POEMS syndrome: A rare entity

POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical examination, and appropriate testing can aid in establishing its diagnosis. We are presenting the case of a 75-year-old man who was diagnosed with POEMS syndrome.

Primary hepatic carcinoma with paraneoplastic syndrome: research progresses on clinical types and their basic mechanisms / 中华肝胆外科杂志

The onset of primary hepatic carcinoma (PHC) is usually occult, and early symptoms are not obvious. Most patients are at advanced stages of disease at diagnosis, and the prognosis is poor. Paraneoplastic syndrome (PNS) refers to the clinical manifestations indirectly caused by tumor metabolites or abnormal immune reactions that cannot be explained by the primary lesion, local tumor spread or distant metastasis. Hypercholesterolemia, hypercalcemia and hypoglycemia are the most commonly seen clinical presentations of PNS in PHC patients. Adequate understanding of PNS is of great importance in early diagnosis and treatment of PHC. In this review, we summarized the clinical manifestations and prognostic mechanisms of PNS in patients with PHC.

Enfermedad de cambios mínimos como glomerulopatía paraneoplásica en un paciente con tumor sólido / Minimal Change Disease as a paraneoplastic glomerulopathy in a patient with solid tumor

Resumen La enfermedad de cambios mínimos (ECM) es la principal causa de síndrome nefrótico en niños y una causa poco común en adultos, asociada usualmente a causas secundarias, predominantemente de origen hematológico. Es poco frecuente que este tipo de glomerulopatía se diagnostique de manera sincrónica en pacientes con tumores sólidos. Presentamos el caso de un paciente de 35 años con diagnostico reciente de cáncer de recto quien ingresa con un cuadro compatible con síndrome nefrótico, a quien se le diagnostica, mediante una biopsia renal, una enfermedad de cambios mínimos, con mejoría parcial del cuadro posterior al manejo quirúrgico de la patología neoplásica. Consideramos que este caso puede ser útil en el enfoque diagnóstico y el manejo de pacientes que presenten tumores sólidos de origen gastrointestinal con síndrome nefrótico concomitante.

Abstract Minimal change disease is the main cause of nephrotic syndrome in children and a rare cause in adults, usually associated with secondary causes, predominantly hematological. This type of glomerulopathy is rarely diagnosed synchronously in patients with solid tumors. We present the case of a 35-year-old patient with a recent diagnosis of rectal cancer who is admitted with a condition compatible with nephrotic syndrome, diagnosed with minimal change disease in the renal biopsy, and with partial improvement in the condition after surgical management of neoplastic pathology. We consider that this case may be useful in the diagnostic approach and the management of patients with solid tumors of gastrointestinal origin with concomitant nephrotic syndrome.

Cáncer de mama triple negativo asociado a dermatomiositis paraneoplásica: Reporte de caso y revisión de la literatura / Triple-negative breast cancer associated with paraneoplastic dermatomyositis: Case report and literature review

Resumen La dermatomiositis (DM) es un tipo de miopatía inflamatoria bien definida, inmunomediada, con afectación específica del músculo esquelético y con compromiso variable de piel y otros órganos. Se caracteriza por debilidad muscular proximal, lesiones cutáneas patognomónicas de dermatomiositis como el signo de Gottron, eritema violáceo o heliotropo, y evidencia de inflamación muscular por enzimas elevadas, cambios miopáticos en electromiografía y biopsia muscular anormal. Tiene una asociación bien establecida con diferentes tipos de cáncer pero es rara su asociación con cáncer de mama. Cuando se presentan de manera concomitante, su diagnóstico requiere un estudio multidisciplinario para orientar el origen paraneoplásico frente a una etiología propiamente autoinmune que requiera terapia inmunosupresora dirigida. Describimos el caso de una paciente con diagnóstico simultáneo de carcinoma infiltrante de mama triple negativo y criterios de dermatomiositis como manifestación paraneoplásica.

