measured by perometry were, on average, 10.6 mL smaller than volumes calculated from CM, while their limits of agreement (LOA) ranged from -202 to 181 mL. The LOA represents the range we could expect the arm volumes measured with the two methods to differ by chance alone, 95% of the times. For VD, LOA was -101 to 141 mL, with a mean difference of 19.9 mL, while PEV had a mean difference of 0.9%, with LOA ranging from -5 to 6.8%. CONCLUSION: There is considerable measurement error between arm volume estimated by perometry and by CM. Volumes calculated with these methods should be compared with caution. Furthermore, we observed an increasingly relevant measurement bias in outcomes that are mathematically derived from arm volumes.


Subject(s)
Arm/pathology , Dielectric Spectroscopy , Lymphedema/diagnosis , Skin Neoplasms/pathology , Adult , Brazil , Consensus , Female , Humans , Lymphedema/pathology , Male , Middle Aged , Observer Variation , Reproducibility of Results , Severity of Illness Index
10.
Einstein (Sao Paulo) ; 15(4): 457-464, 2017.
Article in English, Portuguese | MEDLINE | ID: mdl-29267425

ABSTRACT

OBJECTIVE: Translate to brazilian portuguese, culturally adapt and test the rating and classification scales of cervicofacial lymphedema of the MD Anderson Cancer Center Head and Neck Lymphedema Protocol (MDACC HNL) in patients undergoing treatment for head and neck cancer. METHODS: The process followed international guidelines and translation stages by two head and neck surgeons, and back translation independently by two native Americans. The test of final version was based on the evaluation of 18 patients by one speech pathologist and one physical therapist who applied the scales in Portuguese. RESULTS: The translation of the three scales was carried out independently and the translators reached a consensus for the final version. Minor modifications were made by translating two terms into the Assessment of the Face. Versions of back-translation were similar to each other. The instrument was successfully applied to patients independently. CONCLUSION: The translation and cultural adaptation of the assessment and rating scale of the cervicofacial lymphedema of the MD Anderson Cancer Center Head and Neck Lymphedema Protocol to the Brazilian Portuguese were successful.


Subject(s)
Head and Neck Neoplasms/therapy , Lymphedema/etiology , Surveys and Questionnaires/standards , Adult , Aged , Aged, 80 and over , Brazil , Cross-Cultural Comparison , Edema/etiology , Edema/pathology , Female , Fibrosis , Head and Neck Neoplasms/complications , Head and Neck Neoplasms/pathology , Humans , Language , Lymphedema/diagnosis , Lymphedema/pathology , Lymphedema/surgery , Male , Middle Aged , Neck Dissection/adverse effects , Neoplasm Staging , Reproducibility of Results , Translations
11.
An. bras. dermatol ; An. bras. dermatol;92(6): 847-850, Nov.-Dec. 2017. graf
Article in English | LILACS | ID: biblio-887131

ABSTRACT

Abstract: Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.


Subject(s)
Humans , Male , Middle Aged , Pregnenediones/therapeutic use , Isotretinoin/therapeutic use , Dermatologic Agents/therapeutic use , Erythema/drug therapy , Facial Dermatoses/drug therapy , Anti-Inflammatory Agents/therapeutic use , Recurrence , Treatment Outcome , Rosacea/complications , Erythema/pathology , Facial Dermatoses/pathology , Lymphedema/pathology , Lymphedema/drug therapy
12.
Einstein (Säo Paulo) ; 15(4): 457-464, Oct.-Dec. 2017. tab, graf
Article in English | LILACS | ID: biblio-891430

ABSTRACT

ABSTRACT Objective: Translate to brazilian portuguese, culturally adapt and test the rating and classification scales of cervicofacial lymphedema of the MD Anderson Cancer Center Head and Neck Lymphedema Protocol (MDACC HNL) in patients undergoing treatment for head and neck cancer. Methods: The process followed international guidelines and translation stages by two head and neck surgeons, and back translation independently by two native Americans. The test of final version was based on the evaluation of 18 patients by one speech pathologist and one physical therapist who applied the scales in Portuguese. Results: The translation of the three scales was carried out independently and the translators reached a consensus for the final version. Minor modifications were made by translating two terms into the Assessment of the Face. Versions of back-translation were similar to each other. The instrument was successfully applied to patients independently. Conclusion: The translation and cultural adaptation of the assessment and rating scale of the cervicofacial lymphedema of the MD Anderson Cancer Center Head and Neck Lymphedema Protocol to the Brazilian Portuguese were successful.


