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Neutrophilic dermatosis of the dorsal hands (NDDH) is an uncommon localized variant of Sweet syndrome first described in 1995. It is characterized by tender erythematous plaques, pustules, and bullae on the dorsa of the hands. A total of 123 cases of NDDH are included in this review. The mean patient age was 62.1 years, and there was a slight female preponderance. Overall, 78.0% of cases had bilateral involvement, and other sites were affected in almost a third of cases. Underlying disease was found in â¼40% of patients, with the most common associations being hematologic disorders (gammopathies, myelodysplasias, or malignancies), recent infection, solid organ tumors, and inflammatory bowel disease. Systemic or topical corticosteroids or both were employed in the treatment of 88.1% of cases, while dapsone, colchicine, and tetracyclines were the most common steroid-sparing agents used. Improvement was often rapid and complete resolution the norm. Although uncommon, NDDH is frequently misdiagnosed, and thus, its exact prevalence is probably underestimated. Misdiagnosis might have significant implications, including treatment delays or incorrect management. Moreover, recognition of NDDH is important, since a correct diagnosis should trigger a search for underlying diseases and proper treatment with corticosteroids, steroid-sparing agents, or both, which is almost invariably curative.
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Dermatitis , Dermatosis de la Mano , Síndrome de Sweet , Humanos , Femenino , Persona de Mediana Edad , Dermatosis de la Mano/diagnóstico , Dermatosis de la Mano/tratamiento farmacológico , Dermatosis de la Mano/complicaciones , Glucocorticoides , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/tratamiento farmacológico , Síndrome de Sweet/complicaciones , Antibacterianos , Dermatitis/complicaciones , VesículaRESUMEN
ABSTRACT: Neuroendocrine transdifferentiation refers to the progressive transformation of a nonneuroendocrine tumor to the one showing evidence of neuroendocrine differentiation on morphological and immunohistochemical grounds. Although this phenomenon has been well-documented in certain malignancies, particularly prostatic adenocarcinoma after androgen deprivation, cases of neuroendocrine transdifferentiation in melanomas are exceptionally rare. Herein, we report a case of a conventional superficial spreading melanoma occurring on the skin of the leg in a young male which showed progressive neuroendocrine transdifferentiation as it progressed through 2 in-transit metastases and a nodal metastatic deposit over a 4-year period. The tumor retained the BRAF V600E mutation throughout the disease process, and disease control was achieved through dual BRAF inhibition therapy. The possibility of melanoma masquerading as a high-grade neuroendocrine malignancy when investigating tumors of unknown primary should be kept in the mind of clinicians and histopathologist alike as a potential diagnostic pitfall, thus helping avoid misdiagnosis and guide appropriate treatment strategies.
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Melanoma , Neoplasias de la Próstata , Neoplasias Cutáneas , Masculino , Humanos , Melanoma/genética , Melanoma/terapia , Melanoma/patología , Neoplasias Cutáneas/patología , Transdiferenciación Celular , Proteínas Proto-Oncogénicas B-raf/genética , Antagonistas de Andrógenos , Inmunohistoquímica , Mutación , Melanoma Cutáneo MalignoAsunto(s)
Trasplante de Riñón , Anomalías Cutáneas , Humanos , Trasplante de Riñón/efectos adversos , RiñónRESUMEN
Pyoderma gangrenosum (PG) after breast-conserving surgery is rare, and its diagnosis is often delayed because of the similarity to wound infection and the broad differential diagnosis for PG, making it a diagnosis of exclusion. A 60-year-old woman who underwent breast conserving surgery and sentinel lymph node biopsy for invasive breast carcinoma presented with increasing erythema, fever and serosanguinous discharge in the lower outer quadrant of the right breast at the site of tumour excision on postoperative day (POD) 9. Fever persisted despite antibiotics and the patient was noted to have leucocytosis (0.9 x 109/L), neutrophilia (37.8 x 109/L) and elevated C-reactive protein levels (136 µg/mL) on POD 16. Microbiology and blood culture results were negative but the breast ulcer continued to expand at a rate of 1-2 cm a day. The patient underwent surgical debridement on POD 21 to rule out necrotising soft tissue infection. Persistent ulcer progression, despite debridement and antibiotics, led to clinical suspicion of PG and the patient was started on prednisolone and cyclosporin. A rapid response was seen with treatment and an optimum healing process was noted over the subsequent three-month follow-up period. Early suspicion, careful macroscopic evaluation of disease progression and appropriate use of immunosuppressive therapy are important for the management of PG. Prompt initiation of immunosuppressive therapy may avoid unnecessary treatment and aggravation of the surgical wound.