Abstract Dermatomyositis (DM) is a well-defined immune-mediated inflammatory myopathy, with specific involvement of skeletal muscle and variable involvement of skin and other organs. It is characterized by proximal muscle weakness, pathognomonic skin lesions of dermatomyositis such as Gottron's sign, violaceous or heliotrope rash, and evidence of muscle inflammation due to elevated enzymes, myopathic changes on electromyography, and abnormal muscle biopsy. It has a well-established association with different types of cancer, but its association with breast cancer is rare. When they occur concomitantly, their diagnosis requires a multidisciplinary study to confirm the paraneoplastic origin versus a primarily autoimmune etiology that may require targeted immunosuppressive therapy. We describe the case of a patient with a simultaneous diagnosis of triple-negative infiltrating breast carcinoma and criteria for dermatomyositis as a paraneoplastic manifestation.

Dermatomiositis y cáncer colorrectal: reporte de caso y revisión de la literatura / Dermatomyositis and colorectal cancer: Case report and literature review

Resumen Los pacientes con dermatomiositis presentan un mayor riesgo de asociación con patología maligna subyacente, sin conocerse una causa precisa de esta relación. Son múltiples las posibles localizaciones anatómicas, entre las que se incluyen los ovarios, mamas, zona gástrica, zona colorrectal, sangre, pulmones y próstata. Se presenta el caso de una mujer de 58 años que durante el estudio de pérdida anormal de peso manifestó debilidad muscular grave y alteraciones dermatológicas asociadas con el hallazgo de adenocarcinoma del colon transverso.

Abstract Patients with dermatomyositis are more likely to have an underlying malignancy, although the exact cause of this association is unknown. There are multiple possible anatomical sites, including ovaries, breasts, stomach, colorectum, blood, lungs, and prostate. We present the case of a 58-year-old woman who during abnormal weight loss study showed severe muscle weakness and skin alterations, associated with finding of adenocarcinoma of the transverse colon.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

Variante "no clásica" o "atípica" del síndrome de Stauffer en paciente con carcinoma renal de células claras: reporte de caso / "Non-classical" or "atypical" variant of Stauffer syndrome in a patient with clear cell renal carcinoma: a case report

La manifestación paraneoplásica conocida como síndrome de Stauffer tiene una presentación atípica, caracterizada por ictericia y colestasis intrahepática. Presentamos el caso de un paciente de 53 años de edad, con antecedente de una masa renal derecha en plan de resección quirúrgica programada, con cuadro de evolución de dolor abdominal en hipocondrio derecho e ictericia. A su ingreso se documentó hepatoesplenomegalia, elevación de bilirrubinas a expensas de la directa, y de fosfatasa alcalina junto con elevación de transaminasas. Se descartaron causas obstructivas a nivel de vía biliar intra y extrahepática. No se documentaron metástasis o lesiones focales a nivel de parénquima, ni lesiones de etiología vascular que explicaran el cuadro. También se descartó hepatitis B, C e infección por VIH, por lo cual se consideró un probable síndrome de Stauffer. Fue llevado a nefrectomía intrahospitalaria, con posterior diagnóstico patológico compatible con carcinoma de células claras. Luego del procedimiento se normalizó la bioquímica hepática y se corrigió la ictericia. Es importante reconocer que la afectación hepática en el contexto de neoplasias, no es solo atribuida a metástasis a distancia, sino también a la existencia de síndromes paraneoplásicos como condicionantes.

The paraneoplastic manifestation known as Stauffer syndrome has an atypical presentation, characterized by jaundice and intrahepatic cholestasis. We present the case of a 53-year-old patient, with a history of a right renal mass with a planned surgical resection, who developed abdominal pain in the right upper quadrant and jaundice. Upon admission, hepatosplenomegaly, elevated bilirubin, at the expense of direct bilirubin, alkaline phosphatase and elevated transaminases were documented. Intra- and extrahepatic bile ducts obstruction were ruled out. There were no documented metastases or focal lesions at the level of the parenchyma, or lesions of vascular etiology that could explain the condition. Hepatitis B, C and HIV infection were also ruled out, and a probable Stauffer syndrome was considered. In-hospital nephrectomy was performed, with subsequent pathology compatible with clear cell carcinoma. After the procedure, liver biochemistry was normalized and jaundice was corrected. It is important to recognize that liver involvement in the context of neoplasms is not only attributed to distant metastases but to the existence of paraneoplastic syndromes as determining factors.