RESUMO Objetivo: Traduzir, para o português brasileiro, adaptar culturalmente e testar as escalas de avaliação e classificação do linfedema cérvico-facial do MD Anderson Cancer Center Head and Neck Lymphedema Protocol (MDACC HNL) em pacientes submetidos ao tratamento para o câncer de cabeça e pescoço. Métodos: O processo seguiu as diretrizes internacionais e as etapas de tradução por dois cirurgiões de cabeça e pescoço, além de retrotradução de forma independente por dois nativos norte-americanos. O teste da versão final foi realizado a partir da avaliação de 18 pacientes por um fonoaudiólogo e um fisioterapeuta, por meio da aplicação das escalas em português. Resultados: A tradução das três escalas foi realizada de forma independente, e os tradutores chegaram a um consenso para a versão final. Foram feitas pequenas modificações, ao serem traduzidos dois termos em Assessment of the Face . As versões da retrotradução foram semelhantes entre si. O instrumento foi aplicado com sucesso nos pacientes de forma independente. Conclusão: A tradução e a adaptação das escalas de avaliação e classificação do linfedema cérvico-facial do MD Anderson Cancer Center Head and Neck Lymphedema protocol para o português foram bem sucedidas.


Subject(s)
Humans , Male , Female , Adult , Aged , Aged, 80 and over , Surveys and Questionnaires/standards , Head and Neck Neoplasms/therapy , Lymphedema/etiology , Neck Dissection/adverse effects , Translations , Fibrosis , Brazil , Cross-Cultural Comparison , Reproducibility of Results , Edema/etiology , Edema/pathology , Head and Neck Neoplasms/complications , Head and Neck Neoplasms/pathology , Language , Lymphedema/surgery , Lymphedema/diagnosis , Lymphedema/pathology , Middle Aged , Neoplasm Staging
13.
Perm J ; 21: 16-010, 2017.
Article in English | MEDLINE | ID: mdl-28080951

ABSTRACT

INTRODUCTION: Lymphedema is characterized by a defect in the lymphatic system that causes limb swelling. Impaired uptake and transport of lymphatic fluid through lymphatic vessels causes accumulation of protein-rich fluid in the interstitial spaces, which leads to swelling of the limb. Primary lymphedema often presents at birth. The rare cases that arise after age 35 years are described as lymphedema tarda. The great majority of patients with lymphedema have swelling of the lower limbs-upper limb lymphedema is a rare disorder. CASE PRESENTATION: An 84-year-old woman presented with a 3-year history of unilateral swelling of the right upper limb. There were no constitutional symptoms and no evidence of lymphadenopathy or systemic disease. Blood tests, carcinoembryonic antigen test, computed tomography scans, and venous Doppler ultrasound were all normal. The diagnosis was primary upper limb lymphedema. DISCUSSION: The swelling that occurs in upper limb lymphedema is permanent and usually extends to the hand. About one-third of patients with this condition also present with lower limb lymphedema. Thorough investigations are warranted in cases of unilateral upper limb lymphedema to rule out occult malignancy and systemic disease.


Subject(s)
Lymphedema/pathology , Lymphedema/physiopathology , Upper Extremity/pathology , Upper Extremity/physiopathology , Aged, 80 and over , Female , Humans , Lymphedema/etiology
14.
Am J Dermatopathol ; 39(6): 479-484, 2017 Jun.
Article in English | MEDLINE | ID: mdl-27930381