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BACKGROUND: Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management. CASE PRESENTATION: This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery. CONCLUSION: Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival.
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Immunotherapy has nowadays become part of the mainstay of treatment for several cancers. In this report, we present a case of toxic epidermal necrolysis (TEN) occurring as a relatively rare complication of pembrolizumab, which is a humanized monoclonal anti-programmed cell death-1 (anti-PD1) IgG4 antibody that targets tumours expressing PDL-1. In our case, this monoclonal antibody (MAB) was being used for the treatment of metastatic non-small cell lung cancer, before being withheld because of this potentially life-threatening reaction. There are no guidelines on the optimal management of TEN, with varying reports on the efficacy on steroids, intravenous immunoglobulin (IVIG), and cyclosporin. In this report, we describe a case of TEN following the second dose of three-weekly pembrolizumab in a 50-year-old man, comparing it to four similar cases reported in the literature. It is important that oncologists are aware of this potentially life-threatening adverse reaction of pembrolizumab, as early recognition and treatment of TEN are essential to improve clinical outcome.
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A young man was treated in hospital for sepsis, disseminated intravascular coagulation and multi-organ failure. He was a regular intranasal cocaine user up to 1 day prior to symptom onset. Clinical examination revealed extensive retiform purpura affecting both his lower limbs. Skin biopsy revealed widespread thrombosis in the small- and medium-sized vessels of the mid dermis and the subcutaneous fat with surrounding leucocytoclasis. There was also extensive ischaemic necrosis of the upper reticular and papillary dermis and focal ischaemic necrosis of the epidermis. These findings were in keeping with a thrombotic vasculopathy with associated cutaneous ischaemic necrosis, likely associated with levamisole-adulterated cocaine (LAC). An autoimmune screen showed extremely raised levels of anti-B2-glycoprotein IgM, IgG and anti-cardiolipin IgG antibodies, usually seen in antiphospholipid syndrome (APS). The literature describes how APS could be secondary to various underlying conditions, including LAC, and that levamisole toxicity may mimic APS and hence be missed. LEARNING POINTS: Levamisole is a common adulterant found in cocaine; the resultant toxicity can present with cutaneous manifestations, namely retiform purpura and skin necrosis, similar to antiphospholipid syndrome.Patients presenting with such features should be asked about illicit drug use, specifically cocaine, and investigated by screening urine for drugs of abuse and serum antihuman elastase antibody when possible.
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Diffuse large B-cell lymphoma (DLBCL) of the penis is a rare haematological malignancy, with less than 30 cases being reported in the literature. Our case mentions a 57-year-old man who presented with a penile lesion that was diagnosed as DLBCL on histological biopsy. Targeted investigation and proper diagnosis are essential to distinguish it from squamous cell carcinoma (SCC) of the glans, which presents similarly. Since guidelines are not available, the author compared different management mentioned in case reports and the outcome was noted. Chemotherapy, radiotherapy and surgery were the treatment modalities most used; out of which, chemotherapy had the most successful outcome. On the other hand, surgery is the preferred modality in SCC of the penis. This further consolidates the need for guidelines with regard to proper diagnosis and management of this malignancy.
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Carcinoma de Células Escamosas , Linfoma de Células B Grandes Difuso , Neoplasias del Pene , Biopsia , Humanos , Linfoma de Células B Grandes Difuso/diagnóstico , Linfoma de Células B Grandes Difuso/terapia , Masculino , Persona de Mediana Edad , Neoplasias del Pene/diagnóstico , Neoplasias del Pene/terapia , PeneRESUMEN
Superficial granulomatous pyoderma is a relatively rare variant of pyoderma gangrenosum, characterized by superficial ulceration with a vegetative margin and a clean granulating base. Ulcers in superficial granulomatous pyoderma are typically located on the trunk and may follow minor trauma. It may be misdiagnosed as classic pyoderma gangrenosum, despite having distinct characteristics. Here we report a case of superficial granulomatous pyoderma successfully treated with intravenous immunoglobulin (IVIg). LEARNING POINTS: Superficial granulomatous pyoderma is a rare variant of pyoderma gangrenosum.Histopathological and clinical characteristics of superficial granulomatous pyoderma are distinct from those of classic pyoderma gangrenosum.Superficial granulomatous pyoderma is often slow-growing and is only rarely linked to underlying systemic disease.IVIg may be an effective treatment option for superficial granulomatous pyoderma.