Eletrocardiografia computadorizada em cadelas com tumores mamários / Electrocardiography in bitches with mammary tumors

The incidence of cancer has increased due to greater longevity of the animals as a consequence of better control of other diseases, improvement of nutrition and good practices in preventive medicine. Malignant tumors can cause paraneoplastic syndrome such as hypercalcemia, anemia, cachexia, among others and consequent cardiovascular disorders. The electrocardiography (ECG) is a complementary exam that can reveal with its traces these rhythmic disorders. Based on that, the objective of this study was to evaluate the ECG in bitches with mammary tumors in order to detect in which type of tumor would be found significant changes as in cardiac rhythm, ECG waves, segments or cardiac axis. Twenty-nine female dogs were used in this study, being 19 bitches with mammary tumor, and they were allocated in three groups: G1: control group (n = 10), G2: benign neoplasia group (n = 6) and G3: malignant neoplasia group (n = 13). The most prevalent type of neoplasia in G2 was the benign mixed tumor (83%), followed by adenoma (17%) whereas in G3: carcinoma in mixed tumor (61%); papillary carcinoma (23%). Regarding cardiac rhythm, it was found sinus arrhythmia (SA) and normal sinus rhythm (NS): G1: 50% SA and 50% NS; G2: 67% SA and 33% NS; G3: 54% SA and 46% NS. No ventricular or atrial arrhythmias were detected. For other parameters in G1, G2 and G3, respectively (mean ± Std error): FC (bpm): 110±9.2, 120 ± 8.5, 124±7,5; P (ms): 48 ± 1.6, 51 ± 1.8, 50 ± 1.2; P (mV) 0.19 ± 0.02, 0.2 ± 0.02, 0.19 ± 0.02; PR (ms): 94 ± 4.3, 93 ± 5.5, 89 ± 3.9; QRS (ms): 56 ± 1.54, 60 ± 4, 62 ± 1.2; R (mV): 1.1 ± 0.06, 1.2 ± 0.24, 0.9 ± 0.13; QT (ms): 203 ± 9.4; 204 ± 7.9; 182 ± 15.6; and cardiac axis (°): 66 ± 6.2, 61 ± 7.9, 70 ± 7.5. There were no significant differences for all cardiac parameters and also for ST interval and T wave morphology. All electrocardiographic parameters found are in accordance with other studies carried out in the canine species. The results regarding tumor types differ from what was found in another study, in which in 18 bitches with mammary tumors, 55% were benign, with prevalence of adenomas (38%) followed by benign mixed tumors, and 45% malignant, with adenocarcinoma prevailing (22%). In another study, it was verified in 63 bitches the predominance of tubular carcinoma (26.56%) and carcinoma in mixed tumors (23.44%). Diverging from the electrocardiographic parameters of this study, a significant difference was found in the R wave amplitude value in the research by Barros et al., (2015) who performed computerized electrocardiography in 50 dogs, not only with mammary neoplasms (55% mammary carcinomas), but also in mastocytomas, lymphomas, benign tumors and other sarcomas. This author found out that the R wave amplitude values of the neoplasia group were lower when compared to the control group. In addition, we suspect that other types of tumors could result in more paraneoplastic syndrome than the mammary neoplasms found in this research. Neoplasms as lymphomas (T cells), apocrine gland anal sac adenocarcinomas, multiple myelomas and thymomas are known as a cause of hypercalcemia of malignancy and histiocytic sarcomas, myelomas, leukemia and lymphomas causing anemia. In conclusion, ECG has no changes in benign or malignant mammary tumors in dogs. However, this fact does not exclude the importance of its performance in pre-anesthetic evaluations. Further studies with a larger sample including the clinical staging of these bitches with a balanced number of animals with low and high staging are suggested.(AU)

Bilateral diffuse uveal melanocytic proliferation secondary to thyroid carcinoma

We present a rare case of a bilateral diffuse uveal melanocytic proliferation (BDUMP), which occurred secondary to recurrence of carcinoma of thyroid in a 79-year-old gentleman who was initially misdiagnosed to have age related macular degeneration and/or chronic central serous chorioretinopathy. In spite of being treated with anti-VEGF injection and photodynamic therapy there was progressive loss of vision. Multimodal imaging like autoflourescence, infrared imaging, fluorescein angiography, indocyanine angiography, and OCT angiography helped us in clinching the final diagnosis.