ABSTRACT

One of the pathogenic causes of cutaneous inflammatory pseudotumors is chronic localized fibrosing leukocytoclastic vasculitis (CLFLCV), a vasculitic reaction pattern seen in granuloma faciale (GF), a localized vasculitis, and erythema elevatum diutinum (EED), a generalized vasculitis. Patients with myelodysplastic syndromes (MDSs) are at risk for a diverse spectrum of cutaneous neutrophilic dermatoses such as EED. Herein, we report a 74-year-old man who presented with a large ulcerative, fungating tumor affecting the right flexor ankle caused by CLFLCV. During his workup and management, MDS and Philadelphia chromosome-negative chronic myeloid leukemia was diagnosed. Surgical excision of the inflammatory mass promptly triggered tumor recurrence, whereas antineutrophil therapy (dapsone coupled with hydroxyurea, and prednisone) lead to tumor regression. Histopathologic examination revealed an eosinophilic-rich small-vessel neutrophilic vasculitis associated with storiform and angiocentric fibrosis (GF-like). In the regions of fibrosis, dilated lymphatic and vascular spaces were numerous, some of which were congested with small CD3-positive lymphocytes (intralymphatic and intravascular lymphocytosis). These findings indicate coexisting localized lymphedema. By direct immunofluorescence, IgM and C4d vessel deposits were detected. The pathogenesis of the fibrotic nodules and plaques of CLFLCV is suspected to be due to recurring bouts of immune-complex vasculitis, creating a cycle of vessel damage followed by reparative granulation tissue. Poor lymphatic drainage may be the underlying factor initiating and maintaining recurrent, localized episodes of immune-complex vasculitis and progressive fibrosis. Although his tumor histopathology resembled GF-eosinophilic rich CLFLCV-the clinical context points to a solitary and paraneoplastic case of EED.


Subject(s)
Granuloma, Plasma Cell/pathology , Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative/pathology , Lymphedema/pathology , Lymphocytosis/pathology , Paraneoplastic Syndromes/pathology , Vasculitis, Leukocytoclastic, Cutaneous/pathology , Aged , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy , Dapsone/therapeutic use , Fatal Outcome , Granuloma, Plasma Cell/immunology , Granuloma, Plasma Cell/therapy , Humans , Hydroxyurea/therapeutic use , Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative/drug therapy , Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative/genetics , Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative/immunology , Lymphedema/immunology , Lymphedema/therapy , Lymphocytosis/immunology , Lymphocytosis/therapy , Male , Paraneoplastic Syndromes/immunology , Paraneoplastic Syndromes/therapy , Pentoxifylline/therapeutic use , Prednisone/therapeutic use , Remission Induction , Treatment Outcome , Vasculitis, Leukocytoclastic, Cutaneous/immunology , Vasculitis, Leukocytoclastic, Cutaneous/therapy
15.
An Bras Dermatol ; 92(6): 847-850, 2017.
Article in English | MEDLINE | ID: mdl-29364446

ABSTRACT

Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.


Subject(s)
Anti-Inflammatory Agents/therapeutic use , Dermatologic Agents/therapeutic use , Erythema/drug therapy , Facial Dermatoses/drug therapy , Isotretinoin/therapeutic use , Pregnenediones/therapeutic use , Erythema/pathology , Facial Dermatoses/pathology , Humans , Lymphedema/drug therapy , Lymphedema/pathology , Male , Middle Aged , Recurrence , Rosacea/complications , Treatment Outcome
16.
Acta fisiátrica ; 23(4): 201-207, dez. 2016. ilus
Article in English | LILACS | ID: biblio-859516

ABSTRACT

Although gait problems have been reported in patients with lower limb lymphedema (LLL), the gait pattern (GP) changes have not been documented yet. However, it is possible that patients with LLL show abnormal GP that can be related to biomechanical complications related to osteoarthritis or falls affecting the quality of life. Ground reaction force analysis during gait allows objective assessment of the patients and it can be used to plan a rehabilitation approach. Objective: To analyze the GRF during gait in patients LLL. Methods: An experimental descriptive study was realized with twenty-three LLL patients, both unilateral and bilateral and classified as moderate and severe, participated in the experiments. The patients walked on a force plate while the three ground reaction force (GRF) components, vertical, mediolateral (M-L) and anteroposterior (A-P), under their feet were recorded and analyzed. Results: In the patients with unilateral lymphedema, either moderate or severe, the vertical GRF components of the affected limb were similar to the sound one and also resembling those found in healthy adults. The M-L GRF was smaller in the non-affected side. In patients with bilateral lymphedema gait speed was significantly slower. More interestingly, the vertical GRF pattern was flat, not showing the typical 2-peak shape. Finally, the large M-L forces found suggest gait stability problems. Conclusions: The patients showed abnormal GRF patterns, including compensation with the non-affected leg. The GRF variability was higher in the patients with severe unilateral lymphedema. Bilateral lymphedema results in lower A-P forces. Stance phase duration was longer in patients with bilateral and severe lymphedema


Subject(s)
Humans , Lower Extremity , Gait , Lymphedema/pathology , Obesity , Osteoarthritis/etiology , Epidemiology, Descriptive , Clinical Trial
17.
An. bras. dermatol ; An. bras. dermatol;91(5,supl.1): 119-121, Sept.-Oct. 2016. graf
Article in English | LILACS | ID: biblio-837915

ABSTRACT

Abstract Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled “volcanic crater contour” – the so-called cornoid lamella. We describe a case of porokeratosis in a female patient with chronic lymphedema, which was similar to Bowen’s disease due to the many glomerular vessels seen on clinical examination and dermoscopy.