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Epithelioid trophoblastic tumours are rare neoplasms showing differentiation towards the chorion leave-type intermediate cytotrophoblast, with only a handful of cases being reported in the literature. These tumours are slow-growing and are typically confined to the uterus for extended periods of time. While the pathogenesis is unclear, they are thought to arise from a remnant intermediate trophoblast originating from prior normal pregnancies or, less frequently, gestational trophoblastic tumours. A protracted time period between the gestational event and tumour development is typical. This case describes a 49-year-old previously healthy female who presented with a completely asymptomatic uterine mass, discovered incidentally during a routine gynaecological assessment. The pathological analysis of the hysterectomy specimen confirmed an epithelioid trophoblastic tumour, involving the uterus and cervix. This is a rare gynaecological tumour. A comparative short tandem repeat analysis revealed genetic similarities to a previous healthy gestation seventeen years prior. She was successful treated with adjuvant pembrolizumab, with no evidence of disease recurrence to date.
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Enfermedad Trofoblástica Gestacional , Neoplasias Uterinas , Anticuerpos Monoclonales Humanizados , Femenino , Ligamiento Genético , Enfermedad Trofoblástica Gestacional/tratamiento farmacológico , Enfermedad Trofoblástica Gestacional/genética , Enfermedad Trofoblástica Gestacional/cirugía , Humanos , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Embarazo , Neoplasias Uterinas/tratamiento farmacológico , Neoplasias Uterinas/genética , Neoplasias Uterinas/cirugíaRESUMEN
We describe the case of a 77-year-old woman, presenting with non-specific epigastric pain. Physical examination and subsequent imaging revealed the presence of a large mass in the right liver lobe. This was shown to be a leiomyosarcoma on biopsy histology. Further investigation confirmed this to be a primary hepatic leiomyosarcoma with no evidence of metastases. The patient underwent successful surgical resection. She is currently under imaging follow-up, with no evidence of disease recurrence.
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Hepatectomía , Laparoscopía , Leiomiosarcoma/cirugía , Neoplasias Hepáticas/cirugía , Anciano , Femenino , Humanos , Leiomiosarcoma/diagnóstico por imagen , Neoplasias Hepáticas/diagnóstico por imagenRESUMEN
OBJECTIVE: The aim of the study was to compare the relative predictive value of Marshall Classification System and Rotterdam scores on long-term rehabilitation outcomes. This study hypothesized that Rotterdam would outperform Marshall Classification System. DESIGN: The study used an observational cohort design with a consecutive sample of 88 participants (25 females, mean age = 42.0 [SD = 21.3]) with moderate to severe traumatic brain injury who were admitted to trauma service with subsequent transfer to the rehabilitation unit between February 2009 and July 2011 and who had clearly readable computed tomography scans. Twenty-three participants did not return for the 9-mo postdischarge follow-up. Day-of-injury computed tomography images were scored using both Marshall Classification System and Rotterdam criteria by two independent raters, blind to outcomes. Functional outcomes were measured by length of stay in rehabilitation and the cognitive and motor subscales of the Functional Independence Measure at rehabilitation discharge and 9-mo postdischarge follow-up. RESULTS: Neither Marshall Classification System nor Rotterdam scales as a whole significantly predicted Functional Independence Measure motor or cognitive outcomes at discharge or 9-mo follow-up. Both scales, however, predicted length of stay in rehabilitation. Specific Marshall scores (3 and 6) and Rotterdam scores (5 and 6) significantly predicted subacute outcomes such as Functional Independence Measure cognitive at discharge from rehabilitation and length of stay. CONCLUSIONS: Marshall Classification System and Rotterdam scales may have limited utility in predicting long-term functional outcome, but specific Marshall and Rotterdam scores, primarily linked to increased severity and intracranial pressure, may predict subacute outcomes.