Síndrome de Stauffer como manifestación de carcinoma renal de células claras / Stauffer's syndrome as manifestation of clear cell renal carcinoma

Resumen: La incidencia de carcinoma renal de células claras representa cerca de 2% de todas las neoplasias malignas. La tríada clásica de hematuria, dolor abdominal y masa palpable se estima en 10% de los casos. Stauffer describió en 1961 el primer caso llamado síndrome de disfunción hepato-nefrogénica no metastásica, este padecimiento puede encontrarse en 3 a 20% de los casos. La afectación de las enzimas hepáticas puede estar en relación con la infiltración metastásica del hígado o, bien, por disfunción no metastásica. Es una manifestación poco frecuente en cáncer renal. Ante el hallazgo de enzimas hepáticas y fosfatasa alcalina elevadas y al descartar alteración hepática anatómica y funcional, debe sospecharse el síndrome de Stauffer, lo que puede hacer la diferencia entre que éste pase inadvertido o establecer el diagnóstico de manera oportuna. Se comunica una manifestación poco frecuente de síndrome paraneoplásico en cáncer renal de células claras.

Abstract: The incidence of clear cell renal carcinoma corresponds to about 2% of all malignant neoplasms. The classic triad of hematuria, abdominal pain and palpable mass is estimated in 10% of cases. Stauffer described in 1961 the first case called nonmetastatic hepatophrenic dysfunction syndrome, this condition can be found in 3 to 20% of cases. The involvement of liver enzymes may be related to metastatic liver infiltration or also due to non-metastatic dysfunction. It is a rare manifestation in renal cancer. Before the finding of elevated liver enzymes and alkaline phosphatase, ruling out both anatomic and functional hepatic impairment, Stauffer syndrome should be suspected, being able to make the difference between going unnoticed or being a timely finding. This paper reports a little frequent manifestation of paraneoplastic syndrome of clear cell renal carcinoma.

Unusual paraneoplastic syndrome of inappropriate antidiuretic hormone secretion with gastric cancer

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) results in impaired water excretion and consequent water intoxication and hyponatremia. In patients with cancer, SIADH is most commonly driven by ectopic ADH production, a paraneoplastic syndrome frequently seen in small cell lung cancer. A 63 year old male presented with loss of appetite, vomiting and hiccups. No abnormalities were detected on physical examination. Upper gastrointestinal endoscopy showed a proliferative growth in the stomach and histopathology report confirmed gastric adenocarcinoma. His laboratory investigations revealed low serum sodium and further work up for SIADH confirmed the diagnosis. The patient was treated with hypertonic saline and he improved symptomatically. He was subsequently treated with two cycles of capecitabine and oxaliplatin. The patient was asymptomatic and maintained a serum sodium in the range of 120 to 130 mEq/L, with a serum sodium of 127 mEq/L on follow up.

Mesotelioma pleural con manifestaciones paraneoplásicas de osteoartropatía hipertrófica / Pleural mesothelioma with paraneoplastic manifestations of hypertrophic osteoarthropathy

RESUMEN Se presenta un paciente exfumador de 65 años que, un año antes de comenzar las manifestaciones respiratorias, comenzó con dolores óseos erráticos, tratados con medicamentos comunes hasta que es atendido por disnea, tos y dolor torácico en el Hospital Básico San Antonio, ciudad de Riobamba, provincia de Chimborazo, Ecuador. El paciente presenta un gran derrame pleural, del cual obtienen 1500 ml de líquido serohemático, cuyo estudio citológico es positivo de malignidad. Se somete a cirugía, se confirma histológicamente mesotelioma maligno en etapa IV y se trata posteriormente con quimioterapia, pero el paciente empeora progresivamente hasta fallecer. Los autores resaltan que las manifestaciones paraneoplásicas del cáncer del pulmón son más frecuentes cuando la localización es parenquimatosa y no pleural, y llaman la atención sobre el hecho de que en este paciente comenzaron 1 año antes de que aparecieran los síntomas respiratorios.