Subject(s)
Humans , Female , Aged , Bowen's Disease/pathology , Bowen's Disease/diagnostic imaging , Porokeratosis/pathology , Porokeratosis/diagnostic imaging , Dermoscopy/methods , Chronic Disease , Diagnosis, Differential , Erythema/pathology , Erythema/diagnostic imaging , Lymphedema/pathology , Lymphedema/diagnostic imaging
18.
Blood ; 128(9): 1169-73, 2016 09 01.
Article in English | MEDLINE | ID: mdl-27385789

ABSTRACT

Aside from the established role for platelets in regulating hemostasis and thrombosis, recent research has revealed a discrete role for platelets in the separation of the blood and lymphatic vascular systems. Platelets are activated by interaction with lymphatic endothelial cells at the lymphovenous junction, the site in the body where the lymphatic system drains into the blood vascular system, resulting in a platelet plug that, with the lymphovenous valve, prevents blood from entering the lymphatic circulation. This process, known as "lymphovenous hemostasis," is mediated by activation of platelet CLEC-2 receptors by the transmembrane ligand podoplanin expressed by lymphatic endothelial cells. Lymphovenous hemostasis is required for normal lymph flow, and mice deficient in lymphovenous hemostasis exhibit lymphedema and sometimes chylothorax phenotypes indicative of lymphatic insufficiency. Unexpectedly, the loss of lymph flow in these mice causes defects in maturation of collecting lymphatic vessels and lymphatic valve formation, uncovering an important role for fluid flow in driving endothelial cell signaling during development of collecting lymphatics. This article summarizes the current understanding of lymphovenous hemostasis and its effect on lymphatic vessel maturation and synthesizes the outstanding questions in the field, with relationship to human disease.


Subject(s)
Blood Platelets/metabolism , Chylothorax/metabolism , Lymphatic Vessels/metabolism , Lymphedema/metabolism , Platelet Activation , Thrombosis/metabolism , Animals , Blood Platelets/pathology , Chylothorax/pathology , Chylothorax/physiopathology , Humans , Lectins, C-Type/metabolism , Lymphatic Vessels/pathology , Lymphedema/pathology , Lymphedema/physiopathology , Membrane Glycoproteins/metabolism , Mice , Thrombosis/pathology
19.
An Bras Dermatol ; 91(5 suppl 1): 119-121, 2016.
Article in English | MEDLINE | ID: mdl-28300916

ABSTRACT

Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled "volcanic crater contour" - the so-called cornoid lamella. We describe a case of porokeratosis in a female patient with chronic lymphedema, which was similar to Bowen's disease due to the many glomerular vessels seen on clinical examination and dermoscopy.


Subject(s)
Bowen's Disease/diagnostic imaging , Bowen's Disease/pathology , Dermoscopy/methods , Porokeratosis/diagnostic imaging , Porokeratosis/pathology , Aged , Chronic Disease , Diagnosis, Differential , Erythema/diagnostic imaging , Erythema/pathology , Female , Humans , Lymphedema/diagnostic imaging , Lymphedema/pathology
20.
J Reconstr Microsurg ; 32(1): 2-9, 2016 Jan.
Article in English | MEDLINE | ID: mdl-26375305

ABSTRACT

The anatomic research of the lymphatic system has been a very controversial subject throughout due to the complexity of the methods for its visualization. More than 30 years ago, together with Prof. Caplan, we began the vascular anatomy research, focusing on the lymphatic anatomy, developing and adapting different techniques of injection. On the third Normal Anatomy Chair of Buenos Aires University, we summarized the lymphatic drainage of the breast and the limbs to interpret the anatomic bases of lymphedema.


Subject(s)
Lymphatic System/anatomy & histology , Lymphedema/pathology , Humans
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