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Lesiones Traumáticas del Encéfalo/diagnóstico por imagen , Estadística como Asunto/métodos , Tomografía Computarizada por Rayos X/clasificación , Adulto , Lesiones Traumáticas del Encéfalo/rehabilitación , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Resultado del TratamientoRESUMEN
Systemic to pulmonary fistulas are an unusual entity, even more so in association with Hodgkin's lymphoma. We herein report a case of a 33-year-old woman that presented with an incidental lung lesion on a chest radiograph with an associated high-frequency continuous murmur over the lesion. The diagnosis of primary pulmonary Hodgkin's lymphoma, nodular sclerosis type, was obtained by a CT transthoracic biopsy. We achieved an excellent response after polychemotherapy with near-complete disappearance of the mass and a residual faint systolic murmur over the lesion.
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Enfermedad de Hodgkin/diagnóstico , Neoplasias del Mediastino/patología , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Tos/etiología , Ecocardiografía , Femenino , Soplos Cardíacos/diagnóstico por imagen , Soplos Cardíacos/etiología , Enfermedad de Hodgkin/complicaciones , Enfermedad de Hodgkin/patología , Humanos , Imagen por Resonancia Magnética , Neoplasias del Mediastino/diagnóstico por imagen , Neoplasias del Mediastino/etiología , Tomografía Computarizada por Rayos XRESUMEN
Patients with C1 nerve root schwannomas usually present with signs relating to nerve root compression. However, asymptomatic presentations have never been reported. A healthy, 37-year-old female was referred in view of a slow-growing lump in the left posterosuperior aspect of the neck. The lump was asymptomatic and neurological examination was normal. Magnetic resonance imaging revealed a left C1 nerve root tumour, extending around the C1 vertebra and compressing the thecal sac. The tumour had invaded the basiocciput and was impinging on the left cerebellar hemispheric dura. Stereotactic biopsies of the lesion showed a spindle-cell tumour exhibiting an immunoprofile consistent with a schwannoma. The lesion was surgically excised by blunt dissection using a posterior midline approach. The case report adds to the diverse modes of presentation of C1 nerve root schwannomas, in that such lesions must be included in the differential diagnosis of asymptomatic posterior neck lumps.
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BACKGROUND: Day-of-injury (DOI) brain lesion volumes in traumatic brain injury (TBI) patients are rarely used to predict long-term outcomes in the acute setting. The purpose of this study was to investigate the relationship between acute brain injury lesion volume and rehabilitation outcomes in patients with TBI at a level one trauma center. METHODS: Patients with TBI who were admitted to our rehabilitation unit after the acute care trauma service from February 2009-July 2011 were eligible for the study. Demographic data and outcome variables including cognitive and motor Functional Independence Measure (FIM) scores, length of stay (LOS) in the rehabilitation unit, and ability to return to home were obtained. The DOI quantitative injury lesion volumes and degree of midline shift were obtained from DOI brain computed tomography scans. A multiple stepwise regression model including 13 independent variables was created. This model was used to predict postrehabilitation outcomes, including FIM scores and ability to return to home. A p value less than 0.05 was considered significant. RESULTS: Ninety-six patients were enrolled in the study. Mean age was 43 ± 21 years, admission Glasgow Coma Score was 8.4 ± 4.8, Injury Severity Score was 24.7 ± 9.9, and head Abbreviated Injury Scale score was 3.73 ± 0.97. Acute hospital LOS was 12.3 ± 8.9 days, and rehabilitation LOS was 15.9 ± 9.3 days. Day-of-injury TBI lesion volumes were inversely associated with cognitive FIM scores at rehabilitation admission (p = 0.004) and discharge (p = 0.004) and inversely associated with ability to be discharged to home after rehabilitation (p = 0.006). CONCLUSION: In a cohort of patients with moderate to severe TBI requiring a rehabilitation unit stay after the acute care hospital stay, DOI brain injury lesion volumes are associated with worse cognitive FIM scores at the time of rehabilitation admission and discharge. Smaller-injury volumes were associated with eventual discharge to home. Volumetric neuroimaging in the acute injury phase may improve surgeons' ultimate outcome predictions in TBI patients. LEVEL OF EVIDENCE: Prognostic/epidemiologic study, level V.