ABSTRACT We present a 65-year-old ex-smoker patient, in which respiratory manifestations with erratic bone pain treated with common medications, began a year before he was atended for dyspnea, cough and chest pain at "San Antonio" Basic Hospital, Riobamba city, Chimborazo province, Ecuador. The patient presented a large pleural effusion of 1500 ml of serohematic fluid. Cytological study was positive for malignancy to rule out mesothelioma. Surgery was performed, histologically malignant mesothelioma was confirmed in stage IV and treated with chemotherapy. But the patient worsens progressively until death. Authors emphasized that lung cancer paraneoplastic manifestations were more frequent in not pleural and parenchymal location. They also called attention to patients´ symptoms, which began one year before the respiratory condition appeared.

Congenital B-cell Acute Lymphoblastic Leukemia with Congenital Rubella Infection

Background: Congenital B-cell Acute lymphoblastic leukemia (ALL) is a rare malignancy.Case Characteristics: A newborn infant presented with purpuric spots and ecchymoticpatches, blueberry muffin rash, depressed neonatal reflexes, respiratory distress andhepatosplenomegaly. Peripheral smear revealed atypical blast cells. Serum ELISA waspositive for Rubella IgM and IgG antibodies. Flow cytometry suggested congenital B-cellALL. Outcome: The baby died after 3 days due to suspected intracranial hemorrhage.Message: Congenital leukemia may be rarely associated with congenital rubella infection.

Clinical, electrophysiological profile and prognosis in paraneoplastic syndrome with SRY-like high-mobility group superfamily of developmental transcription factors 1 antibody / 中华神经科杂志

Objective To summarize the clinical,electrophysiological profile and prognosis in paraneoplastic syndrome with SRY-like high-mobility group superfamily of developmental transcription factors (SOX) 1 antibody.Methods The clinical profile,laboratory examination,electrophysiology,tumor,treatment and prognosis of three patients of paraneoplastic syndrome with SOX1 antibody in Tangdu Hospital,Air Force Military Medical University from 2016 to 2018 were retrospectively analyzed.Results Proximal lower limbs weakness was the first symptom in all the three patients,weakness normally spreading proximally to distally,involving feet and hands,finally reaching the oculobulbar region,and dry mouth was the most common autonomic dysfunction.Compound muscle action potential (CMAP) amplitude was low in all the patients,and the CMAP amplitude became even lower at low stimulating frequencies.An increase in CMAP amplitude with high-frequency stimulation was found in two patients during the follow-up.Lambert-Eaton myasthenic syndrome (LEMS) and motor axonal peripheral neuropathy were considered.Acetylcholine receptors antibody was positive in one case and voltage-gated calcium channel antibody was positive in another case.Two cases were found complicated with small cell lung carcinoma,one case with small cell carcinoma of the esophagus.After treatment of intravenous immunoglobulin,chemotherapy and pyridostigmine,the prognosis of the patients was different.Conclusions SOX1 antibody as an antibody in paraneoplastic syndrome,is most common in small cell lung cancer with LEMS,sometimes with axonal peripheral neuropathy.LEMS needs to be considered when patients manifest proximal limb weakness and dry mouth,and screening for tumors is needed.

Clinical analysis of 20 cases of paraneoplastic syndrome with prominent osteoarticular involvement / 中华风湿病学杂志

Objective To investigate the clinical characteristics of paraneoplastic syndrome with prominent osteoarticular involvement. Methods The clinical materials of 20 patients with paraneoplastic syndrome with prominent osteoarticular involvement were collected. The characteristics of clinical manifest-ations, laboratory tests and imagines were analyzed. Results Among the 20 patients, 16 were male and 4 were female, with a mean age of 44.5 years and a median course of 6 months. Ten cases were associated with hematological tumor and 10 cases were associated with solid tumor. Eleven cases presented as peripheral arthritis (7 cases of polyarthritis, 4 cases of oligoarthritis/monoarthritis), 5 cases presented with hypertrophic osteoarthropathy (HOA) and 4 cases presented with tumor-induced osteomalacia (TIO). Three cases were acute lymphocytic leukemia, 2 cases were multiple myeloma, 1 case was lymphoma, and 1 case was bone tumor in polyarthritis. Four oligoarthritis cases were all associated with acute lymphocytic leukemia. All 5 cases of HOA were associated with lung cancer. All 4 cases of TIO were associated with tumor of mesenchymal tissue. Extra-articular manifestations presented in 14 cases and inflammatory markers increased in 15 cases. anti-cyclic cirullinated peptide (anti-CCP) antibodies was low titer positive in only 1 case and other parameters including rheumatoid factor (RF), anti-CCP antibodies, antinuclear antibodies spectrum (ANAs) and human leukocyte antigen (HLA)-B27 were negative. Multiple bone imaging abnormalities appeared in 15 cases. Conclusion Osteoarticular manifestations may be the first symptom of malignancy and difficult to diagnose. It is necessary to be highly aware of potential malignancy.

Lupus-like syndrome / 国际儿科学杂志

Lupus-like syndrome is characterized by positive anti-nuclear antibody and clinical manifestations similar with systemic lupus erythematosus (SLE).Primary immunodeficiencies (PID) are specific underlying causes of lupus-like syndrome in children.Various infections,drugs,and tumors can also have manifestations like SLE.Treatment for the underlying disease is recommended.Whether immunosuppressive therapies should be initiated depending on the condition of the patient.

A Man Presenting with Sudden Weakness and Pain of the Right Hand, by Non-Small Cell Lung Cancer with Brain Metastases

Unexplained pain and weakness, i.e., without obvious predisposing factors, are often encountered by physiatrists and efforts should be made to determine the cause. A 63-year-old male presented with radiating pain in his right arm and mild weakness of the right hand. An electrodiagnostic examination revealed distal symmetric sensory polyneuropathy in the upper and lower extremities, and denervation potentials in the forearm muscles, which were inconsistent with the cervical spine MRI images and symptoms. A predisposing undiscovered disease was revealed, i.e., squamous cell carcinoma in the lung; brain metastasis affecting the left primary motor cortex was also detected. Therefore, we concluded that the pain and weakness were related to paraneoplastic syndrome and brain metastases of the hand knob. The observed denervation potentials were characterized as trans-synaptic changes in the brain metastasis. This case highlights the importance of unexplainable focal pain and weakness in the increasing prevalence of cancer.

Paraneoplastic Dermatomyositis with Severe Itching / 대한피부과학회지

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by typical cutaneous manifestation and inflammation of the skeletal muscles. However, this progressive symmetric proximal muscle weakness may be minimal or absent in clinically amyopathic DM (CADM). Importantly, DM has been associated with malignancy, which is related to poor prognosis. Therefore, every newly diagnosed patient with DM should undergo thorough screening investigations for hidden internal malignancy. We report a case of CADM, with severe intractable itching, which was finally diagnosed as paraneoplastic dermatomyositis associated with gastric cancer in a 77-year-old woman. The patient had typical cutaneous features of DM and mild elevation of muscle enzymes without muscle weakness.

Synchronous Orofacial Granulomatosis in a Patient with Brain Cavernous Hemangioma

Orofacial granulomatosis is a rare granulomatous inflammatory disease, characterized by recurrent orofacial swelling. Infectious, genetic, and immunologic etiologies are suggested, but not fully understood. Herein, we report a case of synchronous orofacial granulomatosis with brain cavernous hemangioma in a 44-year-old female patient, which may be considered paraneoplastic syndrome.

Paraneoplastic Chorea Associated with Anti-Hu Antibody and Small Cell Lung Carcinoma

No abstract